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Updated: May 27, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Acute Systemic Sclerosis-Associated Cardiomyopathy That Improved With Glucocorticoids and Cyclophosphamide
Andrew A Gustafson1, Katherine V Trinh1, Jon W Lomasney2
1Northwestern University Feinberg School of Medicine, Division of Hospital Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
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.
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