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Kalinoski-Dubose VR, Farooqui N, Osborn TG. 47-Year-Old Woman Admitted for Pericardial Effusion. Mayo Clin Proc 2025; 100:890-894. [PMID: 40318907 DOI: 10.1016/j.mayocp.2024.05.035] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/22/2024] [Revised: 05/01/2024] [Accepted: 05/07/2024] [Indexed: 05/07/2025]
Affiliation(s)
| | - Naba Farooqui
- Resident in Internal Medicine, Mayo Clinic School of Graduate Medical Education, Rochester, MN
| | - Thomas G Osborn
- Advisor to residents and Consultant in Rheumatology, Mayo Clinic, Rochester, MN.
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2
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Sanada H, Hara S, Horita M, Kawahara H, Yoshida M, Takahashi Y, Tsuge S, Zoshima T, Nishioka R, Ito K, Mizushima I, Matsushita T, Kawano M. De novo normotensive scleroderma renal crisis six years after living-donor renal transplantation in a patient with overlapping systemic sclerosis/systemic lupus erythematosus syndrome: a case report. BMC Nephrol 2023; 24:355. [PMID: 38049714 PMCID: PMC10696825 DOI: 10.1186/s12882-023-03416-7] [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: 09/30/2023] [Accepted: 11/28/2023] [Indexed: 12/06/2023] Open
Abstract
BACKGROUND Scleroderma renal crisis (SRC) is a critical kidney involvement of systemic sclerosis (SSc), often resulting in end-stage renal disease. Although the recurrence of SRC in the allograft has been reported, the development of de novo SRC after kidney transplantation has not been reported. Furthermore, normotensive SRC, which rarely occurs, makes prompt diagnosis more challenging. This fact should be recognized widely among nephrologists. CASE PRESENTATION We report a 37-year-old Japanese man with overlapping SSc/systemic lupus erythematous syndrome who developed normotensive SRC in the transplanted kidney shortly after glucocorticoid escalation. Six years prior to admission, he underwent an ABO-compatible living donor kidney transplantation because of lupus nephritis. He was admitted to our hospital for gradually worsening kidney dysfunction. A kidney biopsy showed idiopathic granulomatous interstitial nephritis and high-dose prednisolone was prescribed. Although renal function improved tentatively, it deteriorated again a week later. A secondary kidney biopsy revealed acute thrombotic microangiopathy, leading to the diagnosis of normotensive SRC because all other causes were excluded, and blood pressure was within normal range. Adding an angiotensin-converting enzyme inhibitor and tapering glucocorticoid slowed the speed of deterioration of his kidney function, but he finally required hemodialysis induction. CONCLUSIONS SRC can newly develop even in the transplanted kidney, especially when high-dose glucocorticoid is administered. Normotensive SRC makes the diagnosis challenging, so nephrologists should carefully monitor patients with SSc and transplanted kidneys to treat SRC promptly.
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Affiliation(s)
- Hajime Sanada
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan
| | - Satoshi Hara
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan.
| | - Makoto Horita
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan
| | - Hiroyuki Kawahara
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan
| | - Misaki Yoshida
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan
| | - Yoshinori Takahashi
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan
| | - Shunsuke Tsuge
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan
| | - Takeshi Zoshima
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan
| | - Ryo Nishioka
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan
| | - Kiyoaki Ito
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan
| | - Ichiro Mizushima
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan
| | - Takashi Matsushita
- Department of Dermatology, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan
| | - Mitsuhiro Kawano
- Department of Rheumatology, Kanazawa University Hospital, 13-1, Takara-machi, Kanazawa, Ishikawa, Japan
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Portocarrero JP, Tawhari I, Ghossein C. Kidney Transplant Outcomes in Patients With Scleroderma an Experience at Northwestern Memorial Hospital, Chicago, Illinois. Transplant Proc 2023; 55:2410-2413. [PMID: 37923572 DOI: 10.1016/j.transproceed.2023.09.019] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/30/2023] [Revised: 08/15/2023] [Accepted: 09/22/2023] [Indexed: 11/07/2023]
Abstract
BACKGROUND Scleroderma renal crisis (SRC) is a devastating complication of diffuse cutaneous systemic sclerosis (dcSSc) that occurs in 5% to 20% of patients in this population. End-stage kidney disease develops in 25% to 40% of SRC, and mortality occurs in 50% at 5 years. Kidney transplantation (KT) is a viable option, but little data exist on outcomes. METHODS We performed a retrospective study of all patients with dcSSc who underwent KT at Northwestern Hospital between 2000 and 2020. The objective of this study was to determine graft and patient survival at years 5 and 10 post-transplant. RESULTS Both patient and graft survival were 78% and 100% at 5 and 10 years, respectively. Kidney transplantation is associated with favorable outcomes in patients and graft survival at 5 and 10 years in patients with dcSSc.
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Affiliation(s)
| | | | - Cybele Ghossein
- Northwestern University Feinberg School of Medicine, Chicago, IL
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Scheen M, Dominati A, Olivier V, Nasr S, De Seigneux S, Mekinian A, Issa N, Haidar F. Renal involvement in systemic sclerosis. Autoimmun Rev 2023; 22:103330. [PMID: 37031831 DOI: 10.1016/j.autrev.2023.103330] [Citation(s) in RCA: 10] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2023] [Accepted: 04/03/2023] [Indexed: 04/11/2023]
Abstract
Systemic sclerosis is a rare autoimmune vasculopathy associated with dysregulated innate and adaptive immunity that leads to generalized systemic fibrosis. Renal involvement occurs in a significant proportion of systemic sclerosis patients, and is associated with worse outcome. Scleroderma renal crisis (SRC) is the most studied and feared renal complication described in systemic sclerosis. However, with the emergence of ACE inhibitors and better management, the mortality rate of SRC has significantly decreased. Renal disease in systemic sclerosis offers a wide array of differential diagnoses that may be challenging for the clinician. The spectrum of renal manifestations in systemic sclerosis ranges from an isolated decrease in glomerular filtration rate, increased intrarenal arterial stiffness, and isolated proteinuria due to SRC to more rare manifestations such as association with antiphospholipid antibody nephropathy and ANCA-associated vasculitis. The changes observed in the kidneys in systemic sclerosis are thought to be due to a complex interplay of various factors, including renal vasculopathy, as well as the involvement of the complement system, vasoactive mediators such as endothelin-1, autoimmunity, prothrombotic and profibrotic cytokines, among others. This literature review aims to provide an overview of the main renal manifestations in systemic sclerosis by discussing the most recent epidemiological and pathophysiological data available and the challenges for clinicians in making a diagnosis of renal disease in patients with systemic sclerosis.
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Affiliation(s)
- Marc Scheen
- Hôpitaux Universitaires de Genève, Service de Néphrologie, Rue Gabrielle-Perret-Gentil 4, 1205 Genève, Switzerland.
| | - Arnaud Dominati
- Hôpitaux Universitaires de Genève, Service d'allergologie et immunologie, Rue Gabrielle-Perret-Gentil 4, 1205 Genève, Switzerland
| | - Valérie Olivier
- Hôpitaux Universitaires de Genève, Service de Néphrologie, Rue Gabrielle-Perret-Gentil 4, 1205 Genève, Switzerland
| | - Samih Nasr
- Mayo Clinic College of Medicine and Science, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
| | - Sophie De Seigneux
- Hôpitaux Universitaires de Genève, Service de Néphrologie, Rue Gabrielle-Perret-Gentil 4, 1205 Genève, Switzerland
| | - Arsène Mekinian
- Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Médecine Interne, 75012 Paris, France
| | - Naim Issa
- Mayo Clinic College of Medicine and Science, Division of Nephrology and Hypertension, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
| | - Fadi Haidar
- Hôpitaux Universitaires de Genève, Service de Néphrologie, Rue Gabrielle-Perret-Gentil 4, 1205 Genève, Switzerland
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5
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Maritati F, Provenzano M, Lerario S, Corradetti V, Bini C, Busutti M, Grandinetti V, Cuna V, La Manna G, Comai G. Kidney transplantation in systemic sclerosis: Advances in graft, disease, and patient outcome. Front Immunol 2022; 13:878736. [PMID: 35958558 PMCID: PMC9360313 DOI: 10.3389/fimmu.2022.878736] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/18/2022] [Accepted: 06/30/2022] [Indexed: 11/30/2022] Open
Abstract
Systemic sclerosis (SSc) is an immune-mediated rheumatic disease characterized by vascular abnormalities, tissue fibrosis, and inflammation. Renal disease occurring in patients with SSc may have a variable clinicopathological picture. However, the most specific renal condition associated with this disease is the scleroderma renal crisis (SRC), characterized by acute onset of renal failure and severe hypertension. SRC develops in about 20% of cases of SSc, especially in those patients with diffuse cutaneous disease. The prognosis of this condition is often negative, with a rapid progression to end-stage renal disease (ESRD). The advent of the antihypertensive angiotensin-converting enzyme inhibitors in 1980 was associated with a significant improvement in patients’ survival and recovery of renal function. However, the prognosis of these patients can still be improved. The dialytic condition is associated with early death, and mortality is significantly higher than among patients undergoing renal replacement therapy (RRT) due to other conditions. Patients with SRC who show no signs of renal functional recovery despite timely blood pressure control are candidates for kidney transplantation (KT). In this review, we reported the most recent advances in KT in patients with ESRD due to SSc, with a particular overview of the risk of disease recurrence after transplantation and the evolution of other disease manifestations.
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6
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Kidney Involvement in Systemic Sclerosis. J Pers Med 2022; 12:jpm12071123. [PMID: 35887620 PMCID: PMC9324204 DOI: 10.3390/jpm12071123] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2022] [Revised: 07/07/2022] [Accepted: 07/08/2022] [Indexed: 11/30/2022] Open
Abstract
Background: Systemic sclerosis is a chronic multisystem autoimmune disease, characterized by diffuse fibrosis and abnormalities of microcirculation and small arterioles in the skin, joints and visceral organs. Material and Methods: We searched for the relevant articles on systemic sclerosis and kidney involvement in systemic sclerosis in the NIH library of medicine, transplant, rheumatologic and nephrological journals. Results: Half of patients with systemic sclerosis have clinical evidence of kidney involvement. Scleroderma renal crisis represents the most specific and serious renal event associated with this condition. It is characterized by an abrupt onset of moderate to marked hypertension and kidney failure. Early and aggressive treatment is mandatory to prevent irreversible organ damage and death. The advent of ACE-inhibitors revolutionized the management of scleroderma renal crisis. However, the outcomes of this serious complication are still poor, and between 20 to 50% of patients progress to end stage renal disease. Conclusions: Scleroderma renal crisis still represents a serious and life-threatening event. Thus, further studies on its prevention and on new therapeutic strategies should be encouraged.
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7
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Medina-Bielski S, Mohan S, Makol A. 72-Year-Old Man With Puffy Fingers and Raynaud Phenomenon. Mayo Clin Proc 2022; 97:1014-1019. [PMID: 35512871 DOI: 10.1016/j.mayocp.2021.10.029] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/30/2021] [Revised: 10/27/2021] [Accepted: 10/29/2021] [Indexed: 11/24/2022]
Affiliation(s)
- Sara Medina-Bielski
- Resident in Internal Medicine, Mayo Clinic School of Graduate Medical Education, Rochester, MN
| | - Sneha Mohan
- Resident in Internal Medicine, Mayo Clinic School of Graduate Medical Education, Rochester, MN
| | - Ashima Makol
- Advisor to residents and Consultant in Rheumatology, Mayo Clinic, Rochester, MN.
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8
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Abbas F, El Kossi M, Shaheen IS, Sharma A, Halawa A. Journey of a patient with scleroderma from renal failure up to kidney transplantation. World J Transplant 2021; 11:372-387. [PMID: 34631469 PMCID: PMC8465513 DOI: 10.5500/wjt.v11.i9.372] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2021] [Revised: 04/10/2021] [Accepted: 08/19/2021] [Indexed: 02/06/2023] Open
Abstract
The increased awareness of systemic sclerosis (SS) and its pathogenetic background made the management of this disease more amenable than previously thought. However, scleroderma renal crisis (SRC) is a rarely seen as an associated disorder that may involve 2%-15% of SS patients. Patients presented with earlier, rapidly progressing, diffuse cutaneous SS disease, mostly in the first 3-5 years after non-Raynaud clinical manifestations, are more vulnerable to develop SRC. SRC comprises a collection of acute, mostly symptomatic rise in blood pressure, elevation in serum creatinine concentrations, oliguria and thrombotic microangiopathy in almost 50% of cases. The advent of the antihypertensive angiotensin converting enzyme inhibitors in 1980 was associated with significant improvement in SRC prognosis. In a scleroderma patient maintained on regular dialysis; every effort should be exerted to declare any possible evidence of renal recovery. A given period of almost two years has been suggested prior to proceeding in a kidney transplant (KTx). Of note, SS patients on dialysis have the highest opportunity of renal recovery and withdrawal from dialysis as compared to other causes of end-stage renal disease (ESRD). KTx that is the best well-known therapeutic option for ESRD patients can also be offered to SS patients. Compared to other primary renal diseases, SS-related ESRD was considered for a long period of poor patient and allograft survivals. Pulmonary involvement in an SS patient is considered a strong post-transplant independent risk factor of death. Recurrence of SRC after transplantation has been observed in some patients. However, an excellent post-transplant patient and graft outcome have been recently reported. Consequently, the absence of extrarenal manifestations in an SS-induced ESRD patient can be accepted as a robust indicator for a successful KTx.
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Affiliation(s)
- Fedaey Abbas
- Department of Nephrology, Faculty of Health and Science, University of Liverpool, Institute of Learning and Teaching, School of Medicine, Liverpool L69 3GB, United Kingdom
| | - Mohsen El Kossi
- Doncaster Renal Unit, Doncaster Royal Infirmary, Doncaster DN2 5LT, United Kingdom
| | - Ihab Sakr Shaheen
- Department of Paediatric Nephrology, St James’s University Hospital, Glasgow G51 4TF, United Kingdom
| | - Ajay Sharma
- Department of Transplant Surgery, Royal Liverpool University Hospital, Liverpool L7 8XP, United Kingdom
| | - Ahmed Halawa
- Department of Transplant Surgery, Sheffield Teaching Hospital, Sheffield S5 7AU, United Kingdom
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Hachulla E, Agard C, Allanore Y, Avouac J, Bader-Meunier B, Belot A, Berezne A, Bouthors AS, Condette-Wojtasik G, Constans J, De Groote P, Diot E, Dumas F, Jego P, Joly F, Launay D, Le Guern V, Le Quintrec JS, Lescaille G, Meune C, Moulin B, Nguyen C, Omeish N, Pene F, Richard MA, Rochefort J, Roren A, Sitbon O, Sobanski V, Truchetet ME, Mouthon L. French recommendations for the management of systemic sclerosis. Orphanet J Rare Dis 2021; 16:322. [PMID: 34304732 PMCID: PMC8310704 DOI: 10.1186/s13023-021-01844-y] [Citation(s) in RCA: 46] [Impact Index Per Article: 11.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/26/2021] [Indexed: 12/13/2022] Open
Abstract
Systemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis.
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Affiliation(s)
- Eric Hachulla
- Service de Médecine Interne et Immunologie Clinique, Centre de Référence Des Maladies Autoimmunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, 59000, Lille, France.
| | - Christian Agard
- Internal Medicine, Nantes University Hospital, University of Nantes, Nantes, France
| | - Yannick Allanore
- Rheumatology Department, Hôpital Cochin, AP-HP, Université de Paris, Paris, France
| | - Jerome Avouac
- Rheumatology Department, Hôpital Cochin, AP-HP, Université de Paris, Paris, France
| | - Brigitte Bader-Meunier
- Department of Pediatric Immunology and Rheumatology; Hospital Necker, APHP, Paris, France
| | - Alexandre Belot
- Pediatric Nephrology, Rheumatology, Dermatology, HFME, Hospices Civils de Lyon, Bron, France
| | - Alice Berezne
- Department of Internal Medicine, CHR Annecy-Genevois, Annecy, France
| | - Anne-Sophie Bouthors
- Anaesthesia Intensive Care Unit, Jeanne de Flandre Women Hospital, Academic Hospital, ULR 7365 - GRITA - Groupe de Recherche Sur Les Formes Injectables Et Les Technologies Associées, University Lille, Lille, France
| | - Geraldine Condette-Wojtasik
- Service de Médecine Interne et Immunologie Clinique, Centre de Référence Des Maladies Autoimmunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, 59000, Lille, France
| | - Joël Constans
- Vascular Medicine Department, Bordeaux University Hospital Centre, Saint André Hospital, FCRIN INI-CRCT (Cardiovascular and Renal Clinical Trialists) PeripherAL Artery DIsease Network (PALADIN), Bordeaux, France
| | - Pascal De Groote
- Cardiology Department, Lung-Heart Institute, CHU de Lille, 59000, Lille, France
| | | | - Florence Dumas
- Emergency Department, Cochin Hospital, Paris University, Paris, France
| | - Patrick Jego
- Internal Medicine and Clinical Immunology Unit, CHU Rennes, Rennes, France
| | - Francisca Joly
- Department of Gastroenterology, IBD and Nutrition Support, Beaujon Hospital, INSERM UMRS-1149, Assistance Publique-Hôpitaux de Paris, University of Paris, Clichy, France
| | - David Launay
- Service de Médecine Interne et Immunologie Clinique, Centre de Référence Des Maladies Autoimmunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, 59000, Lille, France
| | - Veronique Le Guern
- Service de Médecine Interne, Centre de Référence Maladies Autoimmunes Systémiques Rares D'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), APHP-CUP, Hôpital Cochin, Université de Paris, 75014, Paris, France
| | | | - Geraldine Lescaille
- Centre d'Immunologie et Maladies Infectieuses (CIMI-Paris), Department of Odontology, Paris Diderot/Paris 07, Sorbonne Paris Cité, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France
| | - Christophe Meune
- Cardiology Department, Hôpital Avicenne, AP-HP, Université de Paris, Paris, France
| | - Bruno Moulin
- Department of Nephrology and Kidney Transplantation, Nouvel Hôpital Civil, University Hospitals of Strasbourg, Strasbourg, France
| | - Christelle Nguyen
- Physical Medicine and Rehabilitation Department, Hôpital Cochin, AP-HP, Université de Paris, Paris, France
| | - Nadine Omeish
- Oral and Dental Medicine, Hôpital Pitié-Salpêtrière, APHP, Université de Paris, Paris, France
| | - Frederic Pene
- Medical Intensive Care Unit, Hôpital Cochin, AP-HP. Centre & Université de Paris, Paris, France
| | - Marie-Aleth Richard
- Department of Dermatology, Timone Hospital, University Hospital of Marseille, Marseille, France
| | - Juliette Rochefort
- Oral and Dental Medicine, Hôpital Pitié-Salpêtrière, APHP, Université de Paris, Paris, France
| | - Alexandra Roren
- AP-HP Cochin Hospital, Université Paris Descartes Sorbonne Paris Cité, INSERM U1153, Paris, France
| | - Olivier Sitbon
- Assistance Publique-Hôpitaux de Paris, Service de Pneumologie, Hôpital Bicêtre, Laboratoire d'Excellence en Recherche Sur le Médicament et Innovation Thérapeutique, Université Paris-Sud, Le Kremlin-Bicêtre, France
| | - Vincent Sobanski
- Service de Médecine Interne et Immunologie Clinique, Centre de Référence Des Maladies Autoimmunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, 59000, Lille, France
| | | | - Luc Mouthon
- Service de Médecine Interne, Centre de Référence Maladies Autoimmunes Systémiques Rares D'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), APHP-CUP, Hôpital Cochin, Université de Paris, 75014, Paris, France.
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Kim H, Lefebvre F, Hoa S, Hudson M. Mortality and morbidity in scleroderma renal crisis: A systematic literature review. JOURNAL OF SCLERODERMA AND RELATED DISORDERS 2021; 6:21-36. [PMID: 35382245 PMCID: PMC8922629 DOI: 10.1177/2397198320920422] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/13/2020] [Accepted: 03/19/2020] [Indexed: 07/22/2023]
Abstract
OBJECTIVES The objective of this study was to systematically review the mortality and morbidity associated with scleroderma renal crisis and to determine temporal trends. METHODS We searched MEDLINE, Embase and the Cochrane Database of Systematic Reviews from database inception to 10 February 2020. Bibliographies of selected articles were hand-searched for additional references. Data were extracted using a standardized extraction form. Study quality was assessed using the Newcastle-Ottawa scale. Results were analysed qualitatively. RESULTS Twenty studies with 14,059 systemic sclerosis subjects, of which 854 had scleroderma renal crisis and 4095 had systemic sclerosis-associated end-stage renal disease, met inclusion criteria. Study quality was generally moderate. Cumulative mortality in the post-angiotensin-converting enzyme inhibitor era was approximately 20% at 6 months, 30%-36% at 1 year, 19%-40% at 3 years and almost 50% at 10 years from scleroderma renal crisis onset. Although the introduction of angiotensin-converting enzyme inhibitors in the early 1970s resulted in a 50% improvement in scleroderma renal crisis mortality, there was no further improvement thereafter. Scleroderma renal crisis mortality rates were proportionally higher than mortality rates associated with other systemic sclerosis organ involvement. The rate of permanent dialysis after scleroderma renal crisis in the post-angiotensin-converting enzyme inhibitor era ranged from 19%-40%. Three to 17% of systemic sclerosis patients underwent renal transplant. Survival was better in patients post-renal transplant (54%-91%) compared to those on dialysis (31%-56%). Graft survival improved over time and appeared similar to that of patients with other types of end-stage renal disease. CONCLUSION While there has been considerable improvement in scleroderma renal crisis-related outcomes since the introduction of angiotensin-converting enzyme inhibitors, morbidity and mortality remain high for affected patients without convincing evidence of further improvement in the post-angiotensin-converting enzyme inhibitor era. Novel treatments are required to improve outcomes of scleroderma renal crisis.
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Affiliation(s)
- Hyein Kim
- Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada
| | - Frédéric Lefebvre
- Division of Rheumatology, Department of Medicine, Université de Montréal, Montreal, QC, Canada
| | - Sabrina Hoa
- Division of Rheumatology, Department of Medicine, Université de Montréal, Montreal, QC, Canada
- Centre Hospitalier de l'Université de Montréal Research Center, Montreal, QC, Canada
| | - Marie Hudson
- Lady Davis Institute, Jewish General Hospital, Montreal, QC, Canada
- Department of Medicine, McGill University, Montreal, QC, Canada
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11
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Chrabaszcz M, Małyszko J, Sikora M, Alda-Malicka R, Stochmal A, Matuszkiewicz-Rowinska J, Rudnicka L. Renal Involvement in Systemic Sclerosis: An Update. Kidney Blood Press Res 2020; 45:532-548. [PMID: 32521536 DOI: 10.1159/000507886] [Citation(s) in RCA: 37] [Impact Index Per Article: 7.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2020] [Accepted: 04/14/2020] [Indexed: 12/23/2022] Open
Abstract
BACKGROUND Systemic sclerosis is an immune-mediated rheumatic disease characterized by vascular abnormalities, tissue fibrosis and autoimmune phenomena. SUMMARY Renal disease occurring in patients with systemic sclerosis may have a variable clinicopathological picture. The most specific renal condition associated with systemic sclerosis is scleroderma renal crisis, characterized by acute onset of renal failure and severe hypertension. Although the management of scleroderma renal crisis was revolutionized by the introduction of angiotensin-converting enzyme inhibitors, there is still a significant proportion of patients with poor outcomes. Therefore, research on establishing disease markers (clinical, ultrasonographical and serological) and clear diagnostic criteria, which could limit the risk of developing scleroderma renal crisis and facilitate diagnosis of this complication, is ongoing. Other forms of renal involvement in systemic sclerosis include vasculitis, an isolated reduced glomerular filtration rate in systemic sclerosis, antiphospholipid-associated nephropathy, high intrarenal arterial stiffness and proteinuria. Key Messages: Scleroderma renal crisis is the most specific and life-threatening renal presentation of systemic sclerosis, albeit with declining prevalence. In patients with scleroderma renal crisis, it is mandatory to control blood pressure early with increasing doses of angiotensin-converting enzyme inhibitors, along with other antihypertensive drugs if necessary. There is a strong association between renal involvement and patients' outcomes in systemic sclerosis; consequently, it becomes mandatory to find markers that may be used to identify patients with an especially high risk of scleroderma renal crisis.
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Affiliation(s)
| | - Jolanta Małyszko
- Department of Nephrology, Dialysis and Internal Medicine, Medical University of Warsaw, Warsaw, Poland,
| | - Mariusz Sikora
- Department of Dermatology, Medical University of Warsaw, Warsaw, Poland
| | | | - Anna Stochmal
- Department of Dermatology, Medical University of Warsaw, Warsaw, Poland
| | | | - Lidia Rudnicka
- Department of Dermatology, Medical University of Warsaw, Warsaw, Poland
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Honda S, Katsumata Y, Karasawa K, Yamanaka H, Harigai M. Management of End-stage Renal Disease Associated with Systemic Rheumatic Diseases. JMA J 2020; 3:20-28. [PMID: 33324772 PMCID: PMC7733740 DOI: 10.31662/jmaj.2019-0020] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/28/2019] [Accepted: 07/22/2019] [Indexed: 11/15/2022] Open
Abstract
The outcomes of rheumatic diseases (RDs) have improved over the past decades. However, a significant proportion of the patients still suffer from end-stage renal disease (ESRD) and have to bear the burden of hemodialysis. It is crucial to prevent patients with RDs from developing ESRD from viewpoints of medicine and medical economics. For those who already have ESRD, it is important to improve vial prognosis and quality of life through appropriate management of disease activity and comorbidities related to ESRD. Thus, rheumatologists and nephrologists need to recognize risk factors associated with progression to ESRD along with their appropriate management. Although the activity of most RDs tends to decrease after initiation of hemodialysis, disease activity may still increase, and recognizing how to appropriately use immunosuppressive agents even after the development of ESRD is crucial. The treatment of RDs needs extra attention as hydroxychloroquine requires more frequent monitoring for adverse drug reactions; therapeutic drug monitoring is necessary for mycophenolate mofetil, cyclosporine A, and tacrolimus; cyclophosphamide and azathioprine need dose adjustments; methotrexate and bucillamine are contraindicated in patients with ESRD; leflunomide and sulfasalazine do not require significant dose reduction and iguratimod should be carefully administered. The pharmacokinetics of biological agents such as rituximab or belimumab are not affected by ESRD, and dose adjustments are not necessary. Collaboration between rheumatologists and nephrologists is needed more than ever and is expected to produce a complementary effect and achieve better outcomes in clinical settings, although this cooperation has not always been conducted appropriately.
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Affiliation(s)
- Suguru Honda
- Department of Rheumatology, School of Medicine, Tokyo Women's Medical University, Tokyo, Japan
| | - Yasuhiro Katsumata
- Department of Rheumatology, School of Medicine, Tokyo Women's Medical University, Tokyo, Japan
| | - Kazunori Karasawa
- Department of Nephrology, School of Medicine, Tokyo Women's Medical University, Tokyo, Japan
| | - Hisashi Yamanaka
- Department of Rheumatology, School of Medicine, Tokyo Women's Medical University, Tokyo, Japan
| | - Masayoshi Harigai
- Department of Rheumatology, School of Medicine, Tokyo Women's Medical University, Tokyo, Japan
- Division of Epidemiology and Pharmacoepidemiology, Department of Rheumatology, School of Medicine, Tokyo Women's Medical University, Tokyo, Japan
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Montrief T, Koyfman A, Long B. Scleroderma renal crisis: a review for emergency physicians. Intern Emerg Med 2019; 14:561-570. [PMID: 31076978 DOI: 10.1007/s11739-019-02096-2] [Citation(s) in RCA: 3] [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: 01/09/2019] [Accepted: 04/27/2019] [Indexed: 12/15/2022]
Abstract
Scleroderma renal crisis (SRC) remains a high-risk clinical presentation, and many patients require emergency department (ED) management for complications and stabilization. This narrative review provides an evidence-based summary of the current data for the emergency medicine evaluation and management of SRC. While SRC remains a rare clinical presentation, surveillance data suggest an overall incidence between 4 and 6% of patients with scleroderma. The diagnostic criteria for SRC include a new onset blood pressure > 150/85 mm Hg OR increase ≥ 20 mm Hg from baseline systolic blood pressure, along with a decline in renal function, defined as an increase serum creatinine of ≥ 10% and supportive features. There are many risk factors for SRC, including diffuse and rapidly progressive skin thickening, palpable tendon friction rubs, and new anemia or cardiac events. Critical patients should be evaluated in the resuscitation bay, and consultation with the nephrology team for appropriate patients improves patient outcomes.
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MESH Headings
- Angiotensin-Converting Enzyme Inhibitors/therapeutic use
- Antibodies, Antineutrophil Cytoplasmic/analysis
- Antibodies, Antineutrophil Cytoplasmic/blood
- Emergency Service, Hospital/organization & administration
- Humans
- Kidney Failure, Chronic/drug therapy
- Kidney Failure, Chronic/etiology
- Kidney Failure, Chronic/physiopathology
- Purpura, Thrombotic Thrombocytopenic/blood
- Purpura, Thrombotic Thrombocytopenic/diagnosis
- Purpura, Thrombotic Thrombocytopenic/physiopathology
- Renal Dialysis/methods
- Risk Factors
- Scleroderma, Systemic/blood
- Scleroderma, Systemic/complications
- Scleroderma, Systemic/physiopathology
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Affiliation(s)
- Tim Montrief
- Department of Emergency Medicine, Jackson Memorial Hospital/Miller School of Medicine, University of Miami, 1611 N.W. 12th Avenue, Miami, FL, 33136, USA
| | - Alex Koyfman
- Department of Emergency Medicine, The University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX, 75390, USA
| | - Brit Long
- Department of Emergency Medicine, Brooke Army Medical Center, 3841 Roger Brooke Dr, Fort Sam Houston, TX, 78234, USA.
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Woodworth TG, Furst DE. Timely renal transplantation for scleroderma end-stage kidney disease patients can improve outcomes and quality of life. ANNALS OF TRANSLATIONAL MEDICINE 2019; 7:60. [PMID: 30906764 DOI: 10.21037/atm.2018.12.64] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Affiliation(s)
- Thasia G Woodworth
- Department of Rheumatology, David Geffen School of Medicine, University of California, Los Angeles, CA, USA
| | - Daniel E Furst
- Department of Rheumatology, David Geffen School of Medicine, University of California, Los Angeles, CA, USA.,Division of Rheumatology, Department of Medicine, University of Washington, Seattle, WA, USA.,Department of Experimental Medicine, University of Florence, Florence, Italy
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15
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Lung Transplantation in Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD). CURRENT PULMONOLOGY REPORTS 2018. [DOI: 10.1007/s13665-018-0207-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/28/2022]
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Zanatta E, Polito P, Favaro M, Larosa M, Marson P, Cozzi F, Doria A. Therapy of scleroderma renal crisis: State of the art. Autoimmun Rev 2018; 17:882-889. [DOI: 10.1016/j.autrev.2018.03.012] [Citation(s) in RCA: 42] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/05/2018] [Accepted: 03/12/2018] [Indexed: 12/12/2022]
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17
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Hruskova Z, Pippias M, Stel VS, Abad-Díez JM, Benítez Sánchez M, Caskey FJ, Collart F, De Meester J, Finne P, Heaf JG, Magaz A, Palsson R, Reisæter AV, Salama AD, Segelmark M, Traynor JP, Massy ZA, Jager KJ, Tesar V. Characteristics and Outcomes of Patients With Systemic Sclerosis (Scleroderma) Requiring Renal Replacement Therapy in Europe: Results From the ERA-EDTA Registry. Am J Kidney Dis 2018; 73:184-193. [PMID: 30122544 DOI: 10.1053/j.ajkd.2018.05.016] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2017] [Accepted: 05/26/2018] [Indexed: 01/27/2023]
Abstract
RATIONALE & OBJECTIVE Data for outcomes of patients with end-stage renal disease (ESRD) secondary to systemic sclerosis (scleroderma) requiring renal replacement therapy (RRT) are limited. We examined the incidence and prevalence of ESRD due to scleroderma in Europe and the outcomes among these patients following initiation of RRT. STUDY DESIGN Registry study of incidence and prevalence and a matched cohort study of clinical outcomes. SETTING & PARTICIPANTS Patients represented in any of 19 renal registries that provided data to the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Registry between 2002 and 2013. PREDICTOR Scleroderma as the identified cause of ESRD. OUTCOMES Incidence and prevalence of ESRD from scleroderma. Recovery from RRT dependence, patient survival after ESRD, and graft survival after kidney transplantation. ANALYTICAL APPROACH Incidence and prevalence were calculated using population data from the European Union and standardized to population characteristics in 2005. Patient and graft survival were compared with 2 age- and sex-matched control groups without scleroderma: (1) diabetes mellitus as the cause of ESRD and (2) conditions other than diabetes mellitus as the cause of ESRD. Survival analyses were performed using Kaplan-Meier analysis and Cox regression. RESULTS 342 patients with scleroderma (0.14% of all incident RRT patients) were included. Between 2002 and 2013, the range of adjusted annual incidence and prevalence rates of RRT for ESRD due to scleroderma were 0.11 to 0.26 and 0.73 to 0.95 per million population, respectively. Recovery of independent kidney function was greatest in the scleroderma group (7.6% vs 0.7% in diabetes mellitus and 2.0% in other primary kidney diseases control group patients, both P<0.001), though time required to achieve recovery was longer. The 5-year survival probability from day 91 of RRT among patients with scleroderma was 38.9% (95% CI, 32.0%-45.8%), whereas 5-year posttransplantation patient survival and 5-year allograft survival were 88.2% (95% CI, 75.3%-94.6%) and 72.4% (95% CI, 55.0%-84.0%), respectively. Adjusted mortality from day 91 on RRT was higher among patients with scleroderma than observed in both control groups (HRs of 1.25 [95% CI, 1.05-1.48] and 2.00 [95% CI, 1.69-2.39]). In contrast, patient and graft survival after kidney transplantation did not differ between patients with scleroderma and control groups. LIMITATIONS No data for extrarenal manifestations, treatment, or recurrence. CONCLUSIONS Survival of patients with scleroderma who receive dialysis for more than 90 days was worse than for those with other causes of ESRD. Patient survival after transplantation was similar to that observed among patients with ESRD due to other conditions. Patients with scleroderma had a higher rate of recovery from RRT dependence than controls.
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Affiliation(s)
- Zdenka Hruskova
- Department of Nephrology, 1st Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic
| | - Maria Pippias
- ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, Amsterdam Public Health Research Institute, University of Amsterdam, Amsterdam, the Netherlands.
| | - Vianda S Stel
- ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, Amsterdam Public Health Research Institute, University of Amsterdam, Amsterdam, the Netherlands
| | | | | | - Fergus J Caskey
- UK Renal Registry, Southmead Hospital, Bristol, United Kingdom; Population Health Sciences, University of Bristol, Bristol, United Kingdom
| | | | - Johan De Meester
- Department of Nephrology, Dialysis and Hypertension, Dutch-speaking Belgian Renal Registry (NBVN), Sint-Niklaas, Belgium
| | - Patrik Finne
- Department of Nephrology, Helsinki University and Helsinki University Hospital, Helsinki, Finland; Finnish Registry for Kidney Diseases, Helsinki, Finland
| | - James G Heaf
- Department of Medicine, Zealand University Hospital, Roskilde, Denmark
| | - Angela Magaz
- Unidad de Información sobre Pacientes Renales de la Comunidad Autónoma del País Vasco (UNIPAR), Basque Country, Spain
| | - Runolfur Palsson
- Division of Nephrology, Landspitali-The National University Hospital of Iceland, Reykjavik, Iceland; Faculty of Medicine, School of Health Sciences, University of Iceland, Reykjavik, Iceland
| | - Anna Varberg Reisæter
- Department of Transplantation Medicine, Oslo University Hospital, Rikshospitalet, Norway
| | - Alan D Salama
- University College London Centre for Nephrology, Royal Free Hospital, London, United Kingdom
| | - Mårten Segelmark
- Department of Medical and Health Sciences, Linköping University, Linköping, Sweden; Department of Nephrology, Linköping University, Linköping, Sweden
| | | | - Ziad A Massy
- Division of Nephrology, Ambroise Paré University Hospital, APHP, Boulogne-Billancourt; Institut National de la Santé et de la Recherche Médicale (INSERM) U1018, Team 5, CESP UVSQ, and University Paris Saclay, Villejuif, France
| | - Kitty J Jager
- ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, Amsterdam Public Health Research Institute, University of Amsterdam, Amsterdam, the Netherlands
| | - Vladimir Tesar
- Department of Nephrology, 1st Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic
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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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Bertrand D, Dehay J, Ott J, Sberro R, Brunelle C, Kamar N, Colosio C, Chatelet V, Albano L, Girerd S, Audard V, Barbet C, Dantal J, Ducloux D, Durrbach A, Garrigue V, Hazzan M, Heng AE, Mariat C, Merville P, Rerolle JP, Moulin B, Guerrot D. Kidney transplantation in patients with systemic sclerosis: a nationwide multicentre study. Transpl Int 2017; 30:256-265. [DOI: 10.1111/tri.12923] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2016] [Revised: 09/30/2016] [Accepted: 12/12/2016] [Indexed: 12/19/2022]
Affiliation(s)
- Dominique Bertrand
- Nephrology Dialysis Transplantation; Centre Hospitalier Universitaire de Rouen; Rouen Haute-Normandie France
| | - Julien Dehay
- Nephrology Dialysis Transplantation; Centre Hospitalier Universitaire de Rouen; Rouen Haute-Normandie France
| | - Julien Ott
- Department of Nephrology and Renal Transplantation; Hopitaux Universitaires de Strasbourg; Strasbourg Alsace France
| | - Rebecca Sberro
- Service de Transplantation et Unité de soins intensifs; Hôpital Necker; Paris France
| | | | - Nassim Kamar
- Department of Nephrology and Organ Transplantation Toulouse; CHU Rangueil; Haute Garonne France
| | | | | | - Laetitia Albano
- Nephrology-Dialysis-Transplantation; Nice University Hospital; Nice PACA France
| | - Sophie Girerd
- Nephrology Dialysis Transplantation; CHU Nancy; Nancy France
| | | | | | - Jacques Dantal
- Nephrology Dialysis Transplantation; Centre Hospitalier Universitaire; Nantes France
| | | | | | | | - Marc Hazzan
- Service de Néphrologie; CHRU Lille; Lille France
| | - Anne-Elisabeth Heng
- Service de Néphrologie; Hôpital Gabriel Montpied; CHU Clermont-Ferrand; Clermont-Ferrand France
| | - Christophe Mariat
- Nephrology Dialysis Transplantation; CHU Saint Etienne; Saint Etienne France
| | | | | | - Bruno Moulin
- Department of Nephrology and Renal Transplantation; Hopitaux Universitaires de Strasbourg; Strasbourg Alsace France
| | - Dominique Guerrot
- Nephrology Dialysis Transplantation; Centre Hospitalier Universitaire de Rouen; Rouen Haute-Normandie France
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20
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Balcan B, Simsek E, Ugurlu AO, Demiralay E, Sahin S. Sirolimus-Induced Diffuse Alveolar Hemorrhage: A Case Report. Am J Ther 2017; 23:e1938-e1941. [PMID: 26849007 DOI: 10.1097/mjt.0000000000000427] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Sirolimus is a mammalian target of the rapamycin, a protein kinase, which is responsible for inhibition of T cell and B cell proliferation. Sirolimus has side effects on lugs, and may cause cryptogenic organizing pneumonia, diffuse alveolar hemorrhage, lymphocytic pneumonitis, hypersensitivity pneumonitis, desquamative interstitial pneumonia, and pulmonary alveolar proteinosis. Diagnosis is based on the combination of clinical, radiological, histological, and pathological investigation. We report a case of diffuse alveolar hemorrhage in a 33-year-old, female renal transplant recipient. After discontinuation of sirolimus, radiological images and clinical condition of the patient got better. We also planned steroid therapy for 6 months by tapering the dosage slowly. After steroid therapy, full recovery of pulmonary functions achieved, and the patient is observed in our outpatient clinic with lack of any pulmonary symptoms.
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Affiliation(s)
- Baran Balcan
- Departments of 1Pulmonary Medicine, 2Nephrology, and 3Pathology, Faculty of Medicine, Baskent University, Istanbul, Turkey
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Fernández Morales A, Iniesta N, Fernández-Codina A, Vaz de Cunha J, Pérez Romero T, Hurtado García R, Simeón-Aznar CP, Fonollosa V, Cervera R, Espinosa G. Cardiac tamponade and severe pericardial effusion in systemic sclerosis: report of nine patients and review of the literature. Int J Rheum Dis 2016; 20:1582-1592. [PMID: 27943614 DOI: 10.1111/1756-185x.12952] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
AIM To describe the clinical characteristics, treatment and outcome of patients with systemic sclerosis (SSc) developing severe pericardial effusion or cardiac tamponade. METHODS SSc patients with severe pericardial effusion or cardiac tamponade from three Spanish hospitals were collected. In addition, a computer-assisted (PubMed, MEDLINE) search of the literature to identify all cases of cardiac tamponade or severe pericardial effusion associated with SSc reported in English, French and Spanish from 1987 through September 2015 was performed. RESULTS We included 40 patients (nine cases from the Spanish hospitals and 31 cases from the literature review). Most patients (87%) were female with a mean age at pericardial involvement of 49.3 ± 15.2 years (range: 18-80 years), and 22 (55%) patients had the diffuse cutaneous subtype. Twenty-five (63%) patients presented with cardiac tamponade and the remaining 15 (37%) as severe or massive pericardial effusion. Pericardial involvement was previous or simultaneous to SSc diagnosis in 13 (32.5%) cases. In most cases (88.9%) pericardial fluid analysis disclosed an exudate. Half the patients received steroids and 35% needed surgical treatment. Five (12.5%) patients died due to cardiac tamponade, three of them during the acute phase and the remaining two, 2 and 9 months later, respectively. CONCLUSIONS Although cardiac tamponade or severe pericardial effusion is an infrequent complication in SSc patients, it can be the first manifestation of disease associated with the diffuse cutaneous subset. No specific treatment for this complication is known.
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Affiliation(s)
| | - Nerea Iniesta
- Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain
| | | | | | | | | | | | - Vicent Fonollosa
- Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Catalonia, Spain
| | - Ricard Cervera
- Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain
| | - Gerard Espinosa
- Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain
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Abstract
Scleroderma renal crisis (SRC) is a rare, potentially life-threatening complication that affects 2-15% of patients with systemic sclerosis (SSc, also known as scleroderma). SRC typically presents in patients with early, rapidly progressive, diffuse cutaneous SSc within the first 3-5 years after the onset of a non-Raynaud sign or symptom. SRC is characterized by an acute, usually symptomatic increase in blood pressure, a rise in serum creatinine levels, oliguria and thrombotic microangiopathy in about 50% of patients. The prognosis of SRC substantially improved in the 1980s with the introduction of angiotensin-converting-enzyme inhibitors for rapid blood pressure control, with additional antihypertensive agents as required. However, the survival of patients with SRC can still be improved. Current patient survival is 70-82% at 1 year, but decreases to 50-60% at 5 years despite dialysis support. Patients with SRC who show no signs of renal functional recovery despite timely blood pressure control are candidates for transplantation. In this Review, we discuss progress made in the identification and proactive management of patients at risk of SRC and make recommendations aimed at optimizing management for those who progress to chronic kidney failure.
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Ghossein C, Varga J, Fenves AZ. Recent Developments in the Classification, Evaluation, Pathophysiology, and Management of Scleroderma Renal Crisis. Curr Rheumatol Rep 2016; 18:5. [PMID: 26711696 DOI: 10.1007/s11926-015-0551-y] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
Abstract
Scleroderma renal crisis (SRC) is an uncommon complication of systemic sclerosis. Despite the advent of angiotensin-converting inhibitor therapy, SRC remains a life-threatening complication. Recent studies have contributed to a better understanding of SRC, but much remains unknown regarding its pathophysiology, risk factors, and optimal management. Genetic studies provide evidence that immune dysregulation might be a contributing factor, providing hope that further research in this direction might illuminate pathogenesis and provide novel predictors for this complication.
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Affiliation(s)
- Cybele Ghossein
- Department of Medicine, Division of Nephrology, Northwestern University, Feinberg School of Medicine, 710 North Fairbanks Court-Suite 4500, Chicago, IL, 60611, USA.
| | - John Varga
- Department of Medicine, Northwestern University, Feinberg School of Medicine, 240 E Huron Street McGaw Pavilion Rheumatology M300, Chicago, IL, 60611, USA. .,Department of Medicine, Division of Rheumatology, Northwestern University, Feinberg School of Medicine, 240 E Huron Street McGaw Pavilion Rheumatology M300, Chicago, IL, 60611, USA.
| | - Andrew Z Fenves
- Department of Medicine, Massachusetts General Hospital, Harvard Medical School, 50 Staniford Street, Suite 503B, Boston, MA, 02114, USA.
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Affiliation(s)
- Efua Asamoah-Odei
- Internal Medicine-Nephrology, Christiana Care Health Services, Christiana Care Health System, Newark, Delaware, USA
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26
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[Prognosis and therapy of inflammatory rheumatic diseases : Impact of renal manifestations]. Z Rheumatol 2015; 74:310-21. [PMID: 25962452 DOI: 10.1007/s00393-014-1479-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
BACKGROUND Inflammatory rheumatic diseases and their treatment cause various renal manifestations requiring modification of treatment. OBJECTIVES Discussion of renal manifestations in selected rheumatic diseases, including their impact on general prognosis and therapy. MATERIALS AND METHODS Basic literature and expert opinions are analyzed and discussed. RESULTS Inflammatory rheumatic diseases and their treatment cause various renal manifestations, including glomerular, tubular, interstitial, and vascular damage. The type of damage determines both, associated clinical symptoms (i.e. hematuria, proteinuria, loss of kidney function) and the renal and overall survival as will be discussed here for rheumatoid arthritis, systemic lupus erythematosus, scleroderma, Sjögrens syndrome, cryoglobulinemia and ANCA-associated vasculitis. CONCLUSION Renal manifestations are generally indicators of high disease activity and usually require more intensive treatment of the underlying rheumatic disease. Early and rigorous treatment, which has to be adapted to renal function, is capable of improving renal and overall survival in many of the affected patients.
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27
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Stern EP, Steen VD, Denton CP. Management of Renal Involvement in Scleroderma. CURRENT TREATMENT OPTIONS IN RHEUMATOLOGY 2015. [DOI: 10.1007/s40674-014-0004-1] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
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Bose N, Chiesa-Vottero A, Chatterjee S. Scleroderma renal crisis. Semin Arthritis Rheum 2014; 44:687-94. [PMID: 25613774 DOI: 10.1016/j.semarthrit.2014.12.001] [Citation(s) in RCA: 89] [Impact Index Per Article: 8.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2014] [Revised: 11/28/2014] [Accepted: 12/05/2014] [Indexed: 12/23/2022]
Abstract
OBJECTIVES To discuss the pathophysiology, risk factors, clinical manifestations, diagnosis, treatment, prevention, and outcomes of scleroderma renal crisis (SRC), a serious yet potentially treatable complication of scleroderma (systemic sclerosis). METHODS A PubMed search for articles published up until April 2014 was conducted using the following keywords: scleroderma, systemic sclerosis, scleroderma renal crisis, renal, treatment, and prognosis. Literature was carefully reviewed, and different risk factors, treatment options, prognostic factors, and survival data were assessed. RESULTS SRC occurs in about 10% of all patients with scleroderma. It is characterized by malignant hypertension and progressive renal failure. Around 10% of SRC cases may present with normal blood pressure, termed normotensive renal crisis. The etiopathogenesis is presumed to be a series of insults to the kidneys resulting in endothelial injury, intimal proliferation, and narrowing of renal arterioles leading to decreased blood flow, hyperplasia of the juxtaglomerular apparatus, hyperreninemia, and accelerated hypertension. Risk factors include rapid skin thickening, use of certain medications such corticosteroids or cyclosporine, new-onset microangiopathic hemolytic anemia and/or thrombocytopenia, cardiac complications (pericardial effusion, congestive heart failure, and/or arrhythmias), large joint contractures, and presence of anti-RNA polymerase III antibody. Since the 1970s, with the advent of angiotensin-converting enzyme (ACE) inhibitors, mortality associated with SRC decreased from 76% to <10%. Some patients may progress to end-stage renal disease and need dialysis. Renal transplantation has improved survival, though SRC may recur in transplanted kidneys. CONCLUSIONS More than 60 years after its initial description, SRC still remains an important cause of morbidity and mortality in scleroderma. Since the advent of ACE inhibitors, the prognosis of SRC has improved substantially. Prompt diagnosis and treatment may help prevent adverse outcomes and improve survival.
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Affiliation(s)
- Nilanjana Bose
- Department of Rheumatic and Immunologic Diseases, Orthopaedic and Rheumatologic Institute, Cleveland Clinic, 9500 Euclid Ave, Desk A50, Cleveland, OH 44195
| | - Andres Chiesa-Vottero
- Department of Rheumatic and Immunologic Diseases, Orthopaedic and Rheumatologic Institute, Cleveland Clinic, 9500 Euclid Ave, Desk A50, Cleveland, OH 44195
| | - Soumya Chatterjee
- Department of Rheumatic and Immunologic Diseases, Orthopaedic and Rheumatologic Institute, Cleveland Clinic, 9500 Euclid Ave, Desk A50, Cleveland, OH 44195.
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La crise rénale sclérodermique. MEDECINE INTENSIVE REANIMATION 2014. [DOI: 10.1007/s13546-014-0929-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
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Abstract
Kidney involvement in systemic sclerosis (SSc) is primarily manifested by scleroderma renal crisis (SRC). Formerly, it was the most severe complication in scleroderma and was the most frequent cause of death in these patients. More than 30years ago, with the development of angiotensin converting enzyme (ACE) inhibitors, SRC became a very treatable complication of scleroderma. Although there are still many patients who do not survive and have poor outcomes, early diagnosis of renal crisis and prompt therapeutic intervention can achieve excellent outcomes. Renal abnormalities independent of renal crisis have been noted, but can usually be attributed to other problems. Further understanding of the pathogenesis of renal disease in scleroderma may lead to additional improvement in the therapy of renal crisis and perhaps the disease in general. This chapter reviews the pathogenesis, clinical setting, and therapy of this serious complication of SSc.
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Shu E, Kanoh H, Seishima M. Scleroderma renal crisis following pericardial effusion in a Japanese female. J Dermatol 2014; 41:824-6. [PMID: 25039404 DOI: 10.1111/1346-8138.12574] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2014] [Accepted: 06/16/2014] [Indexed: 11/28/2022]
Abstract
Scleroderma renal crisis (SRC) occurs in 4-5% of Japanese patients with systemic sclerosis (SSc) and is one of the most severe complications, along with interstitial pneumonia and pulmonary hypertension, which lead to a poor outcome. The factors predicting SRC include diffuse progressive skin thickening, a duration of SSc of 4 years or less, having autoantibodies against RNA polymerase III, systemic corticosteroid (CS) administration and recent cardiac events, including pericarditis. We herein report a female case of SRC preceded by pericardial effusion during the treatment with CS for rapidly progressive skin thickening. She had none of the known autoantibodies and was successfully treated with an angiotensin II receptor blocker.
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Affiliation(s)
- En Shu
- Department of Dermatology, School of Medicine, Gifu University, Gifu City, Japan
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Abstract
PURPOSE OF REVIEW The diagnosis of thrombotic microangiopathy (TMA) is complex and often difficult. This review provides an approach to the diagnosis with emphasis on recent relevant developments. RECENT FINDINGS There is increasing evidence that most cases of recurrent TMA in renal allografts are secondary to mutations in genes encoding complement regulatory factors and complement components, such as factor H, factor I, membrane cofactor protein, C3, and others. Genetic work-up for these potential complement abnormalities is now available and recommended. Another important cause for recurrent TMA is the presence of autoantibodies, such as antibodies to factor H and antiphospholipid antibodies. De-novo TMA is much more common than recurrent TMA in renal allografts. De-novo TMA can be secondary to calcineurin inhibitor treatment, mammalian target of rapamycin inhibitor treatment, but frequently also to antibody-mediated rejection and less commonly to infections. Systemic signs of TMA are often absent, and the gold standard for diagnosis is the renal allograft biopsy. Unfortunately, diagnostic criteria for TMA are somewhat subjective, and the biopsy provides limited information regarding the exact underlying cause. SUMMARY TMA is a serious complication of renal transplantation, usually with poor outcome. However, with improving understanding of underlying pathogeneses, more effective disease-specific therapeutic interventions can be designed. Appropriate treatment depends on the correct diagnosis, which relies primarily on renal allograft biopsy. Standardization of pathologic criteria and introduction of new molecular testing methods in renal biopsy specimens hopefully will improve diagnostic accuracy.
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Affiliation(s)
- Tibor Nadasdy
- Department of Pathology, The Ohio State University, Columbus, Ohio, USA
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Aburizik A, Singh S, Al-Rabadi L, Blosser C. Mistaken identity: normotensive scleroderma renal crisis. BMJ Case Rep 2014; 2014:bcr-2013-202566. [PMID: 24876209 DOI: 10.1136/bcr-2013-202566] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
A patient presented with neuromuscular, respiratory and cardiac symptoms and was initially diagnosed with amyotrophic lateral sclerosis (ALS), myocardial ischaemia and pneumonia. He developed unexplained progressive kidney failure over the ensuing week, and his kidney biopsy showed thrombotic microangiopathy that led to the correct diagnosis of normotensive scleroderma renal crisis. His clinical presentation and course were consistent with systemic sclerosis and normotensive scleroderma renal crisis. He was treated with an ACE inhibitor (ACEi) and haemodialysis with significant functional improvement over the next 3 months to his prior baseline with the exception of kidney failure. This case highlights a diagnostic challenge requiring astute history and physical examination skills, and the value of a kidney biopsy in providing the final diagnosis.
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Affiliation(s)
- Arwa Aburizik
- Department of Medicine, University of Iowa, Iowa City, Iowa, USA
| | - Siddharth Singh
- Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA
| | - Laith Al-Rabadi
- Department of Medicine, University of Iowa, Iowa City, Iowa, USA
| | - Christopher Blosser
- Department of Medicine-Nephrology, University of Washington, Seattle, Washington, USA
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Abstract
Scleroderma renal crisis (SRC) is characterized by malignant hypertension and oligo-anuric acute renal failure. It occurs in 5% of patients with systemic sclerosis (SSc), particularly in patients with diffuse disease during the first years. SRC is more common in patients receiving corticosteroids, the risk increasing with increasing dose. The disease is sometimes triggered by use of nephrotoxic drugs and/or intravascular volume depletion. Left ventricular insufficiency and hypertensive encephalopathy are typical clinical features. Thrombotic microangiopathy is detected in 43% of cases, and anti-RNA-polymerase III antibodies are present in one-third of patients. Renal biopsy is not necessary if SRC presents classical features. However, biopsy may help to define the prognosis and guide treatment in atypical forms. The prognosis of SRC has greatly improved with the introduction of angiotensin-converting enzyme (ACE) inhibitors. However, the 5-year survival for SSc patients with full SRC remains low (65%). The treatment of SRC relies on aggressive blood pressure control with an ACE inhibitor, combined with other antihypertensive drugs if needed. Dialysis is frequently indicated but can be stopped in about half of patients, mainly those with good blood pressure control. Patients who need dialysis for more than 2 years qualify for renal transplantation.
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Affiliation(s)
- Luc Mouthon
- From the Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP); Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; Université Paris Descartes, Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP, Paris, France.L. Mouthon, MD, PhD; G. Bussone, MD, PhD; A. Berezné, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; L-H. Noël, MD, Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes, and Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP; L. Guillevin, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes.
| | - Guillaume Bussone
- From the Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP); Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; Université Paris Descartes, Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP, Paris, France.L. Mouthon, MD, PhD; G. Bussone, MD, PhD; A. Berezné, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; L-H. Noël, MD, Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes, and Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP; L. Guillevin, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes
| | - Alice Berezné
- From the Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP); Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; Université Paris Descartes, Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP, Paris, France.L. Mouthon, MD, PhD; G. Bussone, MD, PhD; A. Berezné, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; L-H. Noël, MD, Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes, and Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP; L. Guillevin, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes
| | - Laure-Hélène Noël
- From the Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP); Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; Université Paris Descartes, Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP, Paris, France.L. Mouthon, MD, PhD; G. Bussone, MD, PhD; A. Berezné, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; L-H. Noël, MD, Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes, and Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP; L. Guillevin, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes
| | - Loïc Guillevin
- From the Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP); Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; Université Paris Descartes, Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP, Paris, France.L. Mouthon, MD, PhD; G. Bussone, MD, PhD; A. Berezné, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; L-H. Noël, MD, Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes, and Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP; L. Guillevin, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes
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Kronbichler A, Mayer G. Renal involvement in autoimmune connective tissue diseases. BMC Med 2013; 11:95. [PMID: 23557013 PMCID: PMC3616816 DOI: 10.1186/1741-7015-11-95] [Citation(s) in RCA: 72] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2012] [Accepted: 02/11/2013] [Indexed: 01/04/2023] Open
Abstract
Connective tissue diseases (CTDs) are a heterogeneous group of disorders that share certain clinical presentations and a disturbed immunoregulation, leading to autoantibody production. Subclinical or overt renal manifestations are frequently observed and complicate the clinical course of CTDs. Alterations of kidney function in Sjögren syndrome, systemic scleroderma (SSc), auto-immune myopathies (dermatomyositis and polymyositis), systemic lupus erythematosus (SLE), antiphospholipid syndrome nephropathy (APSN) as well as rheumatoid arthritis (RA) are frequently present and physicians should be aware of that.In SLE, renal prognosis significantly improved based on specific classification and treatment strategies adjusted to kidney biopsy findings. Patients with scleroderma renal crisis (SRC), which is usually characterized by severe hypertension, progressive decline of renal function and thrombotic microangiopathy, show a significant benefit of early angiotensin-converting-enzyme (ACE) inhibitor use in particular and strict blood pressure control in general. Treatment of the underlying autoimmune disorder or discontinuation of specific therapeutic agents improves kidney function in most patients with Sjögren syndrome, auto-immune myopathies, APSN and RA.In this review we focus on impairment of renal function in relation to underlying disease or adverse drug effects and implications on treatment decisions.
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Affiliation(s)
- Andreas Kronbichler
- Department of Internal Medicine IV, Nephrology and Hypertension, Medical University Innsbruck, Anichstraße 35, Innsbruck, 6020, Austria
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Renal disease in scleroderma: an update on evaluation, risk stratification, pathogenesis and management. Curr Opin Rheumatol 2013; 24:669-76. [PMID: 22955019 DOI: 10.1097/bor.0b013e3283588dcf] [Citation(s) in RCA: 70] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]
Abstract
PURPOSE OF REVIEW Renal disease remains an important cause of morbidity and mortality in scleroderma. The spectrum of renal complications in systemic sclerosis includes scleroderma renal crisis (SRC), normotensive renal crisis, antineutrophil cytoplasmic antibodies-associated glomerulonephritis, penacillamine-associated renal disease, and reduced renal functional reserves manifested by proteinuria, microalbuminuria, or isolated reduction in glomerular filtration rate. The purpose of this review is to provide a concise and up-to-date review of the evaluation, risk stratification, pathogenesis, and management of scleroderma-associated renal disease. RECENT FINDINGS Although SRC survival has significantly improved, mortality of this complication remains high outside of specialized centers. Recent data demonstrate strong associations between anti-RNA polymerase III antibodies and SRC. Subclinical renal impairment affects approximately 50% of scleroderma patients and may be associated with other vascular manifestations. Subclinical renal involvement rarely progresses to end-stage renal failure; however, recent studies suggest it may predict mortality in patients with other vasculopathic manifestations. SUMMARY Testing for anti-RNA polymerase III antibodies should be incorporated into clinical care to identify patients at high risk for SRC. Recommendations from European League Against Rheumatism (EULAR), EULAR Scleroderma Trials and Research, and the Scleroderma Clinical Trials Consortium confirm angiotensin-converting enzyme inhibitors as first-line therapy for SRC, and give recommendations for second-line agents.
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Scleroderma renal crisis: a rare but severe complication of systemic sclerosis. Clin Rev Allergy Immunol 2011; 40:84-91. [PMID: 20012923 DOI: 10.1007/s12016-009-8191-5] [Citation(s) in RCA: 46] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Abstract
Scleroderma renal crisis (SRC) is a major complication in patients with systemic sclerosis (SSc). It is characterized by malignant hypertension and oligo/anuric acute renal failure. SRC occurs in 5% of patients with SSc, particularly in the first years of disease evolution and in the diffuse form. The occurrence of SRC is more common in patients treated with glucocorticoids, the risk increasing with increasing dose. Left ventricular insufficiency and hypertensive encephalopathy are typical clinical features. Thrombotic microangiopathy is detected in 43% of the cases. Anti-RNA-polymerase III antibodies are present in one third of patients who develop SRC. Renal biopsy is not necessary if SRC presents with classical features. However, it can help to define prognosis and guide treatment in atypical forms. The prognosis of SRC has dramatically improved with the introduction of angiotensin-converting enzyme inhibitors (ACEi). However, 5 years survival in SSc patients who develop the full picture of SRC remains low (65%). SRC is often triggered by nephrotoxic drugs and/or intravascular volume depletion. The treatment of SRC relies on aggressive control of blood pressure with ACEi, if needed in combination with other types of antihypertensive drugs. Dialysis is frequently indicated, but can be stopped in approximately half of patients, mainly in those for whom a perfect control of blood pressure is obtained. Patients who need dialysis for more than 2 years qualify for renal transplantation.
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Pattanaik D, Brown M, Postlethwaite AE. Vascular involvement in systemic sclerosis (scleroderma). J Inflamm Res 2011; 4:105-25. [PMID: 22096374 PMCID: PMC3218751 DOI: 10.2147/jir.s18145] [Citation(s) in RCA: 35] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022] Open
Abstract
Systemic sclerosis (SSc) is an acquired multiorgan connective tissue disease with variable mortality and morbidity dictated by clinical subset type. The etiology of the basic disease and pathogenesis of the systemic autoimmunity, fibrosis, and fibroproliferative vasculopathy are unknown and debated. In this review, the spectrum of vascular abnormalities and the options currently available to treat the vascular manifestations of SSc are discussed. Also discussed is how the hallmark pathologies (ie, how autoimmunity, vasculopathy, and fibrosis of the disease) might be effected and interconnected with modulatory input from lysophospholipids, sphingosine 1-phosphate, and lysophosphatidic acid.
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Affiliation(s)
- Debendra Pattanaik
- Division of Connective Tissue Diseases, Department of Medicine, University of Tennessee Health Science Center, Memphis, TN, USA
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Bussone G, Bérezné A, Pestre V, Guillevin L, Mouthon L. The scleroderma kidney: progress in risk factors, therapy, and prevention. Curr Rheumatol Rep 2011; 13:37-43. [PMID: 21061100 DOI: 10.1007/s11926-010-0145-7] [Citation(s) in RCA: 33] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
Scleroderma renal crisis (SRC) is characterized by malignant hypertension, oliguric/anuric acute renal failure, and important mortality, with a 5-year survival rate of 65%. SRC occurs in 2% to 5% of patients with systemic sclerosis (SSc), particularly those with diffuse cutaneous SSc in the first years of disease evolution. Several retrospective studies have found high-dose corticosteroid therapy to be associated with increased risk of SRC, and anti-RNA-polymerase III antibodies have been detected in one third of patients with SRC. Treatment relies on the early control of blood pressure with increasing doses of angiotensin-converting enzyme inhibitors, eventually associated with calcium channel blockers together with dialysis if necessary. After 2 years on dialysis, eligible patients should be considered for renal transplantation. The strategy for prevention of SRC lacks consensus. However, corticosteroids and/or nephrotoxic drugs should be avoided in patients with diffuse cutaneous SSc.
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Affiliation(s)
- Guillaume Bussone
- Pôle de Médecine Interne, Hôpital Cochin, 27 Rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France
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Siva B, McDonald SP, Hawley CM, Rosman JB, Brown FG, Wiggins KJ, Bannister KM, Campbell SB, Johnson DW. End-stage kidney disease due to scleroderma--outcomes in 127 consecutive ANZDATA registry cases. Nephrol Dial Transplant 2011; 26:3165-71. [PMID: 21357212 DOI: 10.1093/ndt/gfq861] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022] Open
Abstract
BACKGROUND Scleroderma is an uncommon cause of end-stage kidney disease (ESKD) which carries significant morbidity and mortality risks. The aim of this study was to determine the prevalence, treatment and outcomes of scleroderma patients with ESKD. METHODS A study was conducted of all ESKD patients enrolled in the ANZDATA registry, who commenced dialysis between 15 May 1963 and 31 December 2005, and remained on dialysis for at least 90 days. RESULTS Of the 40 238 patients who commenced dialysis during the study period, 127 (0.3%) patients had ESKD secondary to scleroderma. Scleroderma ESKD patients were more likely than other ESKD patients to be female (72% versus 43%, P < 0.001), Caucasian (98% versus 79%, P < 0.001) and of lower BMI (22.7 ± 4.7 versus 26.0 ± 5.9, P < 0.001) with a higher prevalence of chronic lung disease (36 versus 14%, P < 0.001) and lower prevalence of diabetes mellitus (10% versus 32%, P < 0.001) and coronary artery disease (23% versus 35%, P = 0.01). Median survival was significantly shorter in scleroderma ESKD (2.43 years, 95% confidence interval (CI) 1.75-3.11 years) than other ESKD (6.02 years, 95% CI 5.89-6.14 years, log-rank score 55.7, P < 0.001). Renal recovery was more likely in scleroderma patients (10% versus 1%, P < 0.001) with a shorter time to recovery. Scleroderma was found to be an independent predictor for mortality (HR 2.47, 95% CI 1.99-3.05) and renal recovery (HR 11.1, 95% CI 6.37-19.4). Five year deceased donor and live donor renal allograft survival rates of recipients with scleroderma were 53 and 100%, respectively. CONCLUSIONS Scleroderma is an uncommon cause of ESKD, which is associated with increased risks of both spontaneous renal recovery and mortality.
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Zbiti N, Houssaini TS, Benkirane A, Alhamany Z, Rhou H, Benamar L, Ezaitouni F, Bayahia R, Ouzeddoun N. [Sclerodermic renal crisis: case report]. Nephrol Ther 2010; 6:606-9. [PMID: 20829139 DOI: 10.1016/j.nephro.2010.07.002] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2009] [Revised: 07/07/2010] [Accepted: 07/07/2010] [Indexed: 10/19/2022]
Abstract
Sclerodermic renal crisis is defined as a severe arterial hypertension or rapidly progressive glomerulonephritis in a sclerodermic patient, associated with anuria due to no other cause but systemic sclerodermia. It constitutes a rare and dangerous complication. The renal prognosis can be effectively improved by converting enzyme inhibitors, allowing a better arterial hypertension control. However, the associated mortality remains high, a follow up must be instaured in order to prevent the apparition of renal lesions in all sclerodermic patients. We report a case of rapidly progressive renal failure with hypertension in a patient with no previous problems, which preceded the development of scleroderma diffuse cutaneous form. The biopsy showed a microangiopathy in the context of scleroderma renal crisis. The evolution was marked by the presence of chronic renal insufficiency which necessitated the dialysing start.
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Affiliation(s)
- Najoua Zbiti
- Service de néphrologie-dialyse-transplantation rénale, CHU Ibn Sina, Rabat, Maroc.
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Scleroderma renal crisis: a pathology perspective. Int J Rheumatol 2010; 2010:543704. [PMID: 20981312 PMCID: PMC2958499 DOI: 10.1155/2010/543704] [Citation(s) in RCA: 62] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2010] [Accepted: 06/28/2010] [Indexed: 01/12/2023] Open
Abstract
Scleroderma renal crisis (SRC) is an infrequent but serious complication of systemic sclerosis (SSc). It is associated with increased vascular permeability, activation of coagulation cascade, and renin secretion, which may lead to the acute renal failure typically associated with accelerated hypertension. The histologic picture of SRC is that of a thrombotic microangiopathy process with prominent small vessel involvement manifesting as myxoid intimal changes, thrombi, onion skin lesions, and/or fibrointimal sclerosis. Renal biopsies play an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in SSc patients, helping to predict the clinical outcome and optimizing patient management. Kidney transplantation may be the only treatment option available for a subset of SRC patients who develop end-stage renal failure despite aggressive angiotensin-converting enzyme inhibitor therapy. However, the posttransplant outcome for SSc patients is currently suboptimal compared to the general renal transplant population.
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Bussone G, Berezné A, Mouthon L. Complications infectieuses de la sclérodermie systémique. Presse Med 2009; 38:291-302. [DOI: 10.1016/j.lpm.2008.11.003] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/14/2008] [Accepted: 11/17/2008] [Indexed: 12/29/2022] Open
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Abstract
The pathogenic process of systemic sclerosis targets the skin and internal organs, and involves sequential or concomitant abnormalities in blood vessel function, in immunity and inflammation, and in subsequent fibroblast activation. These characteristics are disease-specific and may partly explain the unresolved therapeutic strategies. These latter must take into account not only the broad biological abnormalities but also the complexities of the disturbances throughout the duration of the disease. However, recent epidemiological data have suggested a decrease in excess mortality, which may be mostly due to the use of cardiovascular drugs. Indeed, the prognoses of the kidney, heart and pulmonary vascular involvements have been improved. Nevertheless and despite several trials, pulmonary fibrosis remains a challenge with no clear efficacy of the regimen of immunosuppressors that have been investigated. This article deals with current and possible future therapeutic options.
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Affiliation(s)
- Yannick Allanore
- Paris Descartes University, Medical Faculty, Department of Rheumatology A, Cochin Hospital, AP-HP and INSERM U781, Necker Hospital, Paris, France.
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Desai S, Clement J, Landaw S. A 51-year-old woman with shortness of breath and edema. ACTA ACUST UNITED AC 2008; 59:1184-90. [DOI: 10.1002/art.23934] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
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Teixeira L, Mahr A, Berezné A, Noël LH, Guillevin L, Mouthon L. Scleroderma renal crisis, still a life-threatening complication. Ann N Y Acad Sci 2007; 1108:249-58. [PMID: 17893990 DOI: 10.1196/annals.1422.027] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
Abstract
Scleroderma renal crisis (SRC) is a major complication in patients with systemic sclerosis (SSc). SRC occurs during the first 4 years of disease evolution in more than 75% of the cases, almost exclusively in patients with diffuse SSc. Other risk factors, including preceding corticosteroid therapy, have been associated with an increased occurrence of SRC. Prior to the late 1970s, SRC was a major cause of death in SSc patients. However, the prognosis has dramatically improved with the introduction of angiotensin-converting enzyme inhibitors. Thus, prompt diagnosis and early initiation of angiotensin-converting enzyme inhibitors therapy will improve the outcome. Nevertheless, additional antihypertensive treatments are often needed. Quite a large proportion of patients require dialysis, although this therapy may be stopped in approximately one-third of patients. Patients remaining on dialysis after 2 years can be proposed for a renal transplantation.
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Affiliation(s)
- Luis Teixeira
- Department of Internal Medicine and Reference Center for Necrotizing Vasculitis and Systemic Sclerosis, Cochin Hospital, Assistance Publique-Hópitaux de Paris (AP-HP), and UPRES EA 4058, Paris-Descartes University, Faculty of Medicine, Paris, France
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Abstract
Scleroderma renal crisis (SRC) occurs in patients with systemic sclerosis (SSc) and is defined by otherwise unexplained rapidly progressive renal insufficiency associated with oliguria or rapidly progressive arterial hypertension or both. SRC is a rare and severe complication of SSc, most often encountered during the first 4 years of disease, almost only in patients with diffuse SSc. Factors predicting SRC were identified, including high-dose corticosteroid administration. Use of angiotensin-converting enzyme inhibitors (ACEI) has dramatically impressed the prognosis of SRC, but it mortality rate is still high. Treatment aims at normalizing blood pressure as soon as possible. ACEI should always be used, and additional anti-hypertensive agents, including calcium channel blockers and alpha- and beta-blockers, may be useful. Renal replacement therapy may be needed, but often (for almost half of patients) only temporarily.
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Affiliation(s)
- Luis Teixeira
- Service de Médecine Interne, Hôpital Cochin, Centre de Référence pour les Vascularites Nécrosantes et la Sclérodermie Systémique, Assistance Publique-Hôpitaux de Paris (AP-HP) et Université Paris-Descartes, Paris
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Recurrent and de novo disease in kidney, heart, lung, pancreas and intestinal transplants. Curr Opin Organ Transplant 2006. [DOI: 10.1097/01.mot.0000227848.67570.50] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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