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

TL;DR

A patient with a rare heart condition linked to systemic sclerosis showed improvement after treatment with glucocorticoids and cyclophosphamide.

Contribution

This case highlights a potential treatment for acute SSc-related cardiomyopathy with immunosuppressive therapy.

Findings

The patient showed significant and prolonged recovery after receiving intravenous cyclophosphamide and glucocorticoids.

The case presented systolic dysfunction, which is less common in SSc-cardiomyopathy.

Endomyocardial biopsies lacked T-lymphocyte infiltration, possibly due to sampling limitations.

Abstract

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.

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.

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.

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