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Cardiovascular degeneration in Erdheim-Chester disease: prevalence, burden, and outcomes.


ABSTRACT:

Abstract

Erdheim-Chester disease (ECD) is a rare systemic histiocytic neoplasm, with cardiac morbidities, including cardiovascular (CV) risk factors and cardiac conditions, playing a significant yet poorly understood role in the disease burden. This study evaluated the prevalence, characteristics, and prognosis of ECD in patients with (ECD-C) and without cardiac involvement (ECD-noC) and compared the burden of cardiac morbidities with matched controls. Patients diagnosed with ECD between 1990 and 2021 at a tertiary center were included, with cardiac involvement centrally assessed using radiographic studies. Cardiac morbidities were compared with a control group without ECD, matched for age, sex, body mass index, and smoking history. Among 104 patients with ECD, 39 (37%) had cardiac

SUBMITTER: Tawfiq RK 

PROVIDER: S-EPMC12686793 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature