Revisiting characteristics, treatment and outcome of cardiomyopathy in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Objectives Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing eosinophil-rich vasculitis. Specific cardiomyopathy (CM) was described in old studies as the most important predictor of mortality. We aimed to revisit EGPA-related CM and its outcome in recent decades. Methods We reviewed all EGPA patients managed from 2000 to 2019 in our vasculitis clinic. Baseline characteristics and outcomes were analyzed. EGPA-related CM was defined as clinical or extra-clinical manifestations of patent myocardial involvement, after exclusion of other causes. Results We included 176 patients. Median age was 47 years (IQR 36-58 years). Specific CM was observed in 70 patients (40%). Cardiac symptoms were observed in 81% of CM+ patients, including mainly typical or atypical chest pain and peripheral edema. Abnormal EKG, TTE and cardiac magnetic resonance imaging (CMRI) were found in 72%, 72% and 99% in CM+ patients, respectively, contrasting with abnormalities in 32%, 38% and 60% in CM-negative patients. Late gadolinium enhancement (LGE) was the most frequent abnormality on CMRI (70%). CM+ patients were less frequently ANCA-positive, had less frequent peripheral neuropathy and had higher eosinophil count. Major adverse cardiovascular events (MACE) occurred in 13%, both in CM+ and CM- patients. Abnormal EKG and LGE on CMRI were associated with the occurrence of MACE. Four patients died, but none from cardiac causes. Conclusions Specific cardiomyopathy is frequent in EGPA, especially in ANCA-negative patients with high eosinophil counts. Long-term outcome was better than previously reported. Abnormal EKG and LGE on CMRI were associated with the occurrence of MACE.