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

Abstract

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.