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Correale M, Bevere EML, Tricarico L, Villani D, Granato M, Guerriero E, Capasso R, Rossi L, Rotondo C, Cantatore FP, Corrado A, Iacoviello M, Brunetti ND. How to Assess Pulmonary Circulation and Right Heart Chambers in Systemic Sclerosis Patients? Diagnostics (Basel) 2025; 15:1029. [PMID: 40310415 PMCID: PMC12026199 DOI: 10.3390/diagnostics15081029] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/12/2025] [Revised: 04/11/2025] [Accepted: 04/13/2025] [Indexed: 05/02/2025] Open
Abstract
Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by a widespread accumulation of extracellular matrix components leading to fibrosis of the skin and internal organs. Vascular changes occur in all involved tissues and are responsible for several distinctive clinical manifestations of the disease. This review focuses on the usefulness of various diagnostic tools in clinical practice for the early identification of clinical, functional, and/or structural RV impairment in SSc patients at risk of PH. It aims to identify specific causes of RV dysfunction, describe potential differences in outcome measures, and, ultimately, determine different cut-off values compared to subjects with PH not related to SSc.
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Affiliation(s)
- Michele Correale
- Cardiothoracic Department, Ospedali Riuniti University Hospital, 71100 Foggia, Italy
| | - Ester Maria Lucia Bevere
- Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (E.M.L.B.); (L.T.); (D.V.); (M.G.); (E.G.); (R.C.); (L.R.); (M.I.); (N.D.B.)
| | - Lucia Tricarico
- Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (E.M.L.B.); (L.T.); (D.V.); (M.G.); (E.G.); (R.C.); (L.R.); (M.I.); (N.D.B.)
| | - Deborah Villani
- Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (E.M.L.B.); (L.T.); (D.V.); (M.G.); (E.G.); (R.C.); (L.R.); (M.I.); (N.D.B.)
| | - Mattia Granato
- Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (E.M.L.B.); (L.T.); (D.V.); (M.G.); (E.G.); (R.C.); (L.R.); (M.I.); (N.D.B.)
| | - Erminia Guerriero
- Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (E.M.L.B.); (L.T.); (D.V.); (M.G.); (E.G.); (R.C.); (L.R.); (M.I.); (N.D.B.)
| | - Raffaele Capasso
- Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (E.M.L.B.); (L.T.); (D.V.); (M.G.); (E.G.); (R.C.); (L.R.); (M.I.); (N.D.B.)
| | - Luciano Rossi
- Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (E.M.L.B.); (L.T.); (D.V.); (M.G.); (E.G.); (R.C.); (L.R.); (M.I.); (N.D.B.)
| | - Cinzia Rotondo
- Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (C.R.); (F.P.C.); (A.C.)
| | - Francesco Paolo Cantatore
- Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (C.R.); (F.P.C.); (A.C.)
| | - Addolorata Corrado
- Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (C.R.); (F.P.C.); (A.C.)
| | - Massimo Iacoviello
- Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (E.M.L.B.); (L.T.); (D.V.); (M.G.); (E.G.); (R.C.); (L.R.); (M.I.); (N.D.B.)
| | - Natale Daniele Brunetti
- Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy; (E.M.L.B.); (L.T.); (D.V.); (M.G.); (E.G.); (R.C.); (L.R.); (M.I.); (N.D.B.)
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Sun G, Fosbøl EL, Faurschou M, Schou M, Torp-Pedersen C, Køber L, Butt JH. Long-term rate of heart failure in patients with autoimmune disease: A nationwide cohort study. Eur J Heart Fail 2025; 27:479-487. [PMID: 39654406 DOI: 10.1002/ejhf.3541] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/20/2024] [Revised: 11/09/2024] [Accepted: 11/13/2024] [Indexed: 04/01/2025] Open
Abstract
AIMS Although certain autoimmune diseases (AIDs) have been associated with an increased rate of heart failure (HF), data on the long-term rate of HF across the spectrum of AIDs are lacking. We investigated the long-term rate of HF in individuals with a history of 28 different AIDs. METHODS AND RESULTS Individuals diagnosed with an AID (2000-2021) were identified through Danish nationwide registries. Each patient with AID was matched with four individuals from the background population by age, sex, and year of inclusion. Multivariable Cox regression was used to compare the rate of HF between the AID and the background population, overall and according to individual AIDs. In total, 272 959 patients diagnosed with AID were matched with 1 091 836 individuals without AID (median age 55 years; 62% women; median follow-up 7.9 years). The 10-year cumulative incidence of HF was 5.2% for patients with AID and 3.5% for matched individuals. Patients with any AID had a higher associated rate of HF than matched individuals (hazard ratio [HR] 1.55, 95% confidence interval [CI] 1.52-1.59). Patients with each of the AIDs had a higher associated rate of incident HF compared with matched individuals from the background population, although the association was not statistically significant for Reiter's and Behcet's disease. The highest relative rates were observed in patients with systemic sclerosis (HR 3.31, 95% CI 2.63-4.16) and Addison's disease (HR 3.03, 95% CI 2.35-3.91). CONCLUSION Patients with AID, irrespective of the type, had a higher associated rate of HF compared to the background population. Further research is needed to clarify whether screening for cardiovascular risk is beneficial.
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Affiliation(s)
- Guoli Sun
- Department of Cardiology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
- Department of Cardiology, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, China
| | - Emil L Fosbøl
- Department of Cardiology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
| | - Mikkel Faurschou
- Department of Rheumatology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
| | - Morten Schou
- Department of Cardiology, Herlev-Gentofte University Hospital, Hellerup, Denmark
| | | | - Lars Køber
- Department of Cardiology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
| | - Jawad H Butt
- Department of Cardiology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
- Department of Cardiology, Zealand University Hospital, Roskilde, Denmark
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Giubertoni A, Bellan M, Cumitini L, Patti G. Cardiopulmonary Exercise Testing: Deciphering Cardiovascular Complications in Systemic Sclerosis. Rev Cardiovasc Med 2025; 26:25914. [PMID: 39867169 PMCID: PMC11759969 DOI: 10.31083/rcm25914] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/30/2024] [Revised: 09/26/2024] [Accepted: 10/15/2024] [Indexed: 01/28/2025] Open
Abstract
Cardiac manifestations in systemic sclerosis (SSc) are variable and are associated with a poor prognosis, frequently resulting in impaired right ventricular function and heart failure. A high proportion of patients with SSc experience pulmonary arterial hypertension (PAH), interstitial lung disease, or myocardial involvement, all of which can lead to exercise intolerance. In this context, cardiopulmonary exercise testing (CPET) is a useful tool for diagnosing exercise intolerance, elucidating its pathophysiology, and assessing its prognosis. CPET can also identify patients with SSc at higher risk of developing PAH. Despite its utility, current guidelines for CPET do not include the evaluation of patients with SSc, nor do standard SSc management guidelines consider CPET in the clinical work-up. This review summarizes the development, supporting evidence, and application of CPET in assessing cardiac involvement in patients with SSc.
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Affiliation(s)
- Ailia Giubertoni
- Division of Cardiology, Maggiore della Carità Hospital, 28100 Novara, Italy
| | - Mattia Bellan
- Department of Translational Medicine, University of Eastern Piedmont, 28100 Novara, Italy
- Division of Internal Medicine, Maggiore della Carità Hospital, 28100 Novara, Italy
| | - Luca Cumitini
- Division of Cardiology, Maggiore della Carità Hospital, 28100 Novara, Italy
- Department of Translational Medicine, University of Eastern Piedmont, 28100 Novara, Italy
| | - Giuseppe Patti
- Division of Cardiology, Maggiore della Carità Hospital, 28100 Novara, Italy
- Department of Translational Medicine, University of Eastern Piedmont, 28100 Novara, Italy
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Magda ȘL, Gheorghiu AM, Mincu RI, Ciobanu AO, Constantinescu T, Popa EC, Mihai C, Vinereanu D. Non-Invasive Cardiac and Vascular Monitoring in Systemic Sclerosis: Impact of Therapy on Subclinical Dysfunction. MEDICINA (KAUNAS, LITHUANIA) 2024; 60:2080. [PMID: 39768959 PMCID: PMC11678526 DOI: 10.3390/medicina60122080] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 10/31/2024] [Revised: 12/11/2024] [Accepted: 12/17/2024] [Indexed: 01/11/2025]
Abstract
Background and objectives: Systemic sclerosis (SSc) causes myocardial and microvascular impairment, with subclinical dysfunction and eventually permanent cardio-vascular damage. The long-term influence of SSc therapies on subclinical cardiovascular dysfunction is insufficiently investigated. We aimed to assess 2D and 4D cardiac ultrasound parameters of heart function in patients with different forms of SSc versus controls and to determine the evolution of cardiac function and arterial stiffness parameters under therapy. Materials and methods: A total of 60 subjects with SSc were studied at baseline; 30 SSc patients were compared to 30 matched controls. A total of 52 SSc subjects were reassessed after 1 year and 30 after 2 years of treatment. Cardiac function was evaluated through 2D standard echocardiography, tissue Doppler, speckle tracking and 4D auto LV quantification echo. Arterial stiffness was determined via the cardio-ankle vascular index and ankle brachial index. Results: At baseline, the standard echo parameters were normal. The 4D and myocardial work parameters, although in normal limits, were significantly altered in the SSc group vs. controls (4D ejection fraction 54.5 ± 8.5% in SSc vs. 63.8 ± 3.1% in controls; 4D longitudinal strain -14.2 ± 2.4% in SSc vs. -22.0 ± 2.7% in controls; global constructive work 2124.2 ± 449.5 mmHg% in SSc vs. 3102.8 ± 337.5 mmHg% in controls, for all p ≤ 0.02). Both at 1 year and 2 years of treatment, all echo and arterial stiffness parameters were similar to baseline, with no correlation to treatment type. Conclusions: SSc determines subclinical systolic dysfunction. Non-invasive assessment methods do not detect a functional cardiovascular decline in patients on classical therapy. Complex cardiac follow-up should be implemented in cases at risk for complications.
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Affiliation(s)
- Ștefania Lucia Magda
- Department of Cardiology and Cardiovascular Surgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- Department of Cardiology and Cardiovascular Surgery, University and Emergency Hospital, 050098 Bucharest, Romania
| | - Ana Maria Gheorghiu
- Department of Cardiology and Cardiovascular Surgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- Department of Rheumatology, Clinical Hospital “Dr. Ioan Cantacuzino”, 050474 Bucharest, Romania
| | - Raluca Ileana Mincu
- Department of Cardiology and Vascular Medicine, West German Heart and Vascular Medicine Center, 45147 Essen, Germany
| | - Andrea Olivia Ciobanu
- Department of Cardiology and Cardiovascular Surgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- Department of Cardiology and Cardiovascular Surgery, University and Emergency Hospital, 050098 Bucharest, Romania
| | - Tudor Constantinescu
- Department of Cardiology and Cardiovascular Surgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- Institute of Pneumology “Marius Nasta”, 050159 Bucharest, Romania
| | - Elisa Cristina Popa
- Department of Cardiology and Cardiovascular Surgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- Department of Cardiology and Cardiovascular Surgery, University and Emergency Hospital, 050098 Bucharest, Romania
| | - Carina Mihai
- Department of Rheumatology, University Hospital Zürich, University of Zürich, 8091 Zürich, Switzerland
| | - Dragoș Vinereanu
- Department of Cardiology and Cardiovascular Surgery, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- Department of Cardiology and Cardiovascular Surgery, University and Emergency Hospital, 050098 Bucharest, Romania
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Guédon AF, Carrat F, Mouthon L, Launay D, Chaigne B, Pugnet G, Lega JC, Hot A, Cottin V, Agard C, Allanore Y, Fauchais AL, Lescoat A, Dhote R, Papo T, Chatelus E, Bonnotte B, Kahn JE, Diot E, Aouba A, Magy-Bertrand N, Queyrel V, Le Quellec A, Kieffer P, Amoura Z, Granel B, Gaultier JB, Balquet MH, Wahl D, Lidove O, Espitia O, Cohen A, Fain O, Hachulla E, Mekinian A, Rivière S. Vasodilator drugs and heart-related outcomes in systemic sclerosis: an exploratory analysis. RMD Open 2024; 10:e004918. [PMID: 39658051 PMCID: PMC11629012 DOI: 10.1136/rmdopen-2024-004918] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/27/2024] [Accepted: 11/04/2024] [Indexed: 12/12/2024] Open
Abstract
BACKGROUND AND AIMS Systemic sclerosis (SSc) is an autoimmune connective disease characterised by excessive extracellular matrix deposition and widespread skin and internal organ fibrosis including various cardiac manifestations. Heart involvement is one of the leading causes of death among patients with SSc. In this study, we aimed to assess the effect of various vasodilator treatments. METHODS We used data from a national multicentric prospective study using the French SSc national database. We estimated the average treatment effect (ATE) of sildenafil, bosentan, angiotensin-converting enzyme (ACE) inhibitors and iloprost on diastolic dysfunction, altered ejection fraction <50% and pulmonary arterial hypertension (PAH) using a causal method, namely the longitudinal targeted minimum loss-based estimation, to adjust for confounding and informative censoring. RESULTS We included 1048 patients with available data regarding treatment. Regarding sildenafil analyses, the ATE on diastolic dysfunction at 3 years was -2.83% (95% CI -4.06; -1.60, p<0.00001), and the estimated ATE on altered ejection fraction <50% was -0.88% (95% CI -1.70; -0.05, p=0.037). We did not find a significative effect on PAH. Regarding bosentan, ACE inhibitors and iloprost, none of them neither showed a significant effect on diastolic dysfunction, altered ejection fraction <50% or PAH. CONCLUSIONS Using causal methods, our study is the first and largest suggesting that sildenafil might have benefits among SSc patients regarding diastolic dysfunction and altered ejection fraction occurrence. However, further studies assessing the effect of vasodilators on heart-related outcome among SSc patients are needed to confirm those exploratory results.
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Affiliation(s)
- Alexis F Guédon
- Institut Pierre Louis d'Epidemiologie et de Sante Publique, Paris, Île-de-France, France
- Sorbonne Université, APHP, Service de Médecine Interne, Hopital Saint-Antoine, Paris, Île-de-France, France
| | - Fabrice Carrat
- Institut Pierre Louis d'Epidemiologie et de Sante Publique, Paris, Île-de-France, France
| | - Luc Mouthon
- Department of Internal Medicine, Hopital Cochin, Paris, Île-de-France, France
| | - David Launay
- Department of Internal Medicine and Clinical immunology, Referral Centre for Rare Systemic Auto-immune Diseases North and North-West of France, Univ. Lille, Inserm, CHU de Lille, Lille, Hauts-de-France, France
| | - Benjamin Chaigne
- Department of Internal Medicine, Hopital Cochin, Paris, Île-de-France, France
| | - Grégory Pugnet
- Internal Medicine Department, CHU Toulouse, Toulouse, Occitanie, France
| | - Jean-Christophe Lega
- Department of Internal and Vascular Medicine, Hospices Civils de Lyon, Lyon, Auvergne-Rhône-Alpes, France
| | - Arnaud Hot
- Department of Internal Medicine, Hospices Civils de Lyon, Lyon, Auvergne-Rhône-Alpes, France
| | - Vincent Cottin
- National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, Auvergne-Rhône-Alpes, France
| | - Christian Agard
- Service de Médecine Interne, Centre Hospitalier Universitaire de Nantes, Nantes, France
| | - Yannick Allanore
- Department of Rheumatology, Hospital Cochin, Paris, Île-de-France, France
| | - Anne Laure Fauchais
- Department of Internal Medicine, CHU Limoges, Limoges, Nouvelle-Aquitaine, France
| | - Alain Lescoat
- Department of Internal Medicine and Clinical Immunology, CHU de Rennes, Rennes, Bretagne, France
| | - Robin Dhote
- Department of Internal Medicine, Hopital Avicenne, Bobigny, France
| | - Thomas Papo
- Department of Internal Medicine, Hôpital Bichat Claude-Bernard, Paris, Île-de-France, France
| | - Emmanuel Chatelus
- Rheumatology, Hopitaux universitaires de Strasbourg, Strasbourg, France
| | - Bernard Bonnotte
- Department of Internal Medicine, Centre Hospitalier Universitaire Dijon Bourgogne, Dijon, Bourgogne-Franche-Comté, France
| | - Jean-Emmanuel Kahn
- Department of Internal Medicine, Hopital Ambroise-Pare, Boulogne-Billancourt, Île-de-France, France
| | - Elisabeth Diot
- Department of Internal Medicine and Clinical Immunology, CHRU de Tours, Tours, Centre-Val de Loire, France
| | - Achille Aouba
- Department of Internal Medicine, CHU Caen, Caen, Normandie, France
| | - Nadine Magy-Bertrand
- Department of Internal Medicine, Centre Hospitalier Universitaire de Besancon, Besancon, Bourgogne-Franche-Comté, France
| | - Viviane Queyrel
- Internal Medicine, CHU Nice, Nice, Provence-Alpes-Côte d'Azu, France
| | - Alain Le Quellec
- Service de Médecine Interne, CHU de Montpellier, Montpellier, Occitanie, France
| | - Pierre Kieffer
- Service de médecine interne, GHR Mulhouse Sud Alsace, Mulhouse, Grand Est, France
| | - Zahir Amoura
- Sorbonne Université, Inserm, Centre d'Immunologie et des Maladies Infectieuses, Hopital Universitaire Pitie-Salpetriere, Paris, Île-de-France, France
| | - Brigitte Granel
- Internal Medicine Department, Assistance Publique - Hopitaux de Marseille, Marseille, Provence-Alpes-Côte d'Azu, France
| | - Jean Baptiste Gaultier
- Service de Médecine Interne, Centre Hospitalier Universitaire de Saint-Etienne, Saint-Etienne, Auvergne-Rhône-Alpes, France
| | | | - Denis Wahl
- Vascular Medicine and Center for autoimmune diseases, Nancy University Hospital Center, Nancy, Grand Est, France
| | - Olivier Lidove
- Department of Internal Medicine, Groupe hospitalier Diaconesses Croix Saint-Simon, Paris, Île-de-France, France
| | - Olivier Espitia
- Departement of internal and vascular medicine, CHU Nantes, Nantes, Pays de la Loire, France
| | - Ariel Cohen
- Service de cardiologie, Hopital Saint-Antoine, Paris, Île-de-France, France
| | - Olivier Fain
- Sorbonne Université, APHP, Service de Médecine Interne, Hopital Saint-Antoine, Paris, Île-de-France, France
| | - Eric Hachulla
- Department of Internal Medicine and Clinical immunology, Referral Centre for Rare Systemic Auto-immune Diseases North and North-West of France, Univ. Lille, Inserm, CHU de Lille, Lille, Hauts-de-France, France
| | - Arsène Mekinian
- Sorbonne Université, APHP, Service de Médecine Interne, Hopital Saint-Antoine, Paris, Île-de-France, France
| | - Sébastien Rivière
- Sorbonne Université, APHP, Service de Médecine Interne, Hopital Saint-Antoine, Paris, Île-de-France, France
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Fairley JL, Ross L, Nikpour M. Heart involvement in systemic sclerosis: emerging concepts. Curr Opin Rheumatol 2024; 36:393-400. [PMID: 39120541 DOI: 10.1097/bor.0000000000001038] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/10/2024]
Abstract
PURPOSE OF REVIEW Systemic sclerosis (SSc)-associated heart involvement (SHI) is a significant cause of both morbidity and mortality in individuals with SSc. SHI can take many different forms, and likely is a spectrum of fibroinflammatory cardiac disease. Presenting features include arrhythmia, ventricular systolic or diastolic dysfunction, pericardial disease, and exercise intolerance. Risk of sudden cardiac death in SSc is likely 10-30-fold greater than general population estimates. In this review, we explore what is known about the pathogenesis of SHI, its prevention and management, and discuss available strategies for screening for SHI in light of new recommendations for the routine screening of SHI in all SSc patients. RECENT FINDINGS We describe the spectrum, clinical features, and pathogenesis of SHI. Furthermore, we review the new recommendations for screening for SHI in individuals with SSc. SUMMARY There is a large, under-recognized burden of SHI in people living with SSc, which likely contributes to the significant increase in sudden cardiac death observed in SSc. However, a broad-based screening approach, including asymptomatic, low-risk patients should be viewed with caution given the lack of evidence-based treatments and interventions for SHI particularly in this group.
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Affiliation(s)
- Jessica L Fairley
- The University of Melbourne
- St. Vincent's Hospital Melbourne, Melbourne, Victoria
| | - Laura Ross
- The University of Melbourne
- St. Vincent's Hospital Melbourne, Melbourne, Victoria
| | - Mandana Nikpour
- The University of Melbourne
- St. Vincent's Hospital Melbourne, Melbourne, Victoria
- The University of Sydney School of Public Health, Sydney
- Royal Prince Alfred Hospital Sydney
- SydneyMSK Research Flagship Centre, University of Sydney, Sydney, New South Wales, Australia
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Batani V, Dagna L, De Luca G. Therapeutic strategies for primary heart involvement in systemic sclerosis. RHEUMATOLOGY AND IMMUNOLOGY RESEARCH 2024; 5:72-82. [PMID: 39015843 PMCID: PMC11248560 DOI: 10.1515/rir-2024-0010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 04/01/2024] [Accepted: 06/11/2024] [Indexed: 07/18/2024]
Abstract
Primary heart involvement (pHI) is frequent in systemic sclerosis (SSc), even though often underdiagnosed. SSc-pHI has been recently defined as cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications. SSc-pHI represents a major determinant of mortality in SSc, accounting alone for about 12% of disease-related deaths; its early recognition and promptly therapeutic interventions are therefore crucial. Both perfusion defects and myocardial inflammation contribute to the occurrence of myocardial fibrosis that precipitates myocardial remodeling, potentially leading to heart failure and arrhythmic complications. To date, clear evidence and guidelines for effectively managing SSc pHI are not established yet, resulting in a lack of a defined therapeutic algorithm. In this review we summarize the most recent scientific literature on the prevailing therapeutic strategies and interventions to manage SSc-pHI, with particular focus on therapeutic strategies to counteract the 3 major pathogenic events of the disease, i.e. microvascular damage, myocardial inflammation and myocardial fibrosis.
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Affiliation(s)
- Veronica Batani
- Vita-Salute San Raffaele University, Milan, Italy
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy
| | - Lorenzo Dagna
- Vita-Salute San Raffaele University, Milan, Italy
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy
| | - Giacomo De Luca
- Vita-Salute San Raffaele University, Milan, Italy
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy
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Fairley JL, Hansen D, Proudman S, Sahhar J, Ngian GS, Walker J, Host LV, La Gerche A, Prior D, Burns A, Morrisroe K, Stevens W, Nikpour M, Ross L. Prognostic and functional importance of both overt and subclinical left ventricular systolic dysfunction in systemic sclerosis. Semin Arthritis Rheum 2024; 66:152443. [PMID: 38631275 DOI: 10.1016/j.semarthrit.2024.152443] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/29/2023] [Revised: 03/13/2024] [Accepted: 03/22/2024] [Indexed: 04/19/2024]
Abstract
OBJECTIVES To quantify the frequency and clinical implications of systemic sclerosis (SSc)-associated left ventricular function (LV) impairment. METHODS Australian Scleroderma Cohort Study participants meeting ACR/EULAR criteria for SSc with ≥1 echocardiographic LVEF measurement were included. Overt LV dysfunction was indicated by reduced LV ejection fraction (LVEF) and subclinical LV dysfunction was measured using impaired LV global longitudinal strain (LV-GLS>-16 %). Those with secondary causes of LV dysfunction (myocardial ischaemia, valvulopathy and pulmonary arterial hypertension) were excluded. Chi-squared tests, two-sample t-tests or Wilcoxon rank-sum tests were used for between-group comparison as appropriate. Generalised estimating equations(GEE) were used to model longitudinal data. Kaplan-Meier and Cox proportional hazard models were used for survival analyses. RESULTS Of 1141 participants with no co-morbid cardiac disease, 2.4 % ever recorded a LVEF<50 %, while only 0.6 % ever recorded a LVEF≤40 %. LV-GLS data were available for 90 % of participants at one centre (n = 218). Impaired LV-GLS was detected in 21 % despite LVEF≥50 %. Those with a LVEF<50 % were more frequently male (p = 0.01) with dcSSc (p < 0.01), higher inflammatory markers (p < 0.02) and skeletal muscle disease (p < 0.05). In multivariable analyses, recording a LVEF<50 % was associated with increased mortality (HR2.3, 95 %CI1.0-4.8, p = 0.04). Impaired LV-GLS was also associated with poorer survival in univariable analyses (HR3.4, 95 %CI1.0-11.8, p = 0.05). Those with a LVEF<50 % more frequently recorded WHO Class III/IV dyspnoea (OR3.5, 95 %CI1.6-7.7, p < 0.01), with shorter six-minute walk distance (p = 0.01), higher Health Assessment Questionnaire-Disability Index scores (p < 0.01) and lower Short Form-36 Physical Component Summary scores (p = 0.02). Increased dyspnoea (WHO Class III/IV dyspnoea; OR3.6, 95 %CI1.4-9.2, p < 0.01) was also seen in those with impaired LV-GLS. CONCLUSIONS Both overt and subclinical SSc-associated LV dysfunction are associated with worse survival and impaired physical function. The frequency of abnormal LV-GLS in those with consistently normal LVEF suggests an under-appreciated burden of subtle LV systolic dysfunction in SSc that has a significant impact on patient symptomatology.
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Affiliation(s)
- Jessica L Fairley
- Department of Medicine, The University of Melbourne, Melbourne, Victoria, Australia; Department of Rheumatology, St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia.
| | - Dylan Hansen
- Department of Rheumatology, St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
| | - Susanna Proudman
- Department of Medicine, The University of Adelaide, Adelaide, South Australia, Australia; Department of Rheumatology, Royal Adelaide Hospital, Adelaide, South Australia, Australia
| | - Joanne Sahhar
- Department of Rheumatology, Monash Health, Melbourne, Victoria, Australia; Department of Medicine, Monash University, Melbourne, Victoria, Australia
| | - Gene-Siew Ngian
- Department of Rheumatology, Monash Health, Melbourne, Victoria, Australia; Department of Medicine, Monash University, Melbourne, Victoria, Australia
| | - Jenny Walker
- Department of Rheumatology, Royal Adelaide Hospital, Adelaide, South Australia, Australia
| | - Lauren V Host
- Fiona Stanley Hospital, Perth, Western Australia, Australia
| | - André La Gerche
- Department of Medicine, The University of Melbourne, Melbourne, Victoria, Australia; Department of Cardiology, St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia; The Baker Heart and Diabetes Institute, Melbourne, Victoria, Australia
| | - David Prior
- Department of Cardiology, St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
| | - Andrew Burns
- Department of Cardiology, St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
| | - Kathleen Morrisroe
- Department of Medicine, The University of Melbourne, Melbourne, Victoria, Australia; Department of Rheumatology, St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
| | - Wendy Stevens
- Department of Rheumatology, St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
| | - Mandana Nikpour
- Department of Medicine, The University of Melbourne, Melbourne, Victoria, Australia; Department of Rheumatology, St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia; The University of Sydney School of Public Health, Sydney, New South Wales, Australia; Royal Prince Alfred Hospital Sydney, New South Wales, Australia
| | - Laura Ross
- Department of Medicine, The University of Melbourne, Melbourne, Victoria, Australia; Department of Rheumatology, St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
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9
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Gargani L, Bruni C, Todiere G, Pugliese NR, Bandini G, Bellando-Randone S, Guiducci S, D’Angelo G, Campochiaro C, De Luca G, Stagnaro C, Lombardi M, Dagna L, Pepe A, Allanore Y, Moggi-Pignone A, Matucci-Cerinic M. Digital Ulcers and Ventricular Arrhythmias as Red Flags to Predict Replacement Myocardial Fibrosis in Systemic Sclerosis. J Clin Med 2023; 13:89. [PMID: 38202095 PMCID: PMC10779804 DOI: 10.3390/jcm13010089] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2023] [Revised: 12/19/2023] [Accepted: 12/20/2023] [Indexed: 01/12/2024] Open
Abstract
BACKGROUND Cardiac involvement in systemic sclerosis (SSc) affects the prognosis of the disease. Echocardiography is the first line imaging tool to detect cardiac involvement, but it is not able to routinely detect myocardial fibrosis. Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) is the gold standard for replacement myocardial fibrosis assessment, but its availability is currently limited. AIM We aimed to assess the clinical and instrumental parameters that would be useful for predicting the presence of LGE-CMR, to achieve a better selection of patients with SSc that could benefit from third-level CMR imaging. METHODS 344 SSc patients underwent a comprehensive echocardiogram and LGE-CMR on the same day; for 189 patients, a 24 h ECG Holter monitoring was available. RESULTS CMR showed non-junctional replacement myocardial fibrosis via LGE in 25.1% patients. A history of digital ulcers (OR 2.188; 95% C.I. 1.069-4.481) and ventricular arrhythmias at ECG Holter monitoring (OR 3.086; 95% C.I. 1.191-7.998) were independent predictors of replacement myocardial fibrosis. CONCLUSIONS CMR can detect patterns of clinical and subclinical cardiac involvement, which are frequent in SSc. A history of digital ulcers and evidence of ventricular arrhythmias at ECG Holter monitoring are red flags for the presence of replacement myocardial fibrosis in CMR. The association between digital ulcers and myocardial fibrosis suggests that a similar pathological substrate of abnormal vascular function may underlie peripheral vascular and cardiac complications.
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Affiliation(s)
- Luna Gargani
- Department of Surgical, Medical and Molecular Pathology and Critical Care Medicine, University of Pisa, 56126 Pisa, Italy
| | - Cosimo Bruni
- Division of Rheumatology, Department of Experimental and Clinical Medicine, Careggi University Hospital, University of Florence, 50121 Florence, Italy
| | - Giancarlo Todiere
- U.O.C. Risonanza Magnetica Specialistica, Fondazione Toscana G. Monasterio, 56124 Pisa, Italy
| | | | - Giulia Bandini
- Internal Medicine Unit, Department of Clinical and Experimental Medicine, University of Florence, 50121 Florence, Italy
| | - Silvia Bellando-Randone
- Division of Rheumatology, Department of Experimental and Clinical Medicine, Careggi University Hospital, University of Florence, 50121 Florence, Italy
| | - Serena Guiducci
- Division of Rheumatology, Department of Experimental and Clinical Medicine, Careggi University Hospital, University of Florence, 50121 Florence, Italy
| | - Gennaro D’Angelo
- U.O.C. Risonanza Magnetica Specialistica, Fondazione Toscana G. Monasterio, 56124 Pisa, Italy
| | - Corrado Campochiaro
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy
| | - Giacomo De Luca
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy
| | - Chiara Stagnaro
- Department of Rheumatology, Azienda Ospedaliero-Universitaria Pisana, 56126 Pisa, Italy
| | - Massimo Lombardi
- Multimodality Cardiac Imaging Section, Policlinico San Donato, 20097 Milan, Italy
| | - Lorenzo Dagna
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy
| | - Alessia Pepe
- Institute of Radiology, Department of Medicine, University of Padua, 35122 Padua, Italy
| | - Yannick Allanore
- French National Institute of Health and Medical Research (INSERM) U1016, Université de Paris, Hôpital Cochin, 75014 Paris, France
| | - Alberto Moggi-Pignone
- Internal Medicine Unit, Department of Clinical and Experimental Medicine, University of Florence, 50121 Florence, Italy
| | - Marco Matucci-Cerinic
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy
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10
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Wang X, Butcher SC, Myagmardorj R, Liem SIE, Delgado V, Bax JJ, De Vries-Bouwstra JK, Marsan NA. Epicardial adipose tissue in patients with systemic sclerosis. EUROPEAN HEART JOURNAL. IMAGING METHODS AND PRACTICE 2023; 1:qyad037. [PMID: 39045073 PMCID: PMC11195713 DOI: 10.1093/ehjimp/qyad037] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 08/02/2023] [Accepted: 10/11/2023] [Indexed: 07/25/2024]
Abstract
Aims Epicardial adipose tissue (EAT) has emerged as a mediator between systemic inflammatory disorders and cardiovascular disease, and may therefore play a role in the pathophysiology of cardiac involvement in systemic sclerosis (SSc). The aim of this study was to assess the correlation between EAT and left ventricular (LV) function, and to determine the prognostic value of EAT in patients with SSc. Methods and results Consecutive patients with SSc who underwent non-contrast thorax computed tomography and echocardiography were included. EAT mass was quantified using dedicated software. The study endpoint was all-cause mortality. A total of 230 SSc patients (age 53 ± 15 years, 14% male) were included. The median value of EAT mass was 67 g (interquartile range: 45-101 g). Patients with increased EAT mass (≥67 g) showed more impaired LV diastolic function as compared with patients with less EAT mass (<67 g), and even after adjusting for age and comorbidities, EAT mass was independently associated with LV diastolic function parameters. During a median follow-up of 8 years, 42 deaths occurred. Kaplan-Meier analysis showed that patients with increased EAT mass had higher all-cause mortality rate as compared with patients with less EAT mass (29% vs. 7%; P < 0.001). In the multivariable analysis, EAT was independently associated with all-cause mortality after adjusting for important covariates (HR: 1.006; 95% CI: 1.001-1.010). Conclusion In patients with SSc, EAT is independently associated with LV diastolic dysfunction and higher mortality rate.
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Affiliation(s)
- Xu Wang
- Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, The Netherlands
- Department of Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart Lung and Blood Vessel Disease, XCF3+6R6, Chaoyang, Beijing 100029, China
| | - Steele C Butcher
- Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, The Netherlands
- Department of Cardiology, Royal Perth Hospital, Victoria Square, Perth WA 6000, Western Australia, Australia
| | - Rinchyenkhand Myagmardorj
- Department of Cardiology, Mongolia-Japan Teaching Hospital, Mongolian National University of Medical Sciences, Botanic street, Ulaanbaatar 13270, Mongolia
| | - Sophie I E Liem
- Department of Rheumatology, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, The Netherlands
| | - Victoria Delgado
- Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, The Netherlands
- Hospital University Germans Trias i Pujol, Fundació Institut d’Investigació en Ciències de la Salut Germans Trias i Pujol, Carretera de Canyet, s/n, Badalona 08916, Spain
| | - Jeroen J Bax
- Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, The Netherlands
- Heart Center, University of Turku and Turku University Hospital, U-sairaala, Kiinamyllynkatu 4-8, Turku 20521, Finland
| | - Jeska K De Vries-Bouwstra
- Department of Cardiology, Mongolia-Japan Teaching Hospital, Mongolian National University of Medical Sciences, Botanic street, Ulaanbaatar 13270, Mongolia
| | - Nina Ajmone Marsan
- Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, The Netherlands
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11
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Purevsuren M, Uehara M, Ishizuka M, Suzuki Y, Shimbo M, Kakuda N, Ishii S, Sumida H, Miyazaki M, Yamashita T, Yoshizaki A, Asano Y, Sato S, Hatano M, Komuro I. Native T1 mapping in early diffuse and limited systemic sclerosis, and its association with diastolic function. J Cardiol 2023; 82:100-107. [PMID: 36921691 DOI: 10.1016/j.jjcc.2023.03.003] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/16/2022] [Revised: 03/03/2023] [Accepted: 03/07/2023] [Indexed: 03/18/2023]
Abstract
BACKGROUND Systemic sclerosis (SSc) is divided into diffuse and limited cutaneous SSc (dcSSc and lcSSc). The dcSSc subtype has more severe internal organ damage. This study aimed to assess whether cardiovascular magnetic resonance (CMR) parametric mapping could detect early cardiac involvement and evaluate differences between these two subtypes. METHODS Eighty SSc patients (37 dcSSc and 43 lcSSc) underwent CMR at 3.0 T (Philips Healthcare, Best, The Netherlands) in our hospital between July 2018 and July 2021. We analyzed myocardial damage by CMR parametric mapping and compared it with clinical data. RESULTS The median duration of the disease was 10.2 months. The left ventricular ejection fraction was preserved in both groups. DcSSc had significantly higher native T1 (1333.4 ± 71.2 ms vs. 1295.0 ± 42.7 ms, p = 0.006) and extracellular volume fraction (32.6 ± 4.1 % vs. 30.3 ± 4.0 %, p = 0.018) in the mid-ventricular septum as compared to lcSSc, although there were no differences in T2 values. Native T1 values were positively correlated with the E/e' ratio and left atrial volume indices evaluated by transthoracic echocardiography in overall SSc and dcSSc, but not in lcSSc. Logistic regression analysis revealed that native T1 was an independent predictor of left ventricular diastolic dysfunction in SSc patients (odds ratio, 1.194; 95 % confidence interval, 1.021-1.396; p = 0.026). Native T1 was higher in SSc patients with progressive skin lesions. Additionally, there were positive correlations between brain natriuretic peptide, New York Heart Association functional classification, and native T1. CONCLUSIONS CMR parametric mapping is a useful tool for detecting myocardial changes. Native T1 was the most sensitive parameter for identifying diffuse myocardial changes in the early stages of SSc and was associated with left ventricular diastolic function. DcSSc had more severe myocardial involvement than lcSSc; therefore, the use of CMR parametric mapping may aid in its prediction.
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Affiliation(s)
- Munkhtuul Purevsuren
- Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Masae Uehara
- Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
| | - Masato Ishizuka
- Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Yuichi Suzuki
- Radiology Center, The University of Tokyo Hospital, Tokyo, Japan
| | - Mai Shimbo
- Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan; Department of Computational Diagnostic Radiology and Preventive Medicine, The University of Tokyo Hospital, Tokyo, Japan
| | - Nobutaka Kakuda
- Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Satoshi Ishii
- Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Hayakazu Sumida
- Department of Dermatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Miki Miyazaki
- Department of Dermatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Takashi Yamashita
- Department of Dermatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Ayumi Yoshizaki
- Department of Dermatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Yoshihide Asano
- Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan
| | - Shinichi Sato
- Department of Dermatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Masaru Hatano
- Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan; Department of Advanced Medical Center for Heart Failure, The University of Tokyo Hospital, Tokyo, Japan
| | - Issei Komuro
- Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
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12
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Yan W, Luo Q, Nie Q, Wang H, Wu J. Association between systemic sclerosis and left ventricle dysfunction: Findings from observational studies. Heliyon 2023; 9:e14110. [PMID: 36938434 PMCID: PMC10020007 DOI: 10.1016/j.heliyon.2023.e14110] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/19/2022] [Revised: 02/22/2023] [Accepted: 02/22/2023] [Indexed: 03/06/2023] Open
Abstract
Objectives Cardiac involvement is common in systemic sclerosis (SSc) patients. In this study, we aimed to systematically evaluate the relationship between SSc and left ventricular dysfunction (LVD), especially the left ventricular diastolic dysfunction, by ultrasound and cardiac magnetic resonance data. Methods We searched The Cochrane Library, PubMed and Embase databases collected studies about comparing LVD parameters in SSc patients and controls from establishment to January 2022. Furthermore, we also performed a two-sample MR using summary statistics from genome-wide association studies (GWAS) important LVD parameters, including left ventricular end-diastolic volume (LVEDV), left ventricular mass (LVM) and left ventricular ejection fraction (LVEF). Results Our meta-analysis included 31 eligible studies with 1448 SSc patients. According to the results, SSc patients had lower peak of early diastolic flow velocity/peak of late diastolic flow velocity ratio (E/A ratio), E, trans-mitral early filling peak velocity (E'), and left ventricular end-diastolic diameter (LVEDD) compared to controls. The E/E' ratio, A, left ventricular isovolumetric relaxation time (IVRT), deceleration Time (DT) and left atrial (LA) diameter were higher in SSc patients in comparison with controls. Moreover, we observed that the SSc patients had lower LVEF than controls. And in MR analysis, we also found that SSc was causally correlated with LVEF (OR = 0.9966, 95% CI 0.9935-0.998, P = 0.0398). However, unfortunately, there was no significant correlation between SSC and LVM (OR = 1.0048, 95% CI 0.9919-1.0179, P = 0.4661) and LVEDV (LVEDV OR = 0.9976, 95%CI 0.9888-1.0066, P = 0.6019). Conclusion SSc patients had diastolic/systolic dysfunction. However, MR analysis cannot confirm the genetic relationship between SSc and LVDD because of insufficient data. More research is needed to confirm the causal relationship between the two.
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Affiliation(s)
- Wei Yan
- Southwest Jiaotong University, Department of Geriatrics, Southwest Jiaotong University College of Medicine, The Third People's Hospital of Chengdu, No.82, Qinglong Street, Sichuan, China
| | - Qiang Luo
- Department of Cardiology, The Affiliated Hospital of Southwest Jiaotong University, The Third People's Hospital of Chengdu, No.82, Qinglong Street, Sichuan, China
| | - Qiong Nie
- Southwest Jiaotong University, Department of Geriatrics, Southwest Jiaotong University College of Medicine, The Third People's Hospital of Chengdu, No.82, Qinglong Street, Sichuan, China
| | - Han Wang
- Department of Cardiology, The Affiliated Hospital of Southwest Jiaotong University, The Third People's Hospital of Chengdu, No.82, Qinglong Street, Sichuan, China
- Corresponding author.
| | - Jing Wu
- Southwest Jiaotong University, Department of Geriatrics, Southwest Jiaotong University College of Medicine, The Third People's Hospital of Chengdu, No.82, Qinglong Street, Sichuan, China
- Corresponding author.
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13
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López Núñez L, Carrión-Barberà I, Molina L, Padró I, Ciria M, Salman-Monte TC, Pros A. Left ventricular dysfunction and arrhythmias in asymptomatic patients with systemic sclerosis. Med Clin (Barc) 2023; 160:434-442. [PMID: 36813685 DOI: 10.1016/j.medcli.2022.11.022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2022] [Revised: 11/15/2022] [Accepted: 11/17/2022] [Indexed: 02/22/2023]
Abstract
INTRODUCTION AND AIMS Cardiac involvement in systemic sclerosis (SS) is frequently silent and a major cause of mortality in these patients. This work aims to study the prevalence and associations of left ventricular dysfunction (LVD) and arrhythmias in SS. METHODS AND RESULTS Prospective study of SS patients (n=36), excluding those with symptoms of (or) cardiac disease, pulmonary arterial hypertension or cardiovascular risk factors (CVRF). A clinical, analytical, electrocardiogram (EKG), Holter, and echocardiogram with global longitudinal strain (GLS) assessment were performed. Arrhythmias were classified into clinically significant arrhythmias (CSA) and non-significant. Twenty-eight percent had left ventricular diastolic dysfunction (LVDD), 22% LV systolic dysfunction (LVSD) according to the GLS, 11.1% both, and 16.7% cardiac dysautonomia. Fifty percent presented alterations by EKG (44% CSA), 55.6% by Holter (75% CSA) and 8.3% CSA by both. An association was found between the elevation of troponin T (TnTc) and CSA and between the elevation of both NT-proBNP and TnTc with LVDD. CONCLUSIONS We found a higher prevalence of LVSD than in the literature, detected by GLS and being 10 times higher than that detected by LVEF, which justifies the need to incorporate this technique in the routine evaluation of these patients. The association of TnTc and NT-proBNP with LVDD suggests that they can be used as minimally invasive biomarkers of this affectation. The absence of correlation between LVD and CSA indicates that the arrhythmias could be due, not only to a supposed structural alteration of the myocardium, but to an independent and early cardiac involvement, which should be actively investigated even in asymptomatic patients without CVRF.
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Affiliation(s)
- Lilian López Núñez
- Rheumatology Department, Hospital del Mar/Parc de Salut Mar, Barcelona, Spain
| | | | - Luis Molina
- Cardiology Department, Hospital del Mar/Parc de Salut Mar, Barcelona, Spain
| | - Isabel Padró
- Rheumatology Department, Hospital del Mar/Parc de Salut Mar, Barcelona, Spain
| | - Manel Ciria
- Rheumatology Department, Hospital del Mar/Parc de Salut Mar, Barcelona, Spain
| | | | - Ana Pros
- Rheumatology Department, Hospital del Mar/Parc de Salut Mar, Barcelona, Spain
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14
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Oliveira MC, Koenigkam-Santos M, Schmidt A. Editorial for "Diastolic Function Assessment of Left and Right Ventricles by MRI in Systemic Sclerosis Patients". J Magn Reson Imaging 2022; 56:1427-1428. [PMID: 35307910 DOI: 10.1002/jmri.28162] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2022] [Revised: 03/05/2022] [Accepted: 03/08/2022] [Indexed: 12/15/2022] Open
Affiliation(s)
- Maria Carolina Oliveira
- Division of Clinical Immunology, Department of Internal Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil
| | - Marcel Koenigkam-Santos
- Department of Medical Images, Hematology and Oncology, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil
| | - André Schmidt
- Division of Cardiology, Department of Internal Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil
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15
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Yoshimi R, Nakajima H. The Treatment of Systemic Sclerosis-related Pericarditis. Intern Med 2022; 61:2997-2998. [PMID: 35283394 PMCID: PMC9646345 DOI: 10.2169/internalmedicine.9471-22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Affiliation(s)
- Ryusuke Yoshimi
- Department of Hematology and Clinical Immunology, Yokohama City University School of Medicine, Japan
| | - Hideaki Nakajima
- Department of Hematology and Clinical Immunology, Yokohama City University School of Medicine, Japan
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16
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Ferlito A, Campochiaro C, Tomelleri A, Dagna L, De Luca G. Primary heart involvement in systemic sclerosis, from conventional to innovative targeted therapeutic strategies. JOURNAL OF SCLERODERMA AND RELATED DISORDERS 2022; 7:179-188. [PMID: 36211207 PMCID: PMC9537702 DOI: 10.1177/23971983221083772] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/03/2022] [Accepted: 02/08/2022] [Indexed: 10/03/2023]
Abstract
Primary heart involvement is frequent in systemic sclerosis, even though often sub-clinical, and includes cardiac abnormalities that are predominantly attributable to systemic sclerosis rather than other causes and/or complications. A timely diagnosis is crucial to promptly start the appropriate therapy and to prevent the potential life-threatening early and late complications. There is little evidence on how to best manage systemic sclerosis-primary heart involvement as no specific treatment recommendations for heart disease are available, and a shared treatment approach is still lacking. The objective of this review is to summarize the state of the art of current literature and the overall management strategies and therapeutic approaches for systemic sclerosis-primary heart involvement. Novel insights into pathogenic mechanisms of systemic sclerosis-primary heart involvement are presented to facilitate the comprehension of therapeutic targets and novel treatment strategies.
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Affiliation(s)
| | - Corrado Campochiaro
- Vita-Salute San Raffaele University,
Milan, Italy
- Unit of Immunology, Rheumatology,
Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy
| | - Alessandro Tomelleri
- Vita-Salute San Raffaele University,
Milan, Italy
- Unit of Immunology, Rheumatology,
Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy
| | - Lorenzo Dagna
- Vita-Salute San Raffaele University,
Milan, Italy
- Unit of Immunology, Rheumatology,
Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy
| | - Giacomo De Luca
- Vita-Salute San Raffaele University,
Milan, Italy
- Unit of Immunology, Rheumatology,
Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy
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17
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Glynn P, Hale S, Hussain T, Freed BH. Cardiovascular Imaging for Systemic Sclerosis Monitoring and Management. Front Cardiovasc Med 2022; 9:846213. [PMID: 35433887 PMCID: PMC9008238 DOI: 10.3389/fcvm.2022.846213] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2021] [Accepted: 03/08/2022] [Indexed: 11/23/2022] Open
Abstract
Systemic sclerosis (SSc) is a complex connective tissue disease with multiple clinical and subclinical cardiac manifestations. SSc can affect most structural components of the heart, including the pericardium, myocardium, valves, and conduction system through a damaging cycle of inflammation, ischemia, and fibrosis. While cardiac involvement is the second leading SSc-related cause of death, it is frequently clinically silent in early disease and often missed with routine screening. To facilitate identification of cardiac disease in this susceptible population, we present here a review of cardiac imaging modalities and potential uses in the SSc patient population. We describe well-characterized techniques including electrocardiography and 2D echocardiography with Doppler, but also discuss more advanced imaging approaches, such as speckle-tracking echocardiography, cardiovascular magnetic resonance imaging (CMR), and stress imaging, among others. We also suggest an algorithm for the appropriate application of these modalities in the workup and management of patients with SSc. Finally, we discuss future opportunities for cardiac imaging in SSc research to achieve early detection and to optimize treatment.
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Affiliation(s)
- Peter Glynn
- Division of Cardiology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, United States
| | - Sarah Hale
- Division of Cardiology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, United States
| | - Tasmeen Hussain
- Division of Hospital Medicine, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, United States
| | - Benjamin H. Freed
- Division of Cardiology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, United States
- *Correspondence: Benjamin H. Freed,
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18
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Sclerodermic Cardiomyopathy—A State-of-the-Art Review. Diagnostics (Basel) 2022; 12:diagnostics12030669. [PMID: 35328222 PMCID: PMC8947572 DOI: 10.3390/diagnostics12030669] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/08/2022] [Revised: 02/27/2022] [Accepted: 03/07/2022] [Indexed: 12/12/2022] Open
Abstract
Systemic sclerosis (SSc) is a chronic autoimmune disorder with unknown triggering factors, and complex pathophysiologic links which lead to fibrosis of skin and internal organs, including the heart, lungs, and gut. However, more than 100 years after the first description of cardiac disease in SSc, sclerodermic cardiomyopathy (SScCmp) is an underrecognized, occult disease with important adverse long-term prognosis. Laboratory tests, electrocardiography (ECG) and cardiovascular multimodality imaging techniques (transthoracic 2D and 3D echocardiography, cardiac magnetic resonance (CMR), and novel imaging techniques, including myocardial deformation analysis) provide new insights into the cardiac abnormalities in patients with SSc. This state-of-the-art review aims to stratify all the cardiac investigations needed to diagnose and follow-up the SScCmp, and discusses the epidemiology, risk factors and pathophysiology of this important cause of morbidity of the SSc patient.
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19
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Zakariaei Z, Sharifpour A, Fakhar M, Soleymani M, Banimostafavi ES, Taheri A. Detection of Lophomonas in pericardial effusion sample in a COVID-19 patient with systemic sclerosis: An unusual case report. SAGE Open Med Case Rep 2022; 10:2050313X221102021. [PMID: 35651516 PMCID: PMC9149606 DOI: 10.1177/2050313x221102021] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/02/2022] [Accepted: 04/29/2022] [Indexed: 11/23/2022] Open
Abstract
Systemic sclerosis is a connective tissue disorder that involves the skin and
many other organs, such as the heart, skin, and gastrointestinal tract. Cardiac
involvement is in the form of pericarditis, pericardial effusion, and pulmonary
hypertension. Several complications and super infections post-COVID-19 have been
reported, such as fungal, bacterial infections, and Lophomonas
blattarum. Lophomoniasis is an emerging pulmonary infection that
mainly involves the lower respiratory tract. Herein, we present an ectopic
Lophomonas infection in an unusual location (pericardial
effusion) in a COVID-19 patient who had systemic sclerosis.
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Affiliation(s)
- Zakaria Zakariaei
- Toxoplasmosis Research Center, Communicable Diseases Institute, Iranian National Registry Center for Lophomoniasis (INRCL), Mazandaran University of Medical Sciences, Sari, Iran
- Toxicology and Forensic Medicine Division, Orthopedic Research Center, Imam Khomeini Hospital, Mazandaran University of Medical Sciences, Sari, Iran
| | - Ali Sharifpour
- Toxoplasmosis Research Center, Communicable Diseases Institute, Iranian National Registry Center for Lophomoniasis (INRCL), Mazandaran University of Medical Sciences, Sari, Iran
- Pulmonary and Critical Care Division, Imam Khomeini Hospital, Mazandaran University of Medical Sciences, Sari, Iran
| | - Mahdi Fakhar
- Toxoplasmosis Research Center, Communicable Diseases Institute, Iranian National Registry Center for Lophomoniasis (INRCL), Mazandaran University of Medical Sciences, Sari, Iran
| | - Mostafa Soleymani
- Toxoplasmosis Research Center, Communicable Diseases Institute, Iranian National Registry Center for Lophomoniasis (INRCL), Mazandaran University of Medical Sciences, Sari, Iran
| | - Elham Sadat Banimostafavi
- Toxoplasmosis Research Center, Communicable Diseases Institute, Iranian National Registry Center for Lophomoniasis (INRCL), Mazandaran University of Medical Sciences, Sari, Iran
- Department of Radiology, Imam Khomeini Hospital, Mazandaran University of Medical Sciences, Sari, Iran
| | - Amirmasoud Taheri
- Student Research Committee, Mazandaran University of Medical Sciences, Sari, Iran
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Colaci M, Schinocca C, Bosco YD, Ronsivalle G, Guggino G, de Andres I, Russo AA, Sambataro D, Sambataro G, Malatino L. Heart Valve Abnormalities in Systemic Sclerosis Patients: A Multicenter Cohort Study and Review of the Literature. J Clin Rheumatol 2022; 28:e95-e101. [PMID: 33252390 DOI: 10.1097/rhu.0000000000001638] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
BACKGROUND Systemic sclerosis (SSc) is a chronic autoimmune disease that is characterized by vasculopathy and fibrosis of the skin and visceral organs. Heart valve diseases are poorly described and generally not considered typical of SSc. We aimed to describe valvular abnormalities in a multicenter cohort of SSc patients and to investigate their correlation with SSc features. METHODS We recruited 118 consecutive SSc patients (male/female, 14/104; mean age, 55.2 ± 12.1 years) in 3 rheumatology centers in Sicily, Italy, from January to October 2019. RESULTS Mitral and tricuspid valve insufficiency was found in 85% and 91% of patients, respectively; regurgitations were generally mild and never severe. Mitral stenosis was rare (2%), and tricuspid stenosis was not observed. Sclerosis and calcification were present in 30% of mitral valves and in only 4% of tricuspid valves. The aortic valve was affected in 25% of cases, and it generally presented as regurgitation or sclerosis, whereas stenosis was rare (3%). Finally, 11% of SSc patients showed regurgitation of the pulmonary valve. No specific associations between SSc features and valve alterations were found. CONCLUSIONS Valvular diseases are frequently observed in SSc patients, with a predominant pattern of valvular regurgitations. Therefore, echocardiography should be routinely performed during SSc patient follow-up, considering the potential influence of additional cardiac involvement in the prognosis of these patients.
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Affiliation(s)
- Michele Colaci
- From the Rheumatology Centre, Internal Medicine Unit, Department of Clinical and Experimental Medicine, c/o Azienda Ospedaliera per l'Emergenza Cannizzaro, University of Catania, Catania
| | - Claudia Schinocca
- From the Rheumatology Centre, Internal Medicine Unit, Department of Clinical and Experimental Medicine, c/o Azienda Ospedaliera per l'Emergenza Cannizzaro, University of Catania, Catania
| | - Ylenia Dal Bosco
- From the Rheumatology Centre, Internal Medicine Unit, Department of Clinical and Experimental Medicine, c/o Azienda Ospedaliera per l'Emergenza Cannizzaro, University of Catania, Catania
| | | | - Giuliana Guggino
- Rheumatology Unit, Policlinico "P. Giaccone", Università di Palermo, Palermo
| | - Ilenia de Andres
- Rheumatology Unit, Azienda Ospedaliera di Rilievo Nazionale ed Alta Specializzazione "Garibaldi"
| | - Alessandra A Russo
- Rheumatology Unit, Azienda Ospedaliera di Rilievo Nazionale ed Alta Specializzazione "Garibaldi"
| | - Domenico Sambataro
- From the Rheumatology Centre, Internal Medicine Unit, Department of Clinical and Experimental Medicine, c/o Azienda Ospedaliera per l'Emergenza Cannizzaro, University of Catania, Catania
| | - Gianluca Sambataro
- Artroreuma srl, Outpatient Clinic of Rheumatology associated with the National Health System
| | - Lorenzo Malatino
- Internal Medicine Unit, Department of Clinical and Experimental Medicine, c/o Azienda Ospedaliera per l'Emergenza Cannizzaro, University of Catania, Catania, Italy
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Kumar K, Seetharam K, Poonam F, Gulati A, Sadiq A, Shetty V. The Role of Cardiac Imaging in the Evaluation of Cardiac Involvement in Systemic Diseases. Cureus 2021; 13:e20708. [PMID: 35106243 PMCID: PMC8788898 DOI: 10.7759/cureus.20708] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/23/2021] [Indexed: 11/05/2022] Open
Abstract
For systemic diseases like rheumatoid arthritis, systemic lupus erythematosus (SLE), systemic sclerosis, systemic vasculitis, myopathies, and mixed connective tissue diseases, cardiac disease is a major contributing factor for morbidity and mortality. The cardiovascular manifestations are the result of various pathophysiological components, which complicate management. Furthermore, the signs and symptoms can be subtle and missed due to the complex nature of the underlying condition. As a result, various imaging approaches play an imperative role in diagnosis and prognosis. The evolving role of these modalities could lead to risk stratification and improved therapies in the future. In conclusion, our review article will highlight the role of cardiac imaging in the evaluation of cardiac involvement for systemic diseases.
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Zagouras AA, Chatterjee S, Tang WHW. Heart Failure with Preserved Ejection Fraction and Cardiomyopathy: an Under-recognized Complication of Systemic Sclerosis. CURRENT TREATMENT OPTIONS IN CARDIOVASCULAR MEDICINE 2021. [DOI: 10.1007/s11936-021-00947-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
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Peverill RE, Ngian GS, Mylrea C, Sahhar J. Determinants of left ventricular structure, filling and long axis function in systemic sclerosis. PLoS One 2021; 16:e0258593. [PMID: 34679117 PMCID: PMC8535357 DOI: 10.1371/journal.pone.0258593] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/04/2021] [Accepted: 09/30/2021] [Indexed: 11/23/2022] Open
Abstract
Background Abnormalities of left ventricular (LV) structure, filling and long-axis function have all been reported in subjects with systemic sclerosis (SSc) and a normal LV ejection fraction (EF), but previous study findings have not been consistent. The aim of this study was to identify factors which could have confounded the analyses in previous studies of SSc, and in particular to consider the variables of body surface area (BSA), sex, age, heart rate, blood pressure (BP), disease duration (DD), disease type (limited versus diffuse) and interstitial lung disease (ILD). Methods Echocardiography was performed on 100 subjects with SSc (79 women; age 56±15 years) with a LVEF ≥50% and free of pulmonary arterial hypertension, coronary artery disease, more than mild valvular heart disease and atrial fibrillation. Measurements were performed of the LV end-diastolic dimension (LVEDD) and septal wall thickness (SWT), the transmitral Doppler E, A and deceleration time (DT), and the peak systolic (s’) and early diastolic (e’) LV long-axis velocities. Multivariate analyses were performed to investigate correlations of the above LV variables with BSA, sex, age, heart rate, BP, DD, disease type, and the presence of ILD. Results DD varied between 0.1 and 41.2 years, 25% had diffuse and 75% had limited disease, and 37% had ILD. SWT and LVEDD were positively correlated with BSA, SWT was also positively correlated with age and larger in males, and LVEDD was larger in diffuse disease. Age was positively correlated with A and DT, and inversely correlated with E and E/A, and heart rate was inversely correlated with E and E/A. None of E, A, E/A, or DT were independently associated with DD or disease type. Septal and lateral LV wall s’ and e’ were all inversely correlated with age, and there was a small independent contribution to the prediction of lateral s’ from DD, but no association of either s’ or e’ with disease type. The presence of ILD was not a predictor of any of the LV variables. Conclusion In SSc there are associations of sex, body size, age and disease type with LV structural variables, of age and heart rate with E/A, and of age with both systolic and early diastolic LV long-axis velocities. Appropriate adjustment for these variables could help to resolve current uncertainties regarding SSc effects on the left ventricle.
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Affiliation(s)
- Roger E. Peverill
- Department of Medicine (School of Clinical Sciences at Monash Health), Monash Cardiovascular Research Centre, Monash Heart, Monash University and Monash Health, Clayton, Victoria, Australia
- * E-mail:
| | - Gene-Siew Ngian
- Rheumatology Department, Monash Health, Monash University, Clayton, Victoria, Australia
- Department of Medicine (School of Clinical Sciences at Monash Health), Monash University, Clayton, Victoria, Australia
| | - Catherine Mylrea
- Department of Medicine (School of Clinical Sciences at Monash Health), Monash Cardiovascular Research Centre, Monash Heart, Monash University and Monash Health, Clayton, Victoria, Australia
| | - Joanne Sahhar
- Rheumatology Department, Monash Health, Monash University, Clayton, Victoria, Australia
- Department of Medicine (School of Clinical Sciences at Monash Health), Monash University, Clayton, Victoria, Australia
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Prevalence and risk factors for left ventricular diastolic dysfunction in systemic sclerosis: a multi-center study of CRDC cohort in China. Clin Rheumatol 2021; 40:4589-4596. [PMID: 34142296 DOI: 10.1007/s10067-021-05804-6] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/08/2021] [Revised: 05/22/2021] [Accepted: 05/30/2021] [Indexed: 10/21/2022]
Abstract
OBJECTIVE Left ventricular diastolic dysfunction (LVDD) is a common manifestation of cardiac involvement in systemic sclerosis (SSc), which is associated with increased mortality, but little is known about the risk factors. The aim is to determine the frequency and potential predictors of SSc-LVDD. METHODS We conducted a prospective multi-center cohort study, enrolling 784 SSc patients assessed by echocardiography between April 2008 and June 2019. Diagnosis of systemic sclerosis was according to the 2013 American College of Rheumatology (ACR)/the European League Against Rheumatism (EULAR) classification criteria. Data were compared between patients with and without LVDD, while univariate and multivariate regression analysis was performed to determine the factors independently associated with LVDD. RESULTS LV diastolic dysfunction was present in 246/784 (31.4%) of the subjects. There were no significant differences in gender, BMI, or disease duration between the two groups. Around 40% of the patients in the SSc-LVDD group and in the SSc-non LVDD group had diffused cutaneous involvements. Factors independently associated with LV diastolic dysfunction in multivariable analysis included age at onset (OR 1.053, 95%CI 1.021-1.086, p = 0.001), pulmonary arterial hypertension (OR 3.057, 95%CI 1.468-6.367, p = 0.003), positivity of anti-RNP antibody (OR 2.455, 95%CI 1.049-5.745, p = 0.038), increased WBC count (OR 1.156, 95%CI 1.037-1.287, p = 0.009), elevated levels of uric acid (OR 1.003, 95%CI 1.000-1.006, p = 0.036), and triglyceride (OR 1.515, 95%CI 1.106-2.077, p = 0.010). CONCLUSION LV diastolic dysfunction was prevalent in the SSc population. Advanced onset age, PAH, positive anti-RNP antibody, increased WBC count, and adverse metabolic status were independent risk factors for SSc-related LVDD. Key Points • In this Chinese multi-center cohort of systemic sclerosis, LVDD is not a rare complication, with a prevalence of 31.4%. • The presence of advanced onset age, PAH, positive anti-RNP antibody, increased WBC count and adverse metabolic status were baseline predictors of developing LVDD in SSc.
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Cardiac involvement in systemic sclerosis: Getting to the heart of the matter. Best Pract Res Clin Rheumatol 2021; 35:101668. [PMID: 33736950 DOI: 10.1016/j.berh.2021.101668] [Citation(s) in RCA: 38] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Primary systemic sclerosis heart involvement (pSHI) is an important disease manifestation that accounts for a significant proportion of systemic sclerosis (SSc)-associated mortality. A broad clinical spectrum of pSHI exists, which ranges from asymptomatic perfusion abnormalities to diastolic dysfunction or acute myocarditis and congestive heart failure. With improving sensitivity of cardiac investigations, it is increasingly recognized that there is a large burden of subclinical cardiac disease in patients with SSc. Early signs of pSHI can be subtle and determining the etiology of cardiac abnormalities from other causes of cardiomyopathy such as hypertension, ischemic heart disease (IHD), and pulmonary vascular disease remain challenging. Early identification of pSHI potentially provides clinicians with a window of opportunity for intervention to avert progression to heart failure. However, optimal screening and treatment guidelines are lacking, and it is an area of much needed further clinical research.
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Narváez J, LLuch J, Ruiz-Majoral A, Sánchez-Corral MA, Claver E, Nolla JM. Increased Prevalence of Moderate to Severe Mitral and Aortic Valve Dysfunction in Systemic Sclerosis: A Case-control Study. J Rheumatol 2020; 48:394-401. [PMID: 33191275 DOI: 10.3899/jrheum.201025] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/02/2020] [Indexed: 01/31/2023]
Abstract
OBJECTIVE To investigate the prevalence, severity, and associated clinical factors of mitral and aortic valvular involvement in patients with systemic sclerosis (SSc). METHODS Our case-control study included 172 patients with SSc and 172 non-SSc adults without known cardiac disease matched by age, sex, and prevalence of cardiovascular (CV) risk factors. The screening of mitral and aortic valvular involvement was performed by transthoracic Doppler echocardiogram. The prevalence of aortic stenosis (AS) was also compared with that reported in a population-based study performed in our community during the same period. RESULTS Patients with SSc showed an almost 5-fold increased prevalence of moderate to severe mitroaortic valve dysfunction compared to non-SSc controls (OR 4.60, 95% CI 1.51-13.98; P = 0.003). The most common lesion was mitral regurgitation (MR), which was observed in 5.2% of patients, followed by AS in 3.5%, and aortic regurgitation (AR) in 1.7%. Analyzing the different types of valvular lesion separately, we observed a significantly higher frequency of MR compared to controls (OR 4.69, 95% CI 1.12-22.04; P = 0.032), as well as a higher frequency of AS in the 65-75 (OR 7.51, 95% CI 1.22-46.23, P = 0.01) and 76-85 age groups (OR 3.53, 95% CI 1.03-12.22, P = 0.043) when compared to the general population in our community. CONCLUSION We found an increased prevalence of moderate to severe MR and AS in SSc compared to age-matched non-SSc controls with similar CV comorbidities. While results from this study do not allow for establishing a direct causal relationship, they strongly support the contribution of SSc-specific factors in the development of these complications.
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Affiliation(s)
- Javier Narváez
- J. Narváez, MD, PhD, J. LLuch, MD, J.M. Nolla, MD, PhD, Department of Rheumatology, Hospital Universitario de Bellvitge;
| | - Judit LLuch
- J. Narváez, MD, PhD, J. LLuch, MD, J.M. Nolla, MD, PhD, Department of Rheumatology, Hospital Universitario de Bellvitge
| | - Alejandro Ruiz-Majoral
- A. Ruiz-Majoral, MD, M.A. Sánchez-Corral, MD, E. Claver, MD, Department of Cardiology, Hospital Universitario de Bellvitge, Barcelona, Spain
| | - Miguel Angel Sánchez-Corral
- A. Ruiz-Majoral, MD, M.A. Sánchez-Corral, MD, E. Claver, MD, Department of Cardiology, Hospital Universitario de Bellvitge, Barcelona, Spain
| | - Eduard Claver
- A. Ruiz-Majoral, MD, M.A. Sánchez-Corral, MD, E. Claver, MD, Department of Cardiology, Hospital Universitario de Bellvitge, Barcelona, Spain
| | - Joan M Nolla
- J. Narváez, MD, PhD, J. LLuch, MD, J.M. Nolla, MD, PhD, Department of Rheumatology, Hospital Universitario de Bellvitge
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Makavos G, Varoudi M, Papangelopoulou K, Kapniari E, Plotas P, Ikonomidis I, Papadavid E. Echocardiography in Autoimmune Rheumatic Diseases for Diagnosis and Prognosis of Cardiovascular Complications. MEDICINA (KAUNAS, LITHUANIA) 2020; 56:medicina56090445. [PMID: 32883041 PMCID: PMC7558642 DOI: 10.3390/medicina56090445] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/28/2020] [Revised: 08/30/2020] [Accepted: 08/31/2020] [Indexed: 12/23/2022]
Abstract
Autoimmune rheumatic diseases are systemic diseases frequently affecting the heart and vessels. The main cardiovascular complications are pericarditis, myocarditis, valvular disease, obstructive coronary artery disease and coronary microcirculatory dysfunction, cardiac failure and pulmonary hypertension. Echocardiography, including transthoracic two and three-dimensional echocardiography, Doppler imaging, myocardial deformation and transesophageal echo, is an established and widely available imaging technique for the identification of cardiovascular manifestations that are crucial for prognosis in rheumatic diseases. Echocardiography is also important for monitoring the impact of drug treatment on cardiac function, coronary microcirculatory function, valvular function and pulmonary artery pressures. In this article we summarize established and evolving knowledge on the role of echocardiography for diagnosis and prognosis of cardiovascular abnormalities in rheumatic diseases.
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Affiliation(s)
- George Makavos
- Second Cardiology Department, Attikon Hospital, Medical School, National and Kapodistrian University of Athens, 12462 Athens, Greece; (M.V.); (K.P.); (P.P.); (I.I.)
- Correspondence: ; Tel.: +30-210-5832187
| | - Maria Varoudi
- Second Cardiology Department, Attikon Hospital, Medical School, National and Kapodistrian University of Athens, 12462 Athens, Greece; (M.V.); (K.P.); (P.P.); (I.I.)
| | - Konstantina Papangelopoulou
- Second Cardiology Department, Attikon Hospital, Medical School, National and Kapodistrian University of Athens, 12462 Athens, Greece; (M.V.); (K.P.); (P.P.); (I.I.)
| | - Eirini Kapniari
- Second Department of Dermatology and Venereology, Attikon Hospital, Medical School, National and Kapodistrian University of Athens, 12462 Athens, Greece; (E.K.); (E.P.)
| | - Panagiotis Plotas
- Second Cardiology Department, Attikon Hospital, Medical School, National and Kapodistrian University of Athens, 12462 Athens, Greece; (M.V.); (K.P.); (P.P.); (I.I.)
| | - Ignatios Ikonomidis
- Second Cardiology Department, Attikon Hospital, Medical School, National and Kapodistrian University of Athens, 12462 Athens, Greece; (M.V.); (K.P.); (P.P.); (I.I.)
| | - Evangelia Papadavid
- Second Department of Dermatology and Venereology, Attikon Hospital, Medical School, National and Kapodistrian University of Athens, 12462 Athens, Greece; (E.K.); (E.P.)
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Tipparot T, Foocharoen C, Mahakkanukrauh A, Suwannaroj S, Nanagara R, Pussadhamma B, Chaosuwannakit N. Clinical and laboratory predictions of myocardial inflammation as detected by cardiac magnetic resonance imaging in patients with systemic sclerosis: A pilot study. Int J Rheum Dis 2019; 22:2125-2133. [PMID: 31659856 DOI: 10.1111/1756-185x.13727] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/11/2019] [Revised: 09/25/2019] [Accepted: 09/26/2019] [Indexed: 12/12/2022]
Abstract
BACKGROUND Cardiac magnetic resonance imaging (cardiac MRI) has high sensitivity and specificity for differentiating cardiac fibrosis from inflammation. There is no data on the clinical and laboratory association or prediction of myocardial inflammation in systemic sclerosis-a major organ involvement in systemic sclerosis (SSc). OBJECTIVES Our aim was to ascertain the clinical and laboratory associations with myocardial inflammation in SSc patients as detected by cardiac MRI. METHODS A cross-sectional study was conducted among Thai adult SSc patients who had: disease onset <4 years; a New York Heart Association functional class ≥ II; and followed up at the Scleroderma Clinic, Khon Kaen University, between June 2018 and January 2019. We excluded patients who were taking steroids and/or immunosuppressants or had a diagnosed heart disease before being diagnosed with SSc. All enrolled patients underwent cardiac MRI, and clinical and laboratory assessments the same date. Myocardial inflammation was defined by cardiac MRI per the Lake Louise Criteria. RESULTS A total of 30 SSc patients were enrolled. The female-to-male ratio was 1.8:1. The majority (73%) had diffuse cutaneous SSc. The respective mean age and median duration of disease was 57 ± 8 and 2.0 years (interquartile range 1.5-2.7). Myocardial inflammation was detected in 22 patients (73.3%). The multivariate analysis revealed that every 5 years of increased age at onset and every 5-point increase in the modified Rodnan skin score (mRSS) at onset was significantly associated with myocardial inflammation (odds ratio 0.47, 95% CI 0.22-0.98; and 2.64 95% CI 1.04-6.74, respectively). Neither the SSc subset, internal organ involvement, inflammatory markers, nor cardiac and muscle enzymes were associated with myocardial inflammation in SSc. CONCLUSION Myocardial inflammation is common in early-onset SSc. An increased risk of myocardial inflammation was associated with young age and high mRSS at onset. Cardiac MRI is the suggested evaluation for high-risk SSc patients experiencing dyspnea on exertion.
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Affiliation(s)
- Thapanee Tipparot
- Division of Rheumatology, Department of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Chingching Foocharoen
- Division of Rheumatology, Department of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Ajanee Mahakkanukrauh
- Division of Rheumatology, Department of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Siraphop Suwannaroj
- Division of Rheumatology, Department of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Ratanavadee Nanagara
- Division of Rheumatology, Department of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Burabha Pussadhamma
- Division of Cardiology, Department of Medicine, Khon Kaen University, Khon Kaen, Thailand
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29
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Arbeláez-Cortés Á, Quintero-González DC, Cuesta-Astroz Y, Villadiego JS, González-Buriticá H, Rueda JM. Restrictive cardiomyopathy in a patient with systemic sclerosis and Fabry disease: a case-based review. Rheumatol Int 2019; 40:489-497. [PMID: 31599343 DOI: 10.1007/s00296-019-04453-y] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2019] [Accepted: 09/26/2019] [Indexed: 12/29/2022]
Abstract
Systemic sclerosis (SSc) is a rare immune-mediated vasculopathy characterized by fibrosis of the skin and internal organs. Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene producing α-galactosidase-A enzyme (α-Gal A) deficiency. Being a systemic disease, cardiac involvement in FD has a high mortality rate due to heart failure and arrhythmia. The coexistence of these two entities has not been reported previously. We describe the case of a female patient with limited SSc (lcSSc), a diagnosis based on the presence of sclerodactyly, Raynaud phenomenon, microvascular involvement, and positive anti-centromere antibodies. On follow-up, she developed chest pain, a second-degree A-V block, and restrictive cardiomyopathy (without cardiovascular risk factors). Although heart involvement is common in these two entities, the abnormal thickening of lateral and inferior wall, the infiltration pattern and the conduction system disorders presented herein are more characteristic in a heterozygous female with a cardiac variant of FD. The diagnosis of FD was confirmed with high globotriaosylsphingosine (Lyso-Gb3) levels and identification of GLA gene mutation. The patient was treated with enzymatic replacement (agalsidase alpha) following mild improvement in ventricular mass at 6th month, without clinical deterioration. The related literature on SSc associated with FD is also reviewed.
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Affiliation(s)
- Álvaro Arbeláez-Cortés
- Internal Medicine, Universidad Libre, Cali, Colombia. .,Arthritis and Rheumatology Clinic, Centro Médico Imbanaco, Cali, Colombia.
| | | | - Yesid Cuesta-Astroz
- School of Microbiology, Universidad de Antioquia, Medellín, Colombia.,Instituto Colombiano de Medicina Tropical, Universidad CES, Sabaneta, Colombia
| | | | - Herman González-Buriticá
- Internal Medicine, Universidad Libre, Cali, Colombia.,Arthritis and Rheumatology Clinic, Centro Médico Imbanaco, Cali, Colombia
| | - Jorge M Rueda
- Internal Medicine, Universidad Libre, Cali, Colombia.,Arthritis and Rheumatology Clinic, Centro Médico Imbanaco, Cali, Colombia
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Tennøe AH, Murbræch K, Andreassen JC, Fretheim H, Garen T, Gude E, Andreassen A, Aakhus S, Molberg Ø, Hoffmann-Vold AM. Left Ventricular Diastolic Dysfunction Predicts Mortality in Patients With Systemic Sclerosis. J Am Coll Cardiol 2019; 72:1804-1813. [PMID: 30286924 DOI: 10.1016/j.jacc.2018.07.068] [Citation(s) in RCA: 68] [Impact Index Per Article: 11.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/02/2018] [Accepted: 07/03/2018] [Indexed: 12/11/2022]
Abstract
BACKGROUND Primary cardiac affection is common and is a major cause of death in systemic sclerosis (SSc), but there are knowledge gaps regarding the effect of cardiac dysfunction on mortality. OBJECTIVES The purpose of this study was to evaluate diastolic function in a large, unselected SSc cohort and assess the effect of diastolic dysfunction (DD) on mortality. METHODS SSc patients followed prospectively at the Oslo University Hospital from 2003 to 2016 with available echocardiographies and matched control subjects were included. DD was assessed by echocardiography according to the 2016 American Society of Echocardiography/European Association of Cardiovascular Imaging guidelines. Pulmonary hypertension (PH) was diagnosed by right heart catheterization. Vital status was available for all patients. Cox regression analyses with hazards ratios (HRs) were conducted. RESULTS Diastolic function was assessed in 275 SSc patients at baseline and in 186 patients at follow-up. At baseline, 46 of the 275 SSc patients (17%) were diagnosed with DD and 195 (71%) had normal diastolic function. After a median follow-up of 3.4 years (interquartile range: 1.6 to 6.2 years), the proportion of DD increased from 17% to 29%. During follow-up, 57% of patients with DD at baseline died, compared with 13% of patients with normal diastolic function. At baseline, 86 patients had performed right heart catheterization, and 43 were diagnosed with PH; of these 60% deceased. In multivariable Cox regression analyses, DD was a stronger predictor of death (HR: 3.7; 95% CI: 1.69 to 8.14; c-index 0.89) than PH (HR: 2.0; 95% CI: 1.1 to 3.9; c-index 0.84). CONCLUSIONS DD is frequent in SSc, and the presence of DD is associated with high mortality. DD exceeds PH with respect to predicting mortality.
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Affiliation(s)
- Anders H Tennøe
- Department of Rheumatology, Oslo University Hospital, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Oslo, Norway
| | - Klaus Murbræch
- Department of Cardiology, Oslo University Hospital, Oslo, Norway
| | | | - Håvard Fretheim
- Department of Rheumatology, Oslo University Hospital, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Oslo, Norway
| | - Torhild Garen
- Department of Rheumatology, Oslo University Hospital, Oslo, Norway
| | - Einar Gude
- Department of Cardiology, Oslo University Hospital, Oslo, Norway
| | - Arne Andreassen
- Department of Cardiology, Oslo University Hospital, Oslo, Norway
| | - Svend Aakhus
- Department of Circulation and Imaging, Faculty of Medicine and Health Sciences, NTNU, Norwegian University of Science and Technology, Trondheim, Norway; Clinic of Cardiology, St. Olav's Hospital, Trondheim, Norway
| | - Øyvind Molberg
- Department of Rheumatology, Oslo University Hospital, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Oslo, Norway
| | - Anna-Maria Hoffmann-Vold
- Department of Rheumatology, Oslo University Hospital, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
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Butt SA, Jeppesen JL, Torp-Pedersen C, Sam F, Gislason GH, Jacobsen S, Andersson C. Cardiovascular Manifestations of Systemic Sclerosis: A Danish Nationwide Cohort Study. J Am Heart Assoc 2019; 8:e013405. [PMID: 31446827 PMCID: PMC6755829 DOI: 10.1161/jaha.119.013405] [Citation(s) in RCA: 67] [Impact Index Per Article: 11.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
Background Cardiovascular involvement in systemic sclerosis (SSc) comprises a wide range of manifestations with prevalence and incidence that remain uncertain. Methods and Results In the Danish administrative registries between 1995 and 2015, all patients aged ≥18 years with a first diagnosis of SSc were matched by age and sex with controls (1:5) from the general population. Prevalence of cardiovascular diseases at the time of the SSc diagnosis and incidence during follow‐up were assessed by in‐ and outpatient discharge diagnoses. Conditional logistic and Cox proportional hazards regression models were used respectively to calculate odds ratios for prevalent cardiovascular diseases and hazard ratios (HRs) for incident diseases associated with SSc. Patients with SSc (n=2778; 76% women; mean±SD age: 55±15 years) had more established cardiovascular risk factors than their respective controls at baseline, including greater prevalence of hypertension (31.2% versus 21.0%, P<0.0001) and treated dyslipidemia (9.8% versus 8.5%, P=0.02). SSc was associated with an increased relative risk of developing most cardiovascular diseases, including myocardial infarction (HR: 2.08; 95% CI, 1.65–2.64), peripheral vascular disease (HR: 5.73; 95% CI, 4.63–7.09), pulmonary hypertension (HR: 21.18; 95% CI, 14.73–30.45), mitral regurgitation (HR: 4.60; 95% CI, 3.12–6.79), aortic regurgitation (HR: 3.78; 95% CI, 2.55–5.58), aortic stenosis (HR: 2.99; 95% CI, 2.25–3.97), pericarditis (HR: 8.78; 95% CI, 4.84–15.93), heart failure (HR: 2.86; 95% CI, 2.43–3.37), atrial fibrillation (HR: 1.75; 95% CI, 1.51–2.04), and venous thromboembolism (HR: 2.10; 95% CI, 1.65–2.67). Additional adjustment for medications and comorbidities yielded results similar to the main analyses. Conclusions In this nationwide study, SSc was associated with greater risks of distinct cardiovascular diseases for patients than for matched controls, suggesting a significant disease‐related adverse impact across the vascular bed and specific cardiac structures.
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Affiliation(s)
- Sheraz A Butt
- Department of Internal Medicine and Cardiology Amager Hvidovre Hospital Glostrup Denmark
| | - Jørgen L Jeppesen
- Department of Internal Medicine and Cardiology Amager Hvidovre Hospital Glostrup Denmark
| | | | - Flora Sam
- Whitaker Cardiovascular Institute and Department of Cardiology Boston University School of Medicine Boston MA
| | - Gunnar H Gislason
- Department of Cardiology Herlev and Gentofte Hospital Gentofte Denmark
| | - Søren Jacobsen
- Copenhagen Lupus and Vasculitis Clinic Rigshospitalet Copenhagen Denmark
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Marques-Alves P, Baptista R, Canha C, Franco F, Santos L, Pêgo M. Early manifestation of myocardial involvement in systemic sclerosis. Rev Port Cardiol 2019; 38:299-303. [PMID: 31203919 DOI: 10.1016/j.repc.2017.07.022] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/04/2017] [Revised: 07/09/2017] [Accepted: 07/24/2017] [Indexed: 01/04/2023] Open
Abstract
INTRODUCTION Systemic sclerosis (SSc) is a systemic autoimmune disease involving multiple organs. We present a rare case of SSc in which clinical manifestations of cardiac fibrosis occurred early in the disease course. CASE REPORT We report the case of a 40-year-old Caucasian man, previously diagnosed with SSc, who presented with decompensated heart failure. Transthoracic echocardiography was remarkable for severe right ventricular systolic dysfunction, abnormal ventricular septal motion, severe functional tricuspid regurgitation and normal pulmonary artery systolic pressure. Left ventricular ejection fraction was 45%. Right heart catheterization revealed no signs of pulmonary hypertension. Cardiac magnetic resonance (CMR) showed diffuse myocardial infiltration, later confirmed as myocardial fibrosis by endomyocardial biopsy. CONCLUSIONS Myocardial fibrosis is an important cause of early heart failure in SSc patients and is associated with poor prognosis. Echocardiography and CMR help establish the diagnosis and enable an appropriate therapeutic strategy to be developed in such cases.
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Affiliation(s)
- Patrícia Marques-Alves
- Department of Cardiology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
| | - Rui Baptista
- Department of Cardiology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal; CNC.IBILI Research Consortium, University of Coimbra, Coimbra, Portugal
| | - Catarina Canha
- Department of Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
| | - Fátima Franco
- Department of Cardiology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
| | - Lèlita Santos
- Department of Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
| | - Mariano Pêgo
- Department of Cardiology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
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Karadag DT, Sahin T, Tekeoglu S, Işik OO, Yazici A, Eraldemir FC, Cefle A. Evaluation of left and right ventricle by two-dimensional speckle tracking echocardiography in systemic sclerosis patients without overt cardiac disease. Clin Rheumatol 2019; 39:37-48. [PMID: 31127462 DOI: 10.1007/s10067-019-04604-3] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/26/2019] [Revised: 05/09/2019] [Accepted: 05/12/2019] [Indexed: 01/10/2023]
Abstract
BACKGROUND/OBJECTIVE The aim was to evaluate the left and right ventricular functions concurrently by two-dimensional speckle tracking echocardiography (STE) in systemic sclerosis (SSc) patients without overt cardiac disease. METHODS A total of 47 patients with SSc and 36 age- and sex-matched controls were evaluated cross-sectionally. Two-dimensional STE was used to assess the longitudinal peak systolic strains (PSS) of both ventricles including apical long-axis (APLAX), apical four-chamber (4-CH), apical two-chamber (2-CH), and global longitudinal measurements. Any association of metabolic, cardiac, and inflammatory biomarkers with PSS was investigated. RESULTS The longitudinal PSS of the left ventricle [APLAX, 4-CH, 2-CH and global] were significantly lower in SSc patients than controls (- 18.2 ± 3.2 vs - 19.8 ± 2.7% p = 0.02; - 17.8 ± 3.5 vs. - 20.3 ± 3.3% p = 0.001; - 18.6 ± 3.1 vs. - 21.8 ± 3% p < 0.001; - 17.5 ± 5.7 vs. - 20.6 ± 2.7% p = 0.003, respectively). No difference was found between the groups for right ventricular strains. The longitudinal PSS-4CH correlated positively with CRP and ESR (r = 0.349, p = 0.016; r = 0.356, p = 0.014, respectively) and negatively with serum Galectin-3 (r = - 0.362, p = 0.012). Global longitudinal PSS-left ventricle (LV) correlated positively with CRP and homocysteine (r = 0.297, p = 0.043; r = 0.313, p = 0.041, respectively) and negatively with serum Galectin-3 (r = -0.314, p = 0.041). After multivariable adjustment, CRP remained the only predictor of longitudinal PSS-4CH (95% CI 0.35, 0.70, p = 0.028) and global longitudinal PSS of left ventricle (95% CI 0.004, 0.22, p = 0.043). CONCLUSIONS Biventricular evaluation of patients with SSc by two dimensional STE revealed reduced left ventricular longitudinal strains, despite preserved right ventricular strain, and no diastolic dysfunction. In SSc without overt cardiac disease, global cardiac assessment with 2DSTE is a promising method which seems to contribute to the detection of patients without clinical findings. KEY POINTS • Two dimensional STE revealed reduced left ventricular longitudinal strains, despite preserved right ventricular strain in SSc patients without overt cardiac disease. • CRP was the predictor of decreased longitudinal strains. • Cardiac assessment in SSc should be made globally.
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Affiliation(s)
- Duygu Temiz Karadag
- Division of Rheumatology, Department of Internal Medicine, Kocaeli University School of Medicine, Kocaeli, Turkey.
| | - Tayfun Sahin
- Division of Cardiology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey
| | - Senem Tekeoglu
- Division of Rheumatology, Department of Internal Medicine, Kocaeli University School of Medicine, Kocaeli, Turkey
| | - Ozlem Ozdemir Işik
- Division of Rheumatology, Department of Internal Medicine, Kocaeli University School of Medicine, Kocaeli, Turkey
| | - Ayten Yazici
- Division of Rheumatology, Department of Internal Medicine, Kocaeli University School of Medicine, Kocaeli, Turkey
| | | | - Ayse Cefle
- Division of Rheumatology, Department of Internal Medicine, Kocaeli University School of Medicine, Kocaeli, Turkey
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Early manifestation of myocardial involvement in systemic sclerosis. REVISTA PORTUGUESA DE CARDIOLOGIA (ENGLISH EDITION) 2019. [DOI: 10.1016/j.repce.2019.05.018] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
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Ross L, Prior D, Proudman S, Vacca A, Baron M, Nikpour M. Defining primary systemic sclerosis heart involvement: A scoping literature review. Semin Arthritis Rheum 2019; 48:874-887. [PMID: 30170705 DOI: 10.1016/j.semarthrit.2018.07.008] [Citation(s) in RCA: 39] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/15/2018] [Revised: 07/09/2018] [Accepted: 07/20/2018] [Indexed: 12/20/2022]
Abstract
BACKGROUND Clinically evident primary heart involvement due to systemic sclerosis (SHI) is considered a poor prognostic factor and is a leading cause of systemic sclerosis (SSc) related death. Yet, there remains no consensus definition of SHI and poor understanding of the natural history and risk factors for the development of SHI. METHODS We performed a scoping literature review of published articles with a primary focus of SHI to capture previously used definitions of SHI and items used to measure SHI. Any factors reported to be associated with an increased risk of SHI were recorded. RESULTS Of the 2436 records identified in a search of MEDLINE, EMBASE and PubMed databases, 295 were included in the final scoping review. Analysis of the literature revealed studies of variable quality, generally low patient numbers and highly heterogeneous definitions of SHI within studies. There is no clear consensus from the literature as to the scope of SHI and the prognostic significance of sub-clinical investigation abnormalities commonly detected. CONCLUSION The lack of a standardised definition of SHI remains a significant unmet need in SSc. The results of this review will assist in the development of consensus classification criteria to enable more accurate quantification of the burden of SHI, identification of factors associated with increased risk of developing SHI, and evaluation of the efficacy of any novel therapeutic strategies.
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Affiliation(s)
- Laura Ross
- Department of Medicine at St Vincent's Hospital, University of Melbourne, 41 Victoria Parade, Fitzroy, VIC 3065, Australia; Department of Rheumatology, St Vincent's Hospital, 41 Victoria Parade, Fitzroy, VIC 3065, Australia.
| | - David Prior
- Department of Medicine at St Vincent's Hospital, University of Melbourne, 41 Victoria Parade, Fitzroy, VIC 3065, Australia; Department of Cardiology, St Vincent's Hospital, 41 Victoria Parade, Fitzroy, VIC 3065, Australia.
| | - Susanna Proudman
- Rheumatology Unit, Royal Adelaide Hospital, Port Road, Adelaide, SA 5000, Australia; Discipline of Medicine, University of Adelaide, Adelaide, SA 5000, Australia.
| | - Alessandra Vacca
- Unit of Rheumatology, University Hospital of Cagliari, S.S. 554, bivio per Sestu, 09042 Monserrato (CA), Italy..
| | - Murray Baron
- Division of Rheumatology, Jewish General Hospital, McGill University, 5750 Côtes-des-Neiges Rd, Montreal, QC H3S 1Y9, Canada.
| | - Mandana Nikpour
- Department of Medicine at St Vincent's Hospital, University of Melbourne, 41 Victoria Parade, Fitzroy, VIC 3065, Australia; Department of Rheumatology, St Vincent's Hospital, 41 Victoria Parade, Fitzroy, VIC 3065, Australia.
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Electrocardiographic Findings in Systemic Sclerosis. SERBIAN JOURNAL OF EXPERIMENTAL AND CLINICAL RESEARCH 2018. [DOI: 10.2478/sjecr-2018-0052] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Abstract
Abstract
Systemic sclerosis (SSc) is an autoimmune connective tissue disease which affects various tissues and organs, including skin, lungs, kidneys, gastrointestinal tract and cardiovascular system. Cardiac involvement is the most commonly recognized problem and a significant cause of morbidity. Abnormal ECG is present in 25-75% of patients with SSc and is considered to be an independent predictor of mortality. It is known that the supraventricular arrhythmias are considered as more common in SSc patients, occurring in about two-thirds of the cases, and more often than ventricular tachyarrhythmias. It has been established that right bundle branch block is associated with an increased risk of mortality and that it is an independent predictor of mortality, and should be considered as a marker of the severity of the disease in SSc. Th e prolonged QTc interval is an independent risk factor for a sudden cardiac death reflecting the instability of repolarization and predisposing the onset of cardiac arrhythmias.The prognosis of the disease depends on the SSc subtype and the involvement of internal organs. SSc is a lifelong disease and cannot be cured, but knowing that cardiac dysfunction significantly worsens the prognosis, early detection of cardiac complications and appropriate therapy can influence its progress and improve the patients’ quality of life.
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Impact of Pulmonary Arterial Hypertension on Left Ventricular Function – a Comparative Study between Scleroderma and Coronary Artery Disease. JOURNAL OF INTERDISCIPLINARY MEDICINE 2018. [DOI: 10.2478/jim-2018-0028] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Abstract
Abstract
Background: The impact of pulmonary arterial hypertension (PAH) on left ventricular performance in patients with scleroderma is still unknown. This study aims to perform a comparative echocardiographic analysis of left ventricular function between two different etiological varieties of PAH, namely PAH caused by systemic sclerosis as a representative of systemic inflammatory diseases and PAH caused by myocardial ischemia.
Material and method: We conducted a prospective observational study on 82 patients, of which 36 were with documented PAH, with the systolic pressure in the pulmonary artery above 35 mmHg, and 46 were patients with normal pulmonary artery pressure. The study population was divided into two groups, based on the etiology of PAH: group 1 included patients diagnosed with scleroderma (n = 48); group 2 included patients with coronary artery disease (n = 35). Patients from each group were divided into two subgroups based on the diagnosis of PAH: subgroup 1A – subjects with scleroderma and associated PAH (n = 20); subgroup 1B – subjects with scleroderma without PAH (n = 28); subgroup 2A – ischemic patients with associated PAH (n = 16); and subgroup 2B – patients with ischemic disease without PAH (n = 19).
Results: A significant difference between LVEF values in patients with PAH versus those without PAH in the ischemic group (p = 0.023) was recorded. Compared to scleroderma subjects, ischemic patients presented significantly lower values of LVEF in both PAH and non-PAH subgroups (p <0.0001 and p <0.0001, respectively). Linear regression analysis between sPAP and LVEF revealed a significant negative correlation only for the ischemia group (r = −0.52, p = 0.001) and the scleroderma 2B subgroup (r = −0.51, p = 0.04). Tissue Doppler analysis of left ventricular function revealed a significant impact of PAH on left ventricular diastolic performance in the ischemic group.
Conclusions: Compared to patients with coronary artery disease, those with scleroderma present a less pronounced deterioration of LVEF in response to pulmonary arterial hypertension.
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Habib G, Bucciarelli-Ducci C, Caforio ALP, Cardim N, Charron P, Cosyns B, Dehaene A, Derumeaux G, Donal E, Dweck MR, Edvardsen T, Erba PA, Ernande L, Gaemperli O, Galderisi M, Grapsa J, Jacquier A, Klingel K, Lancellotti P, Neglia D, Pepe A, Perrone-Filardi P, Petersen SE, Plein S, Popescu BA, Reant P, Sade LE, Salaun E, Slart RHJA, Tribouilloy C, Zamorano J. Multimodality Imaging in Restrictive Cardiomyopathies: An EACVI expert consensus document In collaboration with the "Working Group on myocardial and pericardial diseases" of the European Society of Cardiology Endorsed by The Indian Academy of Echocardiography. Eur Heart J Cardiovasc Imaging 2018; 18:1090-1121. [PMID: 28510718 DOI: 10.1093/ehjci/jex034] [Citation(s) in RCA: 73] [Impact Index Per Article: 10.4] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/09/2017] [Accepted: 02/14/2017] [Indexed: 12/11/2022] Open
Abstract
Restrictive cardiomyopathies (RCMs) are a diverse group of myocardial diseases with a wide range of aetiologies, including familial, genetic and acquired diseases and ranging from very rare to relatively frequent cardiac disorders. In all these diseases, imaging techniques play a central role. Advanced imaging techniques provide important novel data on the diagnostic and prognostic assessment of RCMs. This EACVI consensus document provides comprehensive information for the appropriateness of all non-invasive imaging techniques for the diagnosis, prognostic evaluation, and management of patients with RCM.
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Affiliation(s)
- Gilbert Habib
- Aix- Aix-Marseille Univ, URMITE, Aix Marseille Université-UM63, CNRS 7278, IRD 198, INSERM 1095.,Cardiology Department, APHM, La Timone Hospital, Boulevard Jean Moulin, 13005 Marseille, France
| | - Chiara Bucciarelli-Ducci
- Bristol Heart Institute, National Institute of Health Research (NIHR) Bristol Cardiovascular Biomedical Research Unit (BRU), University of Bristol, Bristol, UK
| | - Alida L P Caforio
- Cardiology, Department of Cardiological Thoracic and Vascular Sciences, University of Padova, Italy
| | - Nuno Cardim
- Multimodality Cardiac Imaging Department, Sports Cardiology and Cardiomyopathies Centre-Hospital da Luz; Lisbon, Portugal
| | - Philippe Charron
- Université Versailles Saint Quentin, INSERM U1018, Hôpital Ambroise Paré, Boulogne-Billancourt, France.,Centre de référence pour les maladies cardiaques héréditaires, APHP, ICAN, Hôpital de la Pitié Salpêtrière, Paris, France
| | | | - Aurélie Dehaene
- Department of Radiology and Cardiovascular Imaging, APHM, Hôpitaux de la Timone, Pôle d'imagerie Médicale, 13005 Marseille, France
| | - Genevieve Derumeaux
- Department of Physiology, INSERM U955, Université Paris-Est Creteil, Henri Mondor Hospital, DHU-ATVB, AP-HP, Créteil, France
| | - Erwan Donal
- Cardiologie-CHU Rennes & CIC-IT 1414 & LTSI INSERM 1099 - Université Rennes-1
| | - Marc R Dweck
- Centre for Cardiovascular Science, University of Edinburgh
| | - Thor Edvardsen
- Department of Cardiology, Center for Cardiological Innovation and Institute for Surgical Research, Oslo University Hospital, Oslo, Norway.,University of Oslo, Oslo, Norway
| | - Paola Anna Erba
- Regional Center of Nuclear Medicine, Department of Translational Research and New Technology in Medicine, University of Pisa, Pisa, Italy
| | - Laura Ernande
- Department of Physiology, INSERM U955, Université Paris-Est Creteil, Henri Mondor Hospital, DHU-ATVB, AP-HP, Créteil, France
| | - Oliver Gaemperli
- University Heart Center Zurich, Interventional Cardiology and Cardiac Imaging 19, Zurich
| | - Maurizio Galderisi
- Department of Advanced Biomedical Sciences, Federico II University, Naples, Italy
| | - Julia Grapsa
- Department of Cardiovascular Sciences, Imperial College of London, London, UK
| | - Alexis Jacquier
- Department of Radiology and Cardiovascular Imaging, APHM, Hôpitaux de la Timone, Pôle d'imagerie Médicale, Aix-Marseille Université, CNRS, CRMBM UMR 7339, 13385 Marseille, France
| | - Karin Klingel
- Department of Molecular Pathology, Institute for Pathology and Neuropathology, University Hospital Tuebingen, Tuebingen, Germany
| | - Patrizio Lancellotti
- Departments of Cardiology, Heart Valve Clinic, University of Liège Hospital, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium.,Gruppo Villa Maria Care and Research, Anthea Hospital, Bari, Italy
| | - Danilo Neglia
- Cardiovascular Department, Fondazione Toscana G. Monasterio, CNR Institute of Clinical Physiology, Scuola Superiore Sant'Anna, Pisa, Italy
| | - Alessia Pepe
- Magnetic Resonance Imaging Unit, Fondazione G. Monasterio C.N.R.-Regione Toscana Pisa, Italy
| | | | - Steffen E Petersen
- Department of Advanced Cardiovascular Imaging, William Harvey Research Institute, National Institute for Health Research Cardiovascular Biomedical Research Unit at Barts, London, UK
| | - Sven Plein
- Division of Biomedical Imaging, Multidisciplinary Cardiovascular Research Centre, Leeds Institute of Cardiovascular and Metabolic Medicine LIGHT Laboratories, University of Leeds, UK
| | - Bogdan A Popescu
- University of Medicine and Pharmacy 'Carol Davila'-Euroecolab, Institute of Cardiovascular Diseases, Bucharest, Romania
| | | | | | - Erwan Salaun
- Cardiology Department, La Timone Hospital, Marseille France
| | - Riemer H J A Slart
- Department of Nuclear Medicine and Molecular Imaging, University of Groningen, University Medical Center Groningen, Hanzeplein 1, Groningen, The Netherlands.,Department of Biomedical Photonic Imaging, University of Twente, PO Box 217, 7500 AEEnschede, The Netherlands
| | - Christophe Tribouilloy
- Department of Cardiology, University Hospital Amiens, Amiens, France and INSERM U-1088, Jules Verne University of Picardie, Amiens, France
| | - Jose Zamorano
- University Hospital Ramon y Cajal Carretera de Colmenar Km 9,100, 28034 Madrid, Spain
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Abstract
The heart is one of the major organs commonly involved in systemic sclerosis (SSc). Myocardial fibrosis has been identified in a high percentage of these patients. Most SSc patients with cardiac involvement (CI) are subclinical, especially early on in the course of their disease. To accurately identify CI and improve diagnosis and treatment, imaging techniques should be implemented on a regular basis following diagnosis. In this review, we discuss the up-to-date pathophysiologic basis of CI, the cardiac manifestations, and the diagnostic methods that have been published in the literature. Recent studies have shown that tissue Doppler imaging is a promising evaluation technique in the bedside detection of CI. Cardiovascular magnetic resonance is an operator-independent method used for detecting SSc CI. It is an especially useful tool in the early stages of the disease when patients may be asymptomatic. At present, it is the most promising imaging technique for the diagnosis, follow-up, and response to therapy in clinical practice.
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Guerra F, Stronati G, Fischietti C, Ferrarini A, Zuliani L, Pomponio G, Capucci A, Danieli MG, Gabrielli A. Global longitudinal strain measured by speckle tracking identifies subclinical heart involvement in patients with systemic sclerosis. Eur J Prev Cardiol 2018; 25:1598-1606. [PMID: 29966435 DOI: 10.1177/2047487318786315] [Citation(s) in RCA: 34] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Background Systemic sclerosis is characterised by progressive cutaneous and organ fibrosis. Among all organs, a subclinical heart involvement is difficult to detect through conventional imaging. Design We evaluated whether speckle tracking-derived global longitudinal strain could help detect early subclinical systolic dysfunction in systemic sclerosis patients without overt clinical involvement. Methods A case-control, single-centre study on 52 systemic sclerosis patients and 52 age and gender-matched controls. Patients with structural heart disease, heart failure, atrial fibrillation and pulmonary hypertension were excluded. For every patient, standard echocardiographic and speckle tracking-derived variables for the systolic and diastolic function of the left ventricle and right ventricle were acquired. Results Traditional parameters of left and right systolic function did not differ between systemic sclerosis patients and controls (all P = ns). Left and right ventricular global longitudinal strain was significantly impaired in patients with systemic sclerosis when compared to controls (-19.2% vs. -21.1%; P = 0.009 and -18.2% vs. -22.3%; P = 0.012, respectively). Systemic sclerosis patients had a 2.5-fold increased risk of subclinical left ventricular systolic impairment (odds ratio 2.5, 95% confidence interval 1.1-5.5; P = 0.027) and a 3.3-fold increased risk of subclinical right ventricular systolic impairment when compared to controls (odds ratio 3.3, 95% confidence interval 1.4-7.7; P = 0.004). Alterations in the myocardial deformation pattern of systemic sclerosis patients were homogeneous in the right ventricle and eccentric in the left ventricle. Conclusions While traditional echocardiographic parameters are ineffective in detecting subclinical systolic impairment, reduced global longitudinal strain is common in patients with systemic sclerosis and significant for both ventricles. Global longitudinal strain could become a low-cost, non-invasive and reliable tool in order to detect early cardiac involvement in systemic sclerosis patients.
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Affiliation(s)
- Federico Guerra
- 1 Cardiology and Arrhythmology Clinic, Marche Polytechnic University, Italy
| | - Giulia Stronati
- 1 Cardiology and Arrhythmology Clinic, Marche Polytechnic University, Italy
| | | | | | - Lucia Zuliani
- 2 Clinica Medica, Marche Polytechnic University, Italy
| | | | - Alessandro Capucci
- 1 Cardiology and Arrhythmology Clinic, Marche Polytechnic University, Italy
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Niklas K, Niklas A, Mularek-Kubzdela T, Puszczewicz M. Prevalence of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease. Medicine (Baltimore) 2018; 97:e11437. [PMID: 29995796 PMCID: PMC6076033 DOI: 10.1097/md.0000000000011437] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
Systemic sclerosis (SSc) and mixed connective tissue disease (MCTD) are 2 conditions in which pulmonary hypertension (PH) can develop.We retrospectively analyzed the probability of PH in case of 83 patients (69 SSc and 14 MCTD). The European Society of Cardiology/European Respiratory Society (ESC/ERS) echocardiographic guidelines of 2015 were used for the evaluation.On the basis of an echocardiography, the patients were divided into 2 subgroups: patients with elevated probability of PH (EP) (n = 16) versus the group with a low probability of PH (LP) (n = 67). Of the 16 patients in the EP group, 15 were SSc patients and 1 was an MCTD patient, respectively, that is, 21.7% and 7.1% of all patients. Of the 16 patients with EP, 10 with SSc had right-heart catheterization, which excluded PH in 7 patients; hence, PH was estimated to be 11.6% in the SSc group. The distribution of the individual causes of PH was arterial PH 2.9%, PH associated with interstitial lung disease 4.3%, PH associated with left ventricular disease 1.5%, and PH of unknown origin 2.9%. Further, there was a significant difference between EP and LP in the incidence of the right bundle branch block in standard electrocardiography, left atrial and right ventricular dimension, tricuspid annular plane systolic excursion, and Doppler-derived tricuspid lateral annular systolic velocity (S') in echocardiography.Echocardiography, particularly those evaluating the parameters included in the ESC/ERS guidelines of 2015, appears to be a useful tool in the detection of patients with a high PH probability. Additional tissue Doppler echocardiography seems to be a good option.
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Clinically Symptomatic Pericardial Effusions in Hospitalized Systemic Sclerosis Patients: Demographics and Management. BIOMED RESEARCH INTERNATIONAL 2018; 2018:6812082. [PMID: 29967777 PMCID: PMC6008774 DOI: 10.1155/2018/6812082] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 01/16/2018] [Revised: 03/28/2018] [Accepted: 04/04/2018] [Indexed: 11/24/2022]
Abstract
Background Pericardial effusions in systemic sclerosis (SSc) may present as acute or chronic with or without clinical symptoms. Best treatment is unknown and whether patients receive medical therapy or a surgical procedure is clinician-dependent. Objective To describe the clinical characteristics, treatment, and outcomes of patients with SSc and clinically symptomatic pericardial effusions treated in the inpatient setting. Methods We evaluated all SSc admissions over a 10-year period to a tertiary care hospital which has a dedicated SSc clinic. Patients who had a clinically symptomatic pericardial effusion were evaluated based on their demographics, disease pattern, and medical or surgical management. Results From January 2005 till October 2015, there were 462 SSc admissions with 32 (6.9%) of them being for a clinically symptomatic pericardial effusion in 23 unique patients. Eleven (47%) of these patients had right heart failure, seventeen (74%) had pulmonary arterial hypertension (PAH), and 4 (17%) had tamponade physiology. Five (22%) patients were treated by a surgical procedure, while eighteen (78%) patients had medical therapy. Patients who received medical therapy tended to be older, have a lower serum Cr level, and more likely have right heart failure. Conclusion Clinically symptomatic pericardial effusion is a rare cause for hospital admissions in SSc, with a high percentage of these patients having PAH. Medical therapy tends to be reserved for older patients with right heart failure, while surgical therapy was more likely in patients with higher serum Cr levels.
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Asano Y, Jinnin M, Kawaguchi Y, Kuwana M, Goto D, Sato S, Takehara K, Hatano M, Fujimoto M, Mugii N, Ihn H. Diagnostic criteria, severity classification and guidelines of systemic sclerosis. J Dermatol 2018; 45:633-691. [PMID: 29687465 DOI: 10.1111/1346-8138.14162] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2017] [Accepted: 11/06/2017] [Indexed: 01/17/2023]
Abstract
Several effective drugs have been identified for the treatment of systemic sclerosis (SSc). However, in advanced cases, not only their effectiveness is reduced but they may be also harmful due to their side-effects. Therefore, early diagnosis and early treatment is most important for the treatment of SSc. We established diagnostic criteria for SSc in 2003 and early diagnostic criteria for SSc in 2011, for the purpose of developing evaluation of each organ in SSc. Moreover, in November 2013, the American College of Rheumatology and the European Rheumatology Association jointly developed new diagnostic criteria for increasing their sensitivity and specificity, so we revised our diagnostic criteria and severity classification of SSc. Furthermore, we have revised the clinical guideline based on the newest evidence. In particular, the clinical guideline was established by clinical questions based on evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guideline easy to use and reliable based on the newest evidence, and to present guidance as specific as possible for various clinical problems in treatment of SSc.
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Affiliation(s)
- Yoshihide Asano
- Department of Dermatology, Faculty of Medicine, The University of Tokyo, Tokyo, Japan
| | - Masatoshi Jinnin
- Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
| | - Yasushi Kawaguchi
- Institute of Rheumatology, Tokyo Woman's Medical University, Tokyo, Japan
| | - Masataka Kuwana
- Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan
| | - Daisuke Goto
- Department of Rheumatology, Faculty of Medicine, Univertity of Tsukuba, Ibaraki, Japan
| | - Shinichi Sato
- Department of Dermatology, Faculty of Medicine, The University of Tokyo, Tokyo, Japan
| | - Kazuhiko Takehara
- Department of Molecular Pathology of Skin, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Ishikawa, Japan
| | - Masaru Hatano
- Graduate School of Medicine Department of Therapeutic Strategy for Heart Failure, The University of Tokyo, Tokyo, Japan
| | - Manabu Fujimoto
- Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan
| | - Naoki Mugii
- Section of Rehabilitation, Kanazawa University Hospital, Ishikawa, Japan
| | - Hironobu Ihn
- Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
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Bournia VK, Tountas C, Protogerou AD, Panopoulos S, Mavrogeni S, Sfikakis PP. Update on assessment and management of primary cardiac involvement in systemic sclerosis. JOURNAL OF SCLERODERMA AND RELATED DISORDERS 2018; 3:53-65. [PMID: 35382127 PMCID: PMC8892878 DOI: 10.1177/2397198317747441] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/16/2017] [Indexed: 09/26/2023]
Abstract
Primary cardiac involvement is a common and severe complication of systemic sclerosis, which may affect all of the hearts' structural components, including pericardium, myocardium, endocardium, cardiac valves, and conduction system. While cardiac disease can be clinically silent and only diagnosed in autopsy, new imaging modalities such as speckle-tracking echocardiography and cardiovascular magnetic resonance may reveal various abnormal findings in the majority of patients. Cardiovascular magnetic resonance evaluation should include assessment of left and right ventricular function, inflammation (STIR T2-weighted sequences (T2-W) for edema detection), and fibrosis (T1-weighted sequences 15 min after Gd-DTPA contrast medium injection (late-gadolinium enhancement). Notably, cardiac disease is responsible for about one-fourth of systemic sclerosis-related deaths. Systematic studies for the assessment and therapy of systemic sclerosis-related cardiac complications, as well as relevant guidelines from the European League Against Rheumatism and the American College of Rheumatology, are currently lacking. However, research advances reviewed herein allow for a better understanding of the mechanisms that alter cardiac function. Implementation of such knowledge should reduce cardiac morbidity and mortality in systemic sclerosis patients.
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Affiliation(s)
- Vasiliki-Kalliopi Bournia
- First Department of Propaedeutic
and Internal Medicine and Joined Rheumatology Program, Medical School,
National and Kapodistrian University of Athens, Laikon Hospital, Athens -
Greece
| | - Christos Tountas
- First Department of Propaedeutic
and Internal Medicine and Joined Rheumatology Program, Medical School,
National and Kapodistrian University of Athens, Laikon Hospital, Athens -
Greece
| | - Athanase D. Protogerou
- Cardiovascular Prevention and
Research Unit, Department of Pathophysiology, Medical School, National and
Kapodistrian University of Athens, Athens - Greece
| | - Stylianos Panopoulos
- First Department of Propaedeutic
and Internal Medicine and Joined Rheumatology Program, Medical School,
National and Kapodistrian University of Athens, Laikon Hospital, Athens -
Greece
| | | | - Petros P. Sfikakis
- First Department of Propaedeutic
and Internal Medicine and Joined Rheumatology Program, Medical School,
National and Kapodistrian University of Athens, Laikon Hospital, Athens -
Greece
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Gyllenhammar T, Kanski M, Engblom H, Wuttge DM, Carlsson M, Hesselstrand R, Arheden H. Decreased global myocardial perfusion at adenosine stress as a potential new biomarker for microvascular disease in systemic sclerosis: a magnetic resonance study. BMC Cardiovasc Disord 2018; 18:16. [PMID: 29382301 PMCID: PMC5791343 DOI: 10.1186/s12872-018-0756-x] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/19/2017] [Accepted: 01/23/2018] [Indexed: 01/26/2023] Open
Abstract
Background Patients with systemic sclerosis (SSc) have high cardiovascular mortality even though there is no or little increase in prevalence of epicardial coronary stenosis. First-pass perfusion on cardiovascular magnetic resonance (CMR) have detected perfusion defects indicative of microvascular disease, but the quantitative extent of hypoperfusion is not known. Therefore, we aimed to determine if patients with SSc have lower global myocardial perfusion (MP) at rest or during adenosine stress, compared to healthy controls, quantified with CMR. Methods Nineteen SSc patients (17 females, 61 ± 10 years) and 22 controls (10 females, 62 ± 11 years) underwent CMR. Twelve patients had limited cutaneous SSc and 7 patients had diffuse cutaneous SSc. One patient had pulmonary arterial hypertension (PAH). MP was quantified using coronary sinus flow (CSF) measurements at rest and during adenosine stress, divided by left ventricular mass (LVM). Results There was no difference in MP at rest between patients and controls (1.1 ± 0.5 vs. 1.1 ± 0.3 ml/min/g, P = 0.85) whereas SSc patients showed statistically significantly lower MP during adenosine stress (3.1 ± 0.9 vs. 4.2 ± 1.3 ml/min/g, P = 0.008). Three out of the 19 SSc patients showed fibrosis in the right ventricle insertion points despite absence of PAH. None had signs of myocardial infarction. Conclusions Patients with SSc have decreased MP during adenosine stress compared to healthy controls. Thus hypoperfusion at stress may be a sensitive marker of cardiac disease in SSc patients possibly signifying microvascular myocardial disease.
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Affiliation(s)
- Tom Gyllenhammar
- Skane University Hospital, Department of Clinical Physiology, Lund University, Lund, Sweden
| | - Mikael Kanski
- Skane University Hospital, Department of Clinical Physiology, Lund University, Lund, Sweden
| | - Henrik Engblom
- Skane University Hospital, Department of Clinical Physiology, Lund University, Lund, Sweden
| | - Dirk M Wuttge
- Skane University Hospital, Department of Rheumatology, Lund University, Lund, Sweden
| | - Marcus Carlsson
- Skane University Hospital, Department of Clinical Physiology, Lund University, Lund, Sweden
| | - Roger Hesselstrand
- Skane University Hospital, Department of Rheumatology, Lund University, Lund, Sweden
| | - Håkan Arheden
- Skane University Hospital, Department of Clinical Physiology, Lund University, Lund, Sweden.
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Bazan IS, Mensah KA, Rudkovskaia AA, Adonteng-Boateng PK, Herzog EL, Buckley L, Fares WH. Pulmonary arterial hypertension in the setting of scleroderma is different than in the setting of lupus: A review. Respir Med 2017; 134:42-46. [PMID: 29413506 DOI: 10.1016/j.rmed.2017.11.020] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/23/2017] [Revised: 11/12/2017] [Accepted: 11/28/2017] [Indexed: 01/08/2023]
Abstract
Pulmonary hypertension (PH) is a clinical syndrome that is subdivided into five groups per the World Health Organization (WHO) classification, based largely on hemodynamic and pathophysiologic criteria. WHO Group 1 PH, termed pulmonary arterial hypertension (PAH), is a clinically progressive disease that can eventually lead to right heart failure and death, and it is hemodynamically characterized by pre-capillary PH and increased pulmonary vascular resistance in the absence of elevated left ventricular filling pressures. PAH can be idiopathic, heritable, or associated with a variety of conditions. Connective tissue diseases make up the largest portion of these associated conditions, most commonly systemic sclerosis (SSc), followed by mixed connective tissue disease and systemic lupus erythematous. These etiologies (namely SSc and Lupus) have been grouped together as connective tissue disease-associated PAH, however emerging evidence suggests they differ in pathogenesis, clinical course, prognosis, and treatment response. This review highlights the differences between SSc-PAH and Lupus-PAH. After introducing the diagnosis, screening, and pathobiology of PAH, we discuss connective tissue disease-associated PAH as a group, and then explore SSc-PAH and SLE-PAH separately, comparing these 2 PAH etiologies.
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Affiliation(s)
- Isabel S Bazan
- Yale University, School of Medicine, Section of Pulmonary, Critical Care & Sleep Medicine, New Haven, CT, USA
| | - Kofi A Mensah
- Yale University, School of Medicine, Section of Rheumatology, New Haven, CT, USA
| | | | | | - Erica L Herzog
- Yale University, School of Medicine, Section of Pulmonary, Critical Care & Sleep Medicine, New Haven, CT, USA
| | - Lenore Buckley
- Yale University, School of Medicine, Section of Rheumatology, New Haven, CT, USA
| | - Wassim H Fares
- Yale University, School of Medicine, Section of Pulmonary, Critical Care & Sleep Medicine, New Haven, CT, USA.
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Dedeoglu R, Adroviç A, Oztunç F, Sahin S, Barut K, Kasapcopur O. New Insights into Cardiac Involvement in Juvenile Scleroderma: A Three-Dimensional Echocardiographic Assessment Unveils Subclinical Ventricle Dysfunction. Pediatr Cardiol 2017; 38:1686-1695. [PMID: 28913656 DOI: 10.1007/s00246-017-1714-6] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/10/2017] [Accepted: 08/29/2017] [Indexed: 12/21/2022]
Abstract
Cardiac manifestations in juvenile scleroderma or systemic sclerosis (JSSc) have poor prognosis, begin in early stages of the disease, and remain clinically asymptomatic. New echocardiography modalities, such as 2D/3D speckle tracking (STE, strain analysis for regional and global ventricular functions), can detect cardiac involvement in early stages. We assessed 21 JSSc patients and 19 controls using 2D/3D STE. The left ventricular end diastolic volume, end systolic volume, and ejection fraction of the patient and control groups were significantly different (99.2 ± 23.8 vs. 52 ± 23.8, 40.6 ± 16.0 vs. 20.2 ± 17.4 and 59.2 ± 7.5 vs. 65.6 ± 5.2, respectively). Global longitudinal strain (GLS) and global circumferential strain (GCS) were lower in the patient group (18.4 ± 4.7 vs. 22.4 ± 3.7, 26.4 ± 5.8 vs. 31.4 ± 3.5), as were the peak systolic strain values of the right ventricular longitudinal strain (RVLS) septum and RVLS free wall (18.1 ± 6.8 vs. 24.8 ± 6.0 and 22.8 ± 5.9 vs. 28.0 ± 6.9, respectively). 3D measurements of RVEDV, RVESV, and RVSV were higher in the patient group (88.2 ± 31.3 vs. 50.8 ± 23.5, 43.1 ± 17.6 vs. 19.0 ± 12.2, and 45.0 ± 16.2 vs. 31.7 ± 12.6). RVLS freewall results were lower in the JSSc patients with interstitial lung fibrosis, arthritis, muscle weakness, weight loss, and anti-scl 70 antibodies than in the JSSc patients without these variables. We found that a GCS of <34.5% could identify patients for left ventricular (LV) dysfunction with a sensitivity of 93.3, specificity of 92.9, while an RVEF of <60.7% could identify patients for left ventricular (RV) dysfunction with a sensitivity of 92.9 and specificity of 21.4%. We highlighted key advantages of 3D STE for the tracking of early systolic dysfunction in patients with JSSc who would benefit from medical intervention for cardiac complications.
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Affiliation(s)
- Reyhan Dedeoglu
- Department of Pediatric Cardiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
| | - Amra Adroviç
- Department of Pediatric Rheumatology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey
| | - Funda Oztunç
- Department of Pediatric Cardiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey
| | - Sezgin Sahin
- Department of Pediatric Rheumatology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey
| | - Kenan Barut
- Department of Pediatric Rheumatology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey
| | - Ozgur Kasapcopur
- Department of Pediatric Rheumatology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey
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Determinants of mortality in systemic sclerosis: a focused review. Rheumatol Int 2017; 38:1847-1858. [PMID: 29116439 DOI: 10.1007/s00296-017-3826-y] [Citation(s) in RCA: 43] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/11/2017] [Accepted: 09/20/2017] [Indexed: 02/06/2023]
Abstract
Scleroderma (systemic sclerosis) is an autoimmune rheumatic disorder that is characterized by fibrosis, vascular dysfunction, and autoantibody production that involves most visceral organs. It is characterized by a high morbidity and mortality rate, mainly due to disease-related complications. Epidemiological data describing mortality and survival in this population have been based on both population and observational studies. Multiple clinical and non-clinical factors have been found to predict higher likelihood of death among thepatients. Here, we do an extensive review of the available literature, utilizing the PubMed database, to describe scleroderma and non-scleroderma related determinants of mortality in this population. We found that even though the mortality among the general population has declined, scleroderma continues to carry a very high morbidity and mortality rate, however we have made some slow progress in improving the mortality among scleroderma patients over the last few decades.
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Roque MCDF, Sampaio-Barros PD, Arruda AL, Barros-Gomes S, Becker D, Andrade JLD, Rodrigues ACT. Evaluation of Left Ventricular Diastolic Function by Echocardiography with Tissue Doppler in Systemic Sclerosis. Arq Bras Cardiol 2017; 109:410-415. [PMID: 28977055 PMCID: PMC5729776 DOI: 10.5935/abc.20170145] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/10/2016] [Accepted: 05/19/2017] [Indexed: 11/26/2022] Open
Abstract
Background Systemic sclerosis (SS) is a connective tissue abnormality characterized by
fibrosis of the skin and internal organs. Cardiac involvement with
consequent myocardial dysfunction in SS is associated with increased
morbidity and mortality. Objective To investigate the left ventricular (LV) diastolic function in patients with
SS and preserved systolic function. Methods Patients with SS were evaluated with two-dimensional echocardiography with
tissue Doppler for analysis of chamber diameters, LV mass index (LVMI),
indexed left atrial volume (iLAV), systolic function of both ventricles, and
presence and degree of diastolic dysfunction (DD). Results We evaluated 50 patients, divided according to the presence of DD into Group
1 (n = 25; normal diastolic function, E/A ratio ≥ 0.8, deceleration
time [DT] > 150 ms and < 200 ms, and septal e’ > 8 cm/s) and Group
2 (n = 25; with DD, subdivided into type I DD [E/A < 0.8, DT > 200
ms], type II [E/A ≥ 0.8, septal e’ < 8 cm/s, iLAV > 34
mL/m2], and type III [E/A > 2, DT < 150 ms, septal e’
< 8 cm/s]). Type I DD was the most frequent (34%), followed by type II DD
(16%). LVMI and iLAV were similar in both groups, but septal and lateral e’
were reduced only in Group 2. In Group 2, we observed that patients with
moderate DD had longer disease duration (p = 0.02). Conclusion The prevalence of type I DD was elevated in SS and associated with aging.
Disease duration emerged as an important factor in moderate DD.
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Affiliation(s)
| | | | - Ana Lucia Arruda
- Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP - Brazil
| | | | - Derly Becker
- Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP - Brazil
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Allanore Y, Distler O, Walker UA, Khanna D, Furst DE, Meune C. Points to consider when doing a trial primarily involving the heart. Rheumatology (Oxford) 2017; 56:v12-v16. [PMID: 28992169 DOI: 10.1093/rheumatology/kex198] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2015] [Indexed: 12/13/2022] Open
Abstract
Cardiac involvement contributes to the severity of SSc and should carefully be investigated and managed in SSc patients. Although it is commonly sub-clinical, once symptomatic it has a poor prognosis. Several complementary tools (circulating biomarkers, electrocardiography, echocardiography, scintigraphy or MRI) allow the assessment of all the various cardiac structures (endocardium, myocardium and pericardium) and heart function. Treatment remains empirical but cardiac trials in SSc can add data to the treatment of this complication.
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Affiliation(s)
- Yannick Allanore
- Department of Rheumatology A, Cochin Hospital and Cochin Institute, AP-HP, Paris-Descartes University, Paris, France
| | - Oliver Distler
- Department of Rheumatology, University Hospital Zurich, Zurich
| | - Ulrich A Walker
- Rheumatology Department, University of Basel, Basel, Switzerland
| | - Dinesh Khanna
- Department of Medicine, University of Michigan, University of Michigan Scleroderma Program, Ann Arbor, MI
| | - Daniel E Furst
- Department of Rheumatology, David Geffen School of Medicine, University of California in Los Angeles, Los Angeles, CA, USA
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