Pericardial disease in scleroderma (systemic sclerosis)

doi:10.1016/0002-9343(74)90008-4

The American Journal of Medicine

Volume 57, Issue 4, October 1974, Pages 566-575

https://doi.org/10.1016/0002-9343(74)90008-4 Get rights and content

Abstract

A review of the records of 210 patients with scleroderma seen between 1952 and 1972 revealed two clinical patterns of pericardial disease in 15 patients: (1) Chronic pericardial effusion (11 patients), confirmed by roentgenography and ultrasound, occurred in association with dyspnea, Raynaud's syndrome, cardiomegaly, congestive heart failure and pleural effusion in the absence of renal failure. In three patients hemodynamic signs of pulsus paradoxus, Kussmaul's sign or pulsus alternans developed. In six patients with chronic effusion renal failure developed within 6 months, an incidence severalfold higher than expected in the scleroderma population at large. (2) Acute pericarditis (four patients) was associated with dyspnea, chest pain, pericardial friction rub, fever, cardiomegaly and elevated latex fixation titers (in two of four patients).

Pericardial disease is a recognizable clinical entity in scleroderma and should be considered in all patients with cardiomegaly, congestive heart failure or chest pain. In 34 autopsy studies, the incidence of pericardial involvement (62 per cent) exceeded the incidence of significant myocardial fibrosis (30 per cent); thus pericardial scleroderma represents a relatively common form of cardiac involvement in this diffuse connective tissue disease.

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2020, Revista Colombiana de Reumatologia
La esclerosis sistémica es una enfermedad autoinmune generalizada que se caracteriza por vasculopatía, inflamación, depósito de colágeno y fibrosis en la piel y órganos internos. Las complicaciones renales son comunes y con un amplio espectro e incluyen crisis renal esclerodérmica, función renal anormal, proteinuria, enfermedad vascular renal crónica, daño renal por fármacos nefrotóxicos, nefropatía por oxalato de calcio, glomerulonefritis y vasculitis asociadas a anticuerpos contra el citoplasma de los neutrófilos. La alteración renal permanece siendo una causa importante de morbilidad y mortalidad en la esclerodermia. El objetivo de esta revisión narrativa es describir las complicaciones renales de esta enfermedad.
Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by vasculopathy, inflammation, collagen deposits and fibrosis in the skin and internal organs. Renal complications are common in SSc. The spectrum of renal complications in systemic sclerosis includes scleroderma renal crisis (SRC), abnormal renal function, proteinuria, renal chronic vascular disease, kidney injury from nephrotoxic drugs, oxalate calcium nephropathy, glomerulonephritis and vasculitis associated to anti-neutrophil cytoplasmic antibody. Renal involvement remains an important cause of morbidity and mortality in scleroderma. The objective of this review is to describe the renal complications of scleroderma.
Defining primary systemic sclerosis heart involvement: A scoping literature review
2019, Seminars in Arthritis and Rheumatism
Background: Clinically evident primary heart involvement due to systemic sclerosis (SHI) is considered a poor prognostic factor and is a leading cause of systemic sclerosis (SSc) related death. Yet, there remains no consensus definition of SHI and poor understanding of the natural history and risk factors for the development of SHI.
Methods: We performed a scoping literature review of published articles with a primary focus of SHI to capture previously used definitions of SHI and items used to measure SHI. Any factors reported to be associated with an increased risk of SHI were recorded.
Results: Of the 2436 records identified in a search of MEDLINE, EMBASE and PubMed databases, 295 were included in the final scoping review. Analysis of the literature revealed studies of variable quality, generally low patient numbers and highly heterogeneous definitions of SHI within studies. There is no clear consensus from the literature as to the scope of SHI and the prognostic significance of sub-clinical investigation abnormalities commonly detected.
Conclusion: The lack of a standardised definition of SHI remains a significant unmet need in SSc. The results of this review will assist in the development of consensus classification criteria to enable more accurate quantification of the burden of SHI, identification of factors associated with increased risk of developing SHI, and evaluation of the efficacy of any novel therapeutic strategies.
Systemic Sclerosis
2017, The Heart in Rheumatic, Autoimmune and Inflammatory Diseases: Pathophysiology, Clinical Aspects and Therapeutic Approaches
Cardiac involvement in systemic sclerosis (SSc) may be either primary or secondary to the impairment of other organs such as the lungs and kidneys. Primary cardiac involvement, which develops as a direct consequence of the disease, may affect all cardiac tunics, endocardium, myocardium, and pericardium. This may conduct to pericardial effusion, arrhythmias, conduction system defects, myocardial ischemia, myocardial hypertrophy, and heart failure. The prevalence of primary cardiac involvement in SSc varies depending on the sensitivity of the diagnostic tools used, but it is ascertained that overt cardiac manifestations are associated with poor prognosis. Microvascular impairment is a pathognomonic feature of SSc, and together with focal fibrosis may involve all cardiac structures. In contrast, the frequency of atherosclerotic coronary artery disease in SSc seems to be comparable to that of the general population. Because cardiac involvement in SSc can be often clinically obscure, a complete routine evaluation should be adopted in every patient, including standard 12-lead electrocardiogram (ECG) and Doppler echocardiography (especially tissue Doppler imaging), while magnetic resonance imaging and cardiac computed tomography are sensitive techniques for earlier detection of both structural and functional scleroderma-related cardiac pathologies, although they still represent a second level examination, not available everywhere. Additional investigations can be required in suspicion of other manifestations such as conduction and rhythm disorders (24-h Holter monitoring, invasive electrophysiological studies), or myocarditis (cardiac magnetic resonance). Routine screening for subclinical cardiac involvement with more sensitive tools leads to early diagnosis, in order to treat patients in a “window of opportunity” with drugs that may improve myocardial perfusion and function, and possibly limit the progression of the most life-threatening complications.
Systemic sclerosis: A rare cause of heart failure?
2015, Revista Portuguesa de Cardiologia
Systemic sclerosis (SS) is a chronic disease in which there may be multisystem involvement. It is rare (estimated prevalence: 0.5–2/10 000) with high morbidity and mortality, and there is as yet no curative treatment.
We report the case of a young woman newly diagnosed with SS, in whom decompensated heart failure was the main manifestation.
A esclerose sistémica (ES) é uma doença crónica com possível apresentação multi-sistémica. É considerada uma doença rara (prevalência estimada: 0.5-2/10,000) com alta morbilidade e mortalidade para a qual não há cura hoje em dia.
Relatamos o caso de uma jovem mulher, recém diagnosticada de ES por afetação pleural e cutânea, com insuficiência cardíaca global no momento da consulta.
Clinical features of systemic sclerosis
2015, Rheumatology: Sixth Edition
Kidney involvement in systemic sclerosis
2014, Presse Medicale
Citation Excerpt :
Patients are not usually symptomatic from this process, except that the anaemia may precipitate congestive heart failure. Patients with SRC often present with congestive heart failure, serious ventricular arrhythmias (even cardiac arrest), or large pericardial effusions [8,10]. This is primarily from the stress of the hypertension on the heart, effects of hyperreninemia, and from fluid overload secondary to oliguric renal failure, although some patients with very severe disease may also have primary scleroderma myocardial involvement contributing to these problems.
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 30 years 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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^☆: This investigation was supported by the New York and New Jersey Chapters of the Arthritis Foundation, the Charlotte and Sidney Lifschultz Foundation, and the Arthur K. Delson Medical Research Fund.

^∗: Present address: National Naval Medical Center, Bethesda, Maryland 20014.

¹: From the Department of Medicine, Columbia University College of Physicians & Surgeons, and the Edward Daniels Faulkner Arthritis Clinic, The Presbyterian Hospital, New York, New York.

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Clinical studyPericardial disease in scleroderma (systemic sclerosis)☆

Abstract

Am Heart J

Am Heart J

Am J Med

Am J Cardiol

Am J Med

Am J Cardiol

Am J Cardiol

Am Heart J

Prog Cardiovasc Dis

Prog Cardiovasc Dis

J Chron Dis

Am Heart J

Lancet

Am J Med

Am J Med

Am J Cardiol

Am J Med

Am J Cardiol

Survival with systemic sclerosis (scleroderma). A lifetable analysis of clinical and demographic factors in 309 patients

Ann Intern Med

Scleroderma heart disease, with a consideration of certain other visceral manifestations of scleroderma

Arch Intern Med

The natural history of progressive systemic sclerosis (diffuse scleroderma)

Bull Rheum Dis

The heart in generalized scleroderma. Progressive systemic sclerosis

Angiology

Progressive systemic sclerosis (scleroderma)

N Engl J Med

Pathology of scleroderma with special reference to the changes in the gastrointestinal tract

Am J Pathol

Progressive systemic sclerosis

Arch Dermatol

The heart in scleroderma

Br Heart J

Progressive systemic sclerosis (or visceral scleroderma). Review of literature and report of cases

Acta Med Scand

The pathophysiology of scleroderma involving the heart and respiratory system

Ann Intern Med

Clinical study
Pericardial disease in scleroderma (systemic sclerosis)☆