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Pericardial involvement in systemic sclerosis

R J Byers1, D A Marshall, A J Freemont

  • 1Department of Pathological Sciences, University of Manchester.

Annals of the Rheumatic Diseases
|June 1, 1997
PubMed
Summary
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Systemic sclerosis frequently causes chronic pericarditis and myocardial fibrosis, suggesting it is a primary cardiac manifestation rather than secondary to kidney disease.

Area of Science:

  • Rheumatology
  • Cardiology
  • Pathology

Background:

  • Systemic sclerosis is a multisystem autoimmune disease characterized by fibrosis.
  • Cardiac involvement is a significant cause of morbidity and mortality in systemic sclerosis.
  • The specific pathology of pericardial involvement in systemic sclerosis requires further elucidation.

Purpose of the Study:

  • To investigate the frequency and histological features of pericardial disease in systemic sclerosis.
  • To differentiate systemic sclerosis-related pericarditis from other causes, such as uraemic pericarditis.
  • To explore the potential role of fibrosis and mast cells in the pathogenesis of cardiac involvement.

Main Methods:

  • Histological examination of pericardial autopsy sections from 44 systemic sclerosis patients and 19 controls.

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  • Staining techniques included haematoxylin and eosin, acid toluidine blue, and elastic van Gieson.
  • Quantification of mast cells and fibrosis using mast cell counts and Chalkley scoring.
  • Main Results:

    • Chronic pericarditis was observed in 77.5% of systemic sclerosis cases, compared to 1 control.
    • Increased pericardial fibrosis was noted in systemic sclerosis patients; mast cell numbers were similar.
    • Myocardial fibrosis was present in 37.5% of systemic sclerosis cases, absent in controls.

    Conclusions:

    • Pericarditis and myocardial fibrosis are significantly more prevalent in systemic sclerosis.
    • These findings suggest pericarditis in systemic sclerosis is a primary cardiac manifestation.
    • The results underscore the importance of cardiac evaluation in systemic sclerosis management.