Work

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

Year: 2025

Type: article

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.

Source: JACC Case Reports

DOI: 10.1016/j.jaccas.2024.102948

Authors: Andrew A. Gustafson, Katherine V. Trinh, Jon W. Lomasney, Sanjiv J. Shah, Monique E. Hinchcliff

Institutions: Northwestern University, Yale University

Language: English

Topic: Systemic Sclerosis and Related Diseases

Subfield: Pathology and Forensic Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $850