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Kao WC, Hu YC, Lee JH, Wang LC, Lin YT, Yang YH, Chiang BL, Yu HH. Serositis as an indicator of poor prognosis in pediatric systemic lupus erythematosus. Pediatr Rheumatol Online J 2025; 23:36. [PMID: 40170018 PMCID: PMC11959889 DOI: 10.1186/s12969-025-01084-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/14/2024] [Accepted: 03/18/2025] [Indexed: 04/03/2025] Open
Abstract
BACKGROUND Systemic lupus erythematosus (SLE) is a multi-systemic autoimmune disease that causes inflammation of the serosa (serositis). This retrospective study aimed to evaluate the clinical characteristics of serositis in childhood-onset SLE (cSLE) and analyze its association with long-term outcomes. METHODS We retrospectively reviewed the medical records of patients with cSLE diagnosed at a medical center in Taiwan, analyzing data collected from January 2002 to December 2022. We analyzed the clinical features of patients with serositis as pleuritis and/or pericarditis with at least a small effusion (> 0.5 cm in depth) on sonography or chest radiography. Cox proportional hazards regression was used to calculate the hazard ratios (HR) and 95% confidence intervals (CI) for the association between serositis and all-cause mortality. RESULTS 185 patients with cSLE were enrolled, of whom 38 (20.54%) had serositis. Patients with serositis had a younger age at SLE diagnosis, a higher SLE Disease Activity Index 2000 score at serositis diagnosis, and an increased prevalence of lupus nephritis, central nervous system manifestations, end-stage renal disease (ESRD), a higher Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) damage index score, and a higher mortality than that of patients without serositis. Multivariate Cox regression analysis showed that both serositis (hazard ratio [HR]: 5.585, confidence interval [CI]: 1.853-17.80) and ESRD (HR: 13.956; CI: 3.822-50.964) were associated with mortality risk. Kaplan-Meier survival curve analysis revealed that patients with both serositis and ESRD had the poorest 20-year survival rate. Patients with late-onset serositis (occurring 1 year after SLE diagnosis) had higher mortality rates than those with early-onset serositis. CONCLUSION Children with lupus serositis had higher disease activity, a higher prevalence of comorbidities, and mortality. Patients with both serositis, especially late-onset serositis, and ESRD had an increased risk of poor long-term survival.
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Affiliation(s)
- Wei-Chen Kao
- Department of Pediatrics, National Taiwan University Children's Hospital, No. 7, Zhongshan S. Road, Zhongzheng District, Taipei City, 100, Taiwan
- Department of Pediatrics, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei, Taiwan
| | - Ya-Chiao Hu
- Department of Pediatrics, National Taiwan University Children's Hospital, No. 7, Zhongshan S. Road, Zhongzheng District, Taipei City, 100, Taiwan
| | - Jyh-Hong Lee
- Department of Pediatrics, National Taiwan University Children's Hospital, No. 7, Zhongshan S. Road, Zhongzheng District, Taipei City, 100, Taiwan
| | - Li-Chieh Wang
- Department of Pediatrics, National Taiwan University Children's Hospital, No. 7, Zhongshan S. Road, Zhongzheng District, Taipei City, 100, Taiwan
| | - Yu-Tsan Lin
- Department of Pediatrics, National Taiwan University Children's Hospital, No. 7, Zhongshan S. Road, Zhongzheng District, Taipei City, 100, Taiwan
| | - Yao-Hsu Yang
- Department of Pediatrics, National Taiwan University Children's Hospital, No. 7, Zhongshan S. Road, Zhongzheng District, Taipei City, 100, Taiwan
| | - Bor-Luen Chiang
- Department of Medical Research, National Taiwan University Hospital, Taipei, Taiwan
- Genome and Systems Biology Degree Program, College of Life Science, National Taiwan University, Taipei, Taiwan
| | - Hsin-Hui Yu
- Department of Pediatrics, National Taiwan University Children's Hospital, No. 7, Zhongshan S. Road, Zhongzheng District, Taipei City, 100, Taiwan.
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Boever S, Bannis K. A Case Report: Unveiling the Underlying Cause of Recurrent Pericarditis in a Patient With Undifferentiated Connective Tissue Disease. Cureus 2024; 16:e54113. [PMID: 38487125 PMCID: PMC10938869 DOI: 10.7759/cureus.54113] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/13/2024] [Indexed: 03/17/2024] Open
Abstract
Undifferentiated connective tissue disease (UCTD) is a condition characterized by symptoms and laboratory findings related to various systematic autoimmune diseases. Severe symptoms like chest pain in patients with UCTD could suggest an underlying secondary condition, such as pericarditis. Our case involves a 36-year-old woman with a history of UCTD and recently diagnosed rheumatoid arthritis (RA) who presented with persistent sub-sternal chest pain and pressure that began three weeks ago. Over the past year, she experienced six similar episodes of chest pain, diagnosed as idiopathic pericarditis. She promptly underwent treatment with oral prednisone and was instructed to continue her current medications (colchicine, methotrexate, and Plaquenil). Subsequent laboratory results, obtained several days posttreatment, revealed an elevated C-reactive protein (CRP), normal erythrocyte sedimentation rate (ESR), an elevated rheumatoid factor, and a normal echocardiogram, suggesting resolution of the acute flare. Despite having a comprehensive treatment regimen, the patient continues to experience recurrent pericarditis episodes. The cause of the recurrence remains uncertain, potentially associated with repeated use of high-dose steroids and a recent diagnosis of RA. Consequently, her rheumatologist opted to initiate treatment with intravenous Golimumab to better manage the RA and potentially address recurrent pericarditis. Physicians should maintain a heightened clinical suspicion of pericarditis in UCTD patients experiencing chest pain, as initiating prompt treatment helps prevent long-term complications and can be lifesaving in certain instances.
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Affiliation(s)
- Samantha Boever
- Internal Medicine, A.T. Still University School of Osteopathic Medicine, Mesa, USA
| | - Kareem Bannis
- Internal Medicine, Adelante Healthcare, Phoenix, USA
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Dawi J, Affa S, Misakyan Y, Fardeheb S, Kades S, Kiriaki A, Mohan AS, Norris B, Yoon S, Venketaraman V. Exploring cardiovascular implications in systemic lupus erythematosus: A holistic analysis of complications, diagnostic criteria, and therapeutic modalities, encompassing pharmacological and adjuvant approaches. Biomol Concepts 2024; 15:bmc-2022-0051. [PMID: 39603656 PMCID: PMC11994118 DOI: 10.1515/bmc-2022-0051] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/11/2024] [Accepted: 10/21/2024] [Indexed: 11/29/2024] Open
Abstract
Systemic lupus erythematosus (SLE) poses a diagnostic challenge due to its heterogeneity. This study examines the cardiac complications of SLE comprehensively, covering pericarditis, myocarditis, pleural effusion, valvular disease, atherosclerosis, and cardiac arrhythmias. Nearly one-third of SLE-related deaths are attributed to cardiovascular diseases, necessitating a deeper understanding of cardiac pathophysiology. The impact of SLE on the cardiovascular system manifests in various ways, including recurrent and resistant pericarditis, severe myocarditis, and pleural effusion. Valvular diseases, atherosclerosis, and cardiac arrhythmias are prevalent, with immune complex deposition playing a role in atherosclerosis. Diagnostic criteria involve clinical features, laboratory findings, and autoantibodies, emphasizing the need for early diagnosis and a multidisciplinary diagnostic approach. The review explores pharmacological and non-pharmacological modalities for managing cardiac manifestations in SLE. Recommendations include NSAIDs, colchicine, and proton pump inhibitors for acute pericarditis, while selective immunosuppressive therapy is emerging for myocarditis. Valvular diseases require individualized treatment approaches, and careful corticosteroid management is crucial to avoid increased cardiovascular events. Anti-malarial therapy, particularly hydroxychloroquine, shows promise in mitigating cardiovascular risk factors. Non-pharmacological modifications, such as diet, exercise, and smoke cessation, significantly contribute to cardiovascular health in SLE patients. Adjuvant therapies involving glutathione and glutathione peroxidase focus on redox balance, offering potential interventions. This integrated approach combines diagnostic insights with diverse treatment modalities, providing a holistic strategy for managing cardiac complications in SLE. Ongoing research is essential to refine these strategies and optimize individualized treatment plans for improved patient outcomes.
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Affiliation(s)
- John Dawi
- College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, CA, 91766, United States of America
| | - Scarlet Affa
- Los Angeles Valley College, Valley Glen, CA, 91401, United States of America
| | - Yura Misakyan
- College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, CA, 91766, United States of America
| | - Sabrina Fardeheb
- College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, CA, 91766, United States of America
| | - Samuel Kades
- College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, CA, 91766, United States of America
| | - Anthony Kiriaki
- College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, CA, 91766, United States of America
| | - Aishvaryaa Shree Mohan
- College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, CA, 91766, United States of America
| | - Brandon Norris
- College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, CA, 91766, United States of America
| | - Sonyeol Yoon
- College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, CA, 91766, United States of America
| | - Vishwanath Venketaraman
- College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, CA, 91766, United States of America
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Pan SY, Tian HM, Zhu Y, Gu WJ, Zou H, Wu XQ, Cheng RJ, Yang Z. Cardiac damage in autoimmune diseases: Target organ involvement that cannot be ignored. Front Immunol 2022; 13:1056400. [PMID: 36483559 PMCID: PMC9722763 DOI: 10.3389/fimmu.2022.1056400] [Citation(s) in RCA: 10] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2022] [Accepted: 11/07/2022] [Indexed: 11/23/2022] Open
Abstract
Autoimmune diseases are diseases that cause damage to the body's own tissues as a result of immune dysfunction, often involving multiple organs and systems. The heart is one of the common target organs of autoimmune diseases. The whole structure of the heart can be affected, causing microcirculatory disorders, arrhythmias, pericardial damage, myocarditis, myocardial fibrosis, and impaired valvular function. However, early clinical manifestations of autoimmune heart damage are often overlooked because they are insidious or have no typical features. The damage is often severe and irreversible when symptoms are apparent, even life-threatening. Therefore, early detection and treatment of heart damage in autoimmune diseases is particularly important. Herein, we review the clinical features and mechanisms of cardiac damage in common rheumatic diseases.
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Affiliation(s)
- Shu-Yue Pan
- Department of Rheumatology and Immunology, The Fifth People’s Hospital Affiliated to Chengdu University of Traditional Chinese Medicine/Chengdu Fifth People’s Hospital, Chengdu, China
| | - Hui-Min Tian
- Department of Rheumatology and Immunology, The Fifth People’s Hospital Affiliated to Chengdu University of Traditional Chinese Medicine/Chengdu Fifth People’s Hospital, Chengdu, China
| | - Yong Zhu
- Department of Rheumatology and Immunology, The Fifth People’s Hospital Affiliated to Chengdu University of Traditional Chinese Medicine/Chengdu Fifth People’s Hospital, Chengdu, China
| | - Wei-Jie Gu
- Department of Rheumatology and Immunology, The Fifth People’s Hospital Affiliated to Chengdu University of Traditional Chinese Medicine/Chengdu Fifth People’s Hospital, Chengdu, China
| | - Hao Zou
- Department of Rheumatology and Immunology, The Fifth People’s Hospital Affiliated to Chengdu University of Traditional Chinese Medicine/Chengdu Fifth People’s Hospital, Chengdu, China
| | - Xu-Qiang Wu
- Department of Rheumatology and Immunology, The Fifth People’s Hospital Affiliated to Chengdu University of Traditional Chinese Medicine/Chengdu Fifth People’s Hospital, Chengdu, China
| | - Rui-Juan Cheng
- Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Zhi Yang
- Department of Radiology, The Fifth People’s Hospital Affiliated to Chengdu University of Traditional Chinese Medicine/Chengdu Fifth People’s Hospital, Chengdu, China
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Santacruz JC, Mantilla MJ, Rueda I, Rodríguez-Salas G, Pulido S, Londono J. Perimyocarditis With Acute Heart Failure as the First Manifestation of Systemic Lupus Erythematosus. Cureus 2022; 14:e26707. [PMID: 35959185 PMCID: PMC9360625 DOI: 10.7759/cureus.26707] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/09/2022] [Indexed: 11/30/2022] Open
Abstract
Cardiac abnormalities are common in patients with systemic lupus erythematosus (SLE). However, many of them tend to be mild or asymptomatic and can be recognized by non-invasive studies such as transthoracic echocardiography and cardiac magnetic resonance imaging (CMR). However, heart failure secondary to perimyocarditis as the initial manifestation of SLE remains an extremely rare form of presentation. Below, we present the case of an adult female patient who initially consulted due to symptoms of acute dyspnea, atypical chest pain, and edema of the lower limbs, who underwent a chest X-ray as part of the initial studies, which described an increase in the cardiac silhouette associated with diffuse opacities in both lung fields. The admission electrocardiogram only showed sinus tachycardia and nonspecific alterations of the T wave, with an initial report of frankly elevated cardiac biomarkers compatible with acute myocardial injury together with the positivity of specific antibodies for SLE.
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Pieta A, Pelechas E, Gerolymatou N, Voulgari PV, Drosos AA. Calcified constrictive pericarditis resulting in tamponade in a patient with systemic lupus erythematosus. Rheumatol Int 2020; 41:651-670. [PMID: 33206224 DOI: 10.1007/s00296-020-04747-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/13/2020] [Accepted: 10/31/2020] [Indexed: 10/23/2022]
Abstract
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with multiorgan involvement, including heart. Pericarditis-the most common cardiac manifestation-occurs in up to 50% of cases, resulting in positive treatment outcomes. Rarely, it evolves to hazardous complications. A 50-year-old woman with SLE in clinical remission, receiving hydroxychloroquine 400 mg/day, presented to us with severe chest pain and low-grade fever. Physical examination revealed a friction rub and decreased breath sounds at the right lung base. Laboratory evaluation demonstrated leukopenia, thrombocytopenia, low C4 levels, and high acute phase reactants. Chest X-ray exhibited cardiomegaly, calcified pericardium, and right pleural effusion, confirmed by CT scan. PPD skin test and IGRA were both negative. Pericardial fluid, blood, and urine cultures for bacteria and fungi, as well as Gram and Ziehl-Neelsen stains were negative. Serological tests for viruses were also negative. The patient was diagnosed with calcified constrictive pericarditis (CP) due to SLE. She was treated with cyclophosphamide and methylprednisolone pulses, without improvement. Her clinical condition deteriorated, developing signs and symptoms compatible with cardiac tamponade (TMP), which was confirmed by Doppler echocardiography. The patient underwent pericardiectomy. A dramatic response was noted and she was discharged with prednisone 50 mg/day and azathioprine 100 mg/day. Thus, we review and discuss the relevant literature of SLE cases with CP or TMP. When an SLE patient presents with CP, infectious causes should be excluded first. To the best of our knowledge, this is the only case of SLE and calcified CP leading to TMP, hence physicians should be aware of this complication.
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Affiliation(s)
- Antigone Pieta
- Rheumatology Clinic, Department of Internal Medicine, School of Health Sciences, University Hospital of Ioannina, University of Ioannina, 45110, Ioannina, Greece
| | - Eleftherios Pelechas
- Rheumatology Clinic, Department of Internal Medicine, School of Health Sciences, University Hospital of Ioannina, University of Ioannina, 45110, Ioannina, Greece
| | - Nafsika Gerolymatou
- Rheumatology Clinic, Department of Internal Medicine, School of Health Sciences, University Hospital of Ioannina, University of Ioannina, 45110, Ioannina, Greece
| | - Paraskevi V Voulgari
- Rheumatology Clinic, Department of Internal Medicine, School of Health Sciences, University Hospital of Ioannina, University of Ioannina, 45110, Ioannina, Greece
| | - Alexandros A Drosos
- Rheumatology Clinic, Department of Internal Medicine, School of Health Sciences, University Hospital of Ioannina, University of Ioannina, 45110, Ioannina, Greece.
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Abstract
PURPOSE OF REVIEW We review the epidemiology, pathophysiology, and management of pericarditis most commonly complicating autoimmune and autoinflammatory conditions. RECENT FINDINGS Typically, pericarditis occurs in the context of a systemic flare of the underlying disease but infrequently, it is the presenting manifestation requiring a high index of suspicion to unravel the indolent cause. Pericardial involvement in rheumatic diseases encompasses a clinical spectrum to include acute, recurrent and incessant pericarditis, constrictive pericarditis, asymptomatic pericardial effusion, and pericardial tamponade. Direct evidence on the pathophysiology of pericarditis in the context of rheumatic diseases is scant. It is theorized that immune perturbations within pericardial tissue result from the underlying central immunopathology of the respective autoimmune or autoinflammatory disease. Pericarditis management depends on acuity, the underlying cause and epidemiological features such as patient's immune status and geographic prevalence of infections such as tuberculosis. Immunosuppressive medications including biologics such as interleukin 1 blockers emerge as possible steroid sparing agents for pericarditis treatment.
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Affiliation(s)
- Apostolos Kontzias
- Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Stony Brook University School of Medicine, Stony Brook, NY, USA.
| | - Amir Barkhodari
- Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Stony Brook University School of Medicine, Stony Brook, NY, USA
| | - QingPing Yao
- Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Stony Brook University School of Medicine, Stony Brook, NY, USA
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Jia E, Geng H, Liu Q, Xiao Y, Zhang Y, Xie J, Zhang L, Qiu X, Zhong L, Xiao M, Zhang J. Cardiac manifestations of Han Chinese patients with systemic lupus erythematosus: a retrospective study. Ir J Med Sci 2018; 188:801-806. [PMID: 30460452 DOI: 10.1007/s11845-018-1934-7] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2018] [Accepted: 11/13/2018] [Indexed: 12/28/2022]
Abstract
OBJECTIVE To investigate cardiac manifestations and the risk factors in Han Chinese patients with systemic lupus erythematosus (SLE). METHODS Seven hundred fifty SLE patients who were hospitalized at our department were recruited in the present study. The patients were divided into two groups-those with or without cardiac manifestations. Cardiac manifestations in those SLE patients, such as pericarditis, myocarditis, heart valve disease, arrhythmia, were analyzed. The risk and protective factors of cardiac diseases in patients with SLE, as well as the predictors of mortality, were assessed, respectively. RESULTS In all 750 SLE patients, there were 339 (45.20%) patients suffered from one or more cardiac manifestations, involving pericarditis in 9.5%, myocarditis in 5.7%, heart valve disease in 15.6%, arrhythmia in 16.67%, and cardiovascular diseases (CVD) in 14%. 15.7% of SLE patients were accompanied with pulmonary arterial hypertension (PAH), of which 13.7% were mild, 1.2% were moderate, and 0.8% were severe. No significant differences were found between the two groups in age, disease duration, gender, antibody, and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). The incidence of pericarditis, heart valve disease, arrhythmia, and PAH was positively correlated with age. The incidence of arrhythmia, CVD, and PAH was correlated with SLEDAI. PAH and myocarditis were the risk factors of mortality in SLE patients with disease duration ≤ 10 years (P = 0.034 and 0.001, respectively). CONCLUSION Cardiac involvement is common in Han Chinese SLE patients and associated with age and disease activity. PAH and myocarditis are the risk factors of mortality in SLE.
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Affiliation(s)
- Ertao Jia
- The Department of Rheumatology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese Medicine, No. 1, Fuhua Road, Futian District, Shenzhen, 518033, Guangdong, China.
| | - Hongling Geng
- The Department of Gynecology, Guangdong Provincial Hospital of Chinese Medicine, The Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, 510120, Guangdong, China
| | - Qingping Liu
- The Department of Rheumatology, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, 510405, Guangdong, China
| | - Yuya Xiao
- The Department of Rheumatology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese Medicine, No. 1, Fuhua Road, Futian District, Shenzhen, 518033, Guangdong, China
| | - Yanying Zhang
- The Department of Rheumatology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese Medicine, No. 1, Fuhua Road, Futian District, Shenzhen, 518033, Guangdong, China
| | - Jingjing Xie
- The Department of Rheumatology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese Medicine, No. 1, Fuhua Road, Futian District, Shenzhen, 518033, Guangdong, China
| | - Luhe Zhang
- The Department of Rheumatology, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, 510405, Guangdong, China
| | - Xia Qiu
- The Department of Rheumatology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese Medicine, No. 1, Fuhua Road, Futian District, Shenzhen, 518033, Guangdong, China
| | - Li Zhong
- The Department of Rheumatology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese Medicine, No. 1, Fuhua Road, Futian District, Shenzhen, 518033, Guangdong, China
| | - Min Xiao
- The Department of Rheumatology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese Medicine, No. 1, Fuhua Road, Futian District, Shenzhen, 518033, Guangdong, China
| | - Jianyong Zhang
- The Department of Rheumatology, Shenzhen Traditional Chinese Medicine Hospital, The Fourth Clinical Medical College of Guangzhou University of Chinese Medicine, No. 1, Fuhua Road, Futian District, Shenzhen, 518033, Guangdong, China.
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Ramasamy V, Mayosi BM, Sturrock ED, Ntsekhe M. Established and novel pathophysiological mechanisms of pericardial injury and constrictive pericarditis. World J Cardiol 2018; 10:87-96. [PMID: 30344956 PMCID: PMC6189073 DOI: 10.4330/wjc.v10.i9.87] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/08/2018] [Revised: 04/06/2018] [Accepted: 04/22/2018] [Indexed: 02/06/2023] Open
Abstract
This review article aims to: (1) discern from the literature the immune and inflammatory processes occurring in the pericardium following injury; and (2) to delve into the molecular mechanisms which may play a role in the progression to constrictive pericarditis. Pericarditis arises as a result of a wide spectrum of pathologies of both infectious and non-infectious aetiology, which lead to various degrees of fibrogenesis. Current understanding of the sequence of molecular events leading to pathological manifestations of constrictive pericarditis is poor. The identification of key mechanisms and pathways common to most fibrotic events in the pericardium can aid in the design and development of novel interventions for the prevention and management of constriction. We have identified through this review various cellular events and signalling cascades which are likely to contribute to the pathological fibrotic phenotype. An initial classical pattern of inflammation arises as a result of insult to the pericardium and can exacerbate into an exaggerated or prolonged inflammatory state. Whilst the implication of major drivers of inflammation and fibrosis such as tumour necrosis factor and transforming growth factor β were foreseeable, the identification of pericardial deregulation of other mediators (basic fibroblast growth factor, galectin-3 and the tetrapeptide Ac-SDKP) provides important avenues for further research.
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Affiliation(s)
- Vinasha Ramasamy
- Institute of Infectious Disease and Molecular Medicine, University of Cape Town, Observatory 7925, South Africa
- Department of Integrative Biomedical Sciences, University of Cape Town, Observatory 7925, South Africa
| | - Bongani M Mayosi
- Institute of Infectious Disease and Molecular Medicine, University of Cape Town, Observatory 7925, South Africa
- Division of Cardiology, University of Cape Town, Observatory 7925, South Africa
| | - Edward D Sturrock
- Institute of Infectious Disease and Molecular Medicine, University of Cape Town, Observatory 7925, South Africa
- Department of Integrative Biomedical Sciences, University of Cape Town, Observatory 7925, South Africa
| | - Mpiko Ntsekhe
- Institute of Infectious Disease and Molecular Medicine, University of Cape Town, Observatory 7925, South Africa
- Division of Cardiology, University of Cape Town, Observatory 7925, South Africa
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Immunological and inflammatory processes in systemic autoimmune disease may not only cause pericardium inflammation, but may also cause mitral valve deterioration and left ventricular wall thickening. Int J Cardiol 2016; 215:466-71. [PMID: 27131766 DOI: 10.1016/j.ijcard.2016.04.126] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/14/2016] [Revised: 04/12/2016] [Accepted: 04/13/2016] [Indexed: 11/22/2022]
Abstract
PURPOSE Systemic autoimmune disease (SAD) frequently affects the pericardium, and pathology is characterized by both immunological and inflammatory processes. We hypothesized that these processes simultaneously influence mitral-valve (MV) deterioration and left-ventricular (LV) wall thickening in SAD subjects. METHODS 101 SAD subjects were selected (76 female; 53±17years; systemic-lupus-erythematosus, 26%; vasculitis, 20%; scleroderma, 14%; polymyositis/dermatomyositis complex, 10%; mixed connective tissue disease, 11% and rheumatoid-arthritis, 2%). MV anterior-mitral-leaflet (AML) length, AML thickness index, AML doming height and LV mass index (LVMI) were measured using transthoracic-echocardiography (TTE) and the presence of MV calcification, MV sub-valvular thickening and pericardial effusion (PE) were estimated. AML thickness index was calculated as the ratio of AML thickness to aortic posterior wall thickness. The correlation between LVMI and ECG V1S+V5R voltage was used to assess the etiology of LV wall thickening. RESULTS 19 subjects (19%) had significant PE. PE subjects had a significantly greater AML thickness index (1.55±0.48 vs. 1.14±0.32, P<0.001), AML doming height (1.26±1.54mm vs. 0.03±0.91mm, P<0.001), more frequent MV sub-valvular thickening (26% vs. 5%, P=0.003) and greater LVMI (104.7±34.6g/m2 vs. 80.6±21.0g/m2, P=0.002). Significant correlation was observed between LVMI and ECG V1S+V5R voltage in 79 subjects without PE (R=0.39, P<0.001). However, in 18 subjects with PE, no such correlation was observed (R=0.30, P=0.23). CONCLUSIONS MV, MV sub-valvular deterioration and increased LVMI, unrelated to high voltage ECG criteria, were frequently detected in SAD subjects with PE. Immunological and inflammatory processes in SAD may not only cause pericardium inflammation, but may also cause MV deterioration and LV wall thickening.
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Abstract
The heart is one of the most frequently affected organs in SLE. Any part of the heart can be affected, including the pericardium, myocardium, coronary arteries, valves, and the conduction system. In addition to pericarditis and myocarditis, a high incidence of CAD has become increasingly recognized as a cause of mortality, especially in older adult patients and those with long-standing SLE. Many unanswered questions remain in terms of understanding the pathogenesis of cardiac manifestations of SLE. It is not currently possible to predict the patients who are at greatest risk for the various types of cardiac involvement. However, with the rapid advancement of basic science and translational research approaches, it is now becoming easier to identify specific mutations associated with SLE. A better understanding of these genetic factors may eventually allow clinicians to categorize and predict the patients who are at risk for specific cardiac manifestations of SLE.
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Abstract
The objective of this study was to describe the prevalence and outcome of disease-related serositis in Chinese patients with systemic lupus erythematosus (SLE). The records of all SLE patients who attended the medical clinics of Tuen Mun Hospital, Hong Kong were retrospectively reviewed. Patients with disease-related serositis at any stage of their illness were identified and the outcome of these serositis episodes was reported. Three-hundred and ten patients (90% women) who fulfilled at least four of the ACR criteria for SLE were studied. The mean age of SLE onset was 32.6 +/- 13.1 years. sixty-nine episodes of SLE-related serositis occurred in 37 patients - 18 (26%) episodes were pericarditis/ pericardial effusion, 30 (44%) were pleuritis/pleural effusion and 21 (30%) were peritonitis/ascites. The prevalence of serositis was 12%. At the time of serositis, 34 (92%) patients had active SLE in other systems. Nonsteroidal anti-inflammatory drugs (NSAIDs) were initially used in 13 (35%) patients. Moderate to high doses of oral prednisolone was used in 28 (76%) patients for both serositis and concomitant disease activity in other organs. All episodes of serositis resolved completely within two months. Over a mean observation of 46 months, nine patients had 18 relapses of serositis, which were responsive to either NSAIDs or augmentation of prednisolone dosage. Pleural fibrosis developed in three patients. Serosal complications are not uncommon in patients with SLE and can be life-threatening. NSAIDs and corticosteroids are often effective but more aggressive immunosuppressive therapy is required for severe or refractory cases. The prognosis of lupus serositis is generally good. Relapse or progression to fibrotic disease is uncommon.
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Affiliation(s)
- B L Man
- Department of Medicine, Tuen Mun Hospital, Hong Kong SAR, China
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Trock D, Voinea A, Wolk J, Majoros S. New-Onset Lupus Presenting as Serositis in an 80-Year-Old Woman. J Clin Rheumatol 2005; 11:292-3. [PMID: 16357784 DOI: 10.1097/01.rhu.0000182205.26490.b3] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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Vilá LM, Rivera del Río JR, Ríos Z, Rivera E. Lymphocyte populations and cytokine concentrations in pericardial fluid from a systemic lupus erythematosus patient with cardiac tamponade. Ann Rheum Dis 1999; 58:720-1. [PMID: 10610674 PMCID: PMC1752804 DOI: 10.1136/ard.58.11.720] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
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Abstract
Systemic lupus erythematosus (SLE), a connective tissue disease characterized by the production of autoantibodies, can affect all organ systems. Cardiac involvement in patients with SLE has been described since the early 20th century. The manifestations are numerous and can involve all components of the heart, including the pericardium, conduction system, myocardium, valves, and coronary arteries. In recent years, echocardiography has yielded additional information about the heart in patients who have SLE with and without clinical cardiac involvement. Moreover, antiphospholipid antibodies have been linked to several cardiac manifestations in patients with SLE, including valvular abnormalities and possibly coronary artery disease. This updated, comprehensive review summarizes the new literature on SLE and the heart.
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Affiliation(s)
- K G Moder
- Division of Rheumatology and Internal Medicine, Mayo Clinic Rochester, MN 55905, USA
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Kahl LE. The spectrum of pericardial tamponade in systemic lupus erythematosus. Report of ten patients. ARTHRITIS AND RHEUMATISM 1992; 35:1343-9. [PMID: 1445451 DOI: 10.1002/art.1780351115] [Citation(s) in RCA: 58] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
OBJECTIVE To describe the range of clinical manifestations and the outcome of pericardial tamponade in patients with systemic lupus erythematosus (SLE). METHODS Patients with pericarditis and with pericardial tamponade were identified from our computerized database of 395 SLE patients. Medical records were reviewed to establish activity of SLE at the time of tamponade, as well as clinical and laboratory features, treatment, and outcome of the tamponade. RESULTS Pericarditis occurred in 75 patients (19%), with 11 episodes of tamponade in 10 of them (13%; 2.5% of entire series). Tamponade was the initial manifestation of SLE in 4 patients. Seven episodes occurred during active lupus, with nephritis present in 6. Signs of venous congestion, including ascites and facial or peripheral edema, were the most common manifestation of tamponade. Pericardial fluid was exudative, and pericardial tissue demonstrated a range of findings including fibrinous and fibrotic changes, acute and chronic inflammatory infiltrates, and vascular proliferation. Tamponade was fatal in 1 patient, and 2 patients each had recurrent effusions and pericardial thickening. CONCLUSION Pericardial tamponade may occur at any point in the course of SLE, and should be considered in patients with unexplained signs of venous congestion. The differential diagnosis includes active SLE, uremia, and infection. Treatment with high-dose steroids and either pericardiocentesis or placement of a pericardial window is indicated, but recurrent effusions or pericardial thickening may develop.
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Affiliation(s)
- L E Kahl
- Department of Medicine, Washington University School of Medicine, St. Louis, Missouri
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Van Offel JF, De Clerck LS, Kersschot IE. Cholesterol crystals and IgE-containing immune complexes in rheumatoid pericarditis. Clin Rheumatol 1991; 10:78-80. [PMID: 2065512 DOI: 10.1007/bf02208037] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
A 72-year-old rheumatoid arthritis patient is described presenting with acute dyspnoea and peripheral oedema. A pericardial effusion with signs of tamponade was diagnosed. Examination of the pericardial fluid revealed the presence of cholesterol crystals and IgE-containing immune complexes. The significance of these findings in the differential diagnosis of pericardial disease is discussed.
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Affiliation(s)
- J F Van Offel
- Department of Rheumatology, Sint-Vincentius Hospital, Antwerp, Belgium
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Negoro N, Kanayama Y, Yasuda M, Okamura M, Amatsu K, Koda S, Takeda T, Inoue T. Nuclear ribonucleoprotein immune complexes in pericardial fluid of a patient with mixed connective tissue disease. ARTHRITIS AND RHEUMATISM 1987; 30:97-101. [PMID: 3814202 DOI: 10.1002/art.1780300114] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/07/2023]
Abstract
We performed immunopathologic studies of the pericarditis present in a patient with mixed connective tissue disease. A large number of nuclear RNP (nRNP) immune complexes (ICs) were found in the pericardial fluid, but not in the serum. The pericardial small vessels had no deposits of IgG. These results suggest that locally formed nRNP ICs were closely associated with the pathogenesis of pericarditis in this patient.
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Stevens MB. Systemic lupus erythematosus clinical issues. SPRINGER SEMINARS IN IMMUNOPATHOLOGY 1986; 9:251-70. [PMID: 3544281 DOI: 10.1007/bf02099025] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/06/2023]
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Abstract
SLE affects most aspects of cardiac function, and recent studies have reported increasing cardiovascular morbidity and mortality. Pathologically, SLE is characterized by a pancarditis involving pericardium, myocardium, endocardium, and coronary arteries. In autopsy series, pericarditis has been found in 43% to 100% (mean 62%, Table I), and myocarditis was found in 8% to 78% (mean 40%, Table II), but both have been underdiagnosed clinically. Libman-Sacks lesions have been noted in 25% to 100% (mean 43%) and infective endocarditis in 1.1% to 4.9% of clinical and autopsy studies (Table III). Coronary disease may be due to arteritis, which should be treated with high-dose steroids, or it may be due to atherosclerosis, which is amenable to medical or surgical therapy. Valvular disease has been treated surgically, but with a combined surgical mortality as high as 25%. Aortic insufficiency and mitral regurgitation are the most common valvular problems, although aortic and mitral stenosis have also been reported. Hypertension has been noted in 14% to 69%, and heart failure in 5% to 44%. Evidence for a lupus cardiomyopathy, which may be subclinical, is reviewed. While steroids may ameliorate SLE pancarditis, they have also been associated with hypertension, LV hypertrophy, purulent and constrictive pericarditis, mitral regurgitation, and perhaps accelerated atherosclerosis. It remains to be seen if improved diagnosis and treatment of the cardiovascular manifestations of SLE can enhance survival.
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Bakke AC, Kirkland PA, Kitridou RC, Quismorio FP, Rea T, Ehresmann GR, Horwitz DA. T lymphocyte subsets in systemic lupus erythematosus. Correlations with corticosteroid therapy and disease activity. ARTHRITIS AND RHEUMATISM 1983; 26:745-50. [PMID: 6602614 DOI: 10.1002/art.1780260607] [Citation(s) in RCA: 81] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Abstract
The contribution of immune regulation to the etiology of systemic lupus erythematosus (SLE) is poorly understood. Using the monoclonal antibodies OKT4 and OKT8, we quantitated, by flow cytometry, T inducer/helper and T cytotoxic/suppressor cells in patients with SLE. Serologically active patients, who had clinical manifestations such as arthritis or rash and were not receiving prednisone, were characteristically lymphopenic due to a marked reduction in OKT4+ cells. Prednisone therapy produced the same phenomenon. Untreated patients, who were serologically inactive, demonstrated no abnormalities. These studies have thus revealed two presumably independent factors that can produce similar immunoregulatory aberrations.
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Kaufman RL, Hoefs JC, Quismorio FP. Immune complexes in the portal and systemic circulation of patients with alcoholic liver diseases. CLINICAL IMMUNOLOGY AND IMMUNOPATHOLOGY 1982; 22:44-54. [PMID: 6749354 DOI: 10.1016/0090-1229(82)90020-4] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
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