gms | German Medical Science

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis characterized by tissue and blood hypereosinophilia. Cardiac involvement has been cited as the most important predictor of mortality, occurring in approximately a third of EGPA patients. The aim of our study was to identify and characterize EGPA patients and their cardiac manifestations in detail.

Methods: A retrospective monocenter study was performed on 78 patients who were diagnosed with EGPA based on Chapel Hill consensus conference definitions, ACR- and MIRRA-criteria. A detailed multi-disciplinary physical evaluation and laboratory work-up was executed on patient presentation. Diagnostics, clinical manifestations, and disease activity were recorded throughout disease course. Cardiac events were defined as documented EGPA-related abnormalities in ECG, echocardiography, cardiovascular MRI, invasive coronary angiography, or cardiac histopathology.

Results: Symptomatic cardiac manifestations were diagnosed in 28 of 78 EGPA patients (35.9%). Pericarditis (92.9%) and myocarditis (78.6%) were the most common cardiac abnormalities. Additionally, myocardial infarctions with non-obstructed, and sometimes vasospastic, coronary arteries were also a frequent feature (25%). Patients with cardiac involvement (EGPA-CI) were marginally younger (45 years; 18–66) than those without cardiac involvement (50 years; 18–72), with a higher prevalence in females (41.1% vs. 31.8%). ANCA positivity (21.4% vs. 24%) and extracardiac organ manifestations were similarly distributed in both groups. EGPA-CI patients showed higher disease activity by BVAS (20.2 vs. 14.4) and worse prognosis by FFS (1.1 vs. 0.2). Eosinophilic count (6147/mm3 vs. 3221/mm3) and CRP (76.1 mg/L vs. 46.9 mg/L) were also elevated in EGPA-CI patients. Use of methylprednisolone pulses (92.8% vs. 56%) and cyclophosphamide (50% vs. 26%) was more frequent, and higher dosages of prednisone were applied as part of standard care at baseline (64.4 mg vs. 43.6 mg) for EGPA-CI patients.

Conclusion: Cardiac involvement in EGPA patients is associated with younger age, elevated disease activity, and requires intensified therapeutic interventions. Importantly, some patients were diagnosed with EGPA only after invasive cardiac procedures. This emphasizes that eosinophilia is an important differential diagnostic criterion for patients presenting with cardiac manifestations but no cardiovascular risk factors. Earlier detection and management of EGPA in these patients could positively affect treatment modalities and prognosis.