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Related Experiment Videos

Acute myocarditis in fulminant systemic sclerosis.

B S Clemson1, W R Miller, J C Luck

  • 1Department of Medicine, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey 17033.

Chest
|March 1, 1992
PubMed
Summary

Systemic sclerosis can cause myocarditis, leading to heart failure. This case highlights the importance of considering acute myocarditis in scleroderma patients with new-onset heart failure, even without muscle weakness.

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Area of Science:

  • Cardiology
  • Rheumatology
  • Pathology

Background:

  • Progressive systemic sclerosis is associated with myositis and myocarditis.
  • Intravenous pulse methylprednisolone has shown therapeutic benefits in such patients.
  • Premortem biopsy documentation of myocarditis and myocardial fibrosis in scleroderma is rare.

Observation:

  • A patient presented with subacute congestive heart failure six months after Raynaud's phenomenon onset.
  • Clinical signs were consistent with scleroderma, but proximal muscle weakness was absent.
  • Elevated creatine kinase, MB-creatine kinase, and hypothyroidism were noted.

Findings:

  • Echocardiogram revealed four-chamber dilatation and severe left ventricular dysfunction.
  • Endomyocardial biopsy showed mild fibrosis and lymphocytic infiltrate.

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  • Cardiac catheterization indicated normal coronary arteries and a severely decreased cardiac index.

    • Implications:

    • This case underscores the potential for acute myocarditis in scleroderma patients presenting with heart failure.
    • Early diagnosis and management of cardiac involvement in systemic sclerosis are crucial.
    • Further research is needed to elucidate the mechanisms and optimal treatment strategies for scleroderma-associated myocarditis.