Detecting cardiac involvement with magnetic resonance in patients with active eosinophilic granulomatosis with polyangiitis.

Abstract

Cardiac involvement is the most important prognostic factor in eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). The aims of this study were to describe findings of cardiac magnetic resonance (CMR) in patients with active EGPA and to find factors associated with cardiac involvement detected by CMR that could help identify patients who would benefit from the examination. Medical records and CMR images in 16 consecutive EGPA patients (8 women and 8 men, median age of 47years ranging from 34 to 68years) were reviewed. Clinical features and results of laboratory tests were compared according to the presence of myocardial late gadolinium enhancement (LGE) on CMR images. The patients were followed for the development of cardiac symptoms and signs (mean follow up duration, 40.5±12.8months). Among the total of 16 patients, 8 (50%) had myocardial LGE according to CMR, located in the subendocardial layer in 7 of them (87.5%). The extent of LGE had a significant negative correlation with left ventricular ejection fraction (LVEF, ρ=-0.723, p=0.043). The presence of LGE was associated with larger end-systolic left ventricle internal dimension (34 vs. 28mm, p=0.027) and presence of diastolic dysfunction (75 vs. 0%, p=0.008) on echocardiography, elevated NT-proBNP (75 vs. 12.5%, p=0.012), and elevated CK-MB (62.5 vs. 0%, p=0.010) compared to the group without LGE. Only one patient (6.3%) had cardiac symptoms before CMR and another patient (6.3%) developed heart failure 4years later during remission. The other 14 patients remained free from cardiac signs and symptoms during the follow-up period. In patients with active EGPA, CMR enables detection of cardiac involvement when cardiac symptoms are not present. Echocardiographic diastolic dysfunction and elevated NT-proBNP or CK-MB may help identify active EGPA patients who can benefit from CMR to detect cardiac involvement without cardiac symptoms.