Acute Systemic Sclerosis-Associated Cardiomyopathy That Improved With Glucocorticoids and Cyclophosphamide.

BACKGROUND

Systemic sclerosis (SSc) cardiomyopathy has a prevalence of 7 to 39% and is associated with increased mortality. Despite this, little evidence informs SSc cardiomyopathy treatment.

CASE SUMMARY: We present a patient with diffuse cutaneous SSc with acute heart failure. Extensive workup supported a diagnosis of SSc myopericarditis, although endomyocardial biopsies were unrevealing. She received intravenous cyclophosphamide and glucocorticoids and achieved significant and prolonged recovery.

DISCUSSION: Our patient presented with systolic dysfunction as opposed to diastolic dysfunction that is more typical in patients with SSc-cardiomyopathy. Endomyocardial biopsies lacked T-lymphocyte infiltration that may be due to sampling error because >17 samples are needed to diagnose myocarditis in >80% of cases.