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

• Tác giả: Andrew A Gustafson, Monique E Hinchcliff, Jon W Lomasney, Sanjiv J Shah, Katherine V Trinh
• Ngôn ngữ: eng
• Ký hiệu phân loại: 510 Mathematics
• Thông tin xuất bản: Netherlands : JACC. Case reports , 2025
• Bộ sưu tập: NCBI
• ID: 702259

Tóm tắt

 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.