Year: 2025
Type: article
Study design: Observational Study
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 involvement. Patients with ECD-C had higher rates of hypertension (67% vs 46%), hyperlipidemia (67% vs 40%), heart failure (36% vs 8%), and pericardial effusion (28% vs 2%) than those with ECD-noC. Compared with the matched non-ECD cohort, patients with ECD had higher prevalence of coronary artery disease (20% vs 7%), heart failure (18% vs 4%), and antihypertensive drug use (55% vs 40%). Notably, patients with ECD-C had inferior progression-free survival (PFS) from frontline therapy compared with patients with ECD-noC (5-year PFS, 28.3% vs 70.5%). These findings highlight the burden of CV risk factors and cardiac conditions in ECD, even without a clinical diagnosis of ECD-C. Importantly, this cardiac morbidity burden is substantial for patients with ECD-C compared with ECD-noC. Our findings highlight the need for comprehensive cardiac risk assessment and management strategies to improve patient outcomes.
Source: Blood Advances
Authors: Reema K. Tawfiq, Grant M. Spears, Jason R. Young, Madeline K. Mahowald, W. Oliver Tobin, Ubenthira Patgunarajah, Gordon J. Ruan, Dongni Yi, Saurabh Zanwar, Karen Rech, Matthew J. Koster, Aldo A. Acosta-Medina, Lucinda Gruber, Aishwarya Ravindran, Mithun Vinod Shah, N. Nora Bennani, Muhamad Alhaj Moustafa, Talal Hilal, Julio C. Sartori Valinotti, Robert Vassallo, Jay H. Ryu, Caroline Davidge-Pitts, Surendra Dasari, Thomas E. Witzig, Ronald S. Go, Gaurav Goyal, Jithma P. Abeykoon
Language: English
Subfield: Physiology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,800
OpenAlex ID: W4413787106