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SLE with recurrent heart failure and a dermatological clue to another added possibility

Luciano Candilio1, David D'Cruz, Divaka Perera

  • 1Department of Cardiology, St. George's Hospital, London, UK.

BMJ Case Reports
|June 16, 2012
PubMed

Insights

Systemic lupus erythematosus (SLE) can cause myocarditis, leading to heart dysfunction. Scleroderma was suspected as the underlying cause in a patient with refractory SLE myocarditis and characteristic skin findings.

Area of Science:

  • Cardiology
  • Rheumatology
  • Immunology

Background:

  • Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical manifestations.
  • Myocarditis, inflammation of the heart muscle, can be a serious cardiac complication of SLE.
  • Obstructive coronary artery disease (CAD) must be excluded in patients presenting with chest pain and cardiac biomarkers.

Observation:

  • A 36-year-old man with SLE presented with chest pain, ECG changes, and elevated cardiac/inflammatory markers.
  • Echocardiography revealed impaired left ventricular (LV) function, and coronary angiography ruled out obstructive CAD.
  • Despite treatment for SLE myocarditis with immunosuppressants, his condition showed partial improvement and a relapsing-remitting course with progressive LV dysfunction.

Findings:

  • Cardiac MRI demonstrated active inflammation and extensive transmural scarring.
  • Endomyocardial biopsy (EMB) showed patchy myocardial fibrosis and low-grade myocarditis, with negative PCR for viral causes.
  • Lack of response to immunosuppression and the presence of the "En coup de Sabre" sign suggested scleroderma as the underlying etiology.

Implications:

  • This case highlights the importance of considering scleroderma as an underlying cause of refractory myocarditis in SLE patients.
  • Aggressive immunosuppression may not always be sufficient for managing autoimmune myocarditis.
  • Multimodality imaging and biopsy are crucial for accurate diagnosis and management of complex cardiac conditions in autoimmune diseases.

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