Key result
Cardiac involvement in EGPA is linked to lower LVEF and a ~62% pericardial effusion rate.
Cohort (n=53)
Yes
Absolute Event Rate: 61.5% vs 0%
p-value: p=0.011
Cardiac involvement in EGPA is associated with a severe initial clinical presentation and residual systolic dysfunction, although contemporary management yields low overall mortality.
Cardiac involvement in EGPA signals high-risk prognosis; leaves open targeted screening and therapy trials in this rare vasculitis.
Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis marked by asthma, eosinophilia, and multi–organ involvement. Cardiac involvement (CI)—from pericardial effusion to eosinophilic myocarditis—is among the most severe manifestations and strongly affects prognosis. Despite advances in diagnosis and therapy, CI features and outcomes remain incompletely defined. Purpose To characterize CI in EGPA, compare patients with CI versus non–cardiac involvement (nCI), and assess prognostic implications in a contemporary multicenter cohort. Methods Retrospective bicenter study of 53 EGPA patients (CI n=26; nCI n=27). Baseline demographics, biomarkers, echocardiography, cardiac magnetic resonance (CMR), and endomyocardial biopsy (EMB, when available) were analysed. Groups were compared using standard statistical tests. Results At diagnosis, mean age was 50.5±15.7 years; 45.3% were male, with balanced traditional cardiovascular risk factors. Figure 1 displays main results of the study. CI patients had higher hospitalization rates at diagnosis (p‹0.01), mainly due to cardiac symptoms. CI showed greater severity, with lower LVEF (42.8±15.9% vs 59.7±4.5%; p=0.002) and more frequent pericardial effusion (61.5% vs 0%; p=0.011). Severe presentations were common: complicated course (91.7%), cardiogenic shock (24%), fulminant myocarditis (28%), and temporary mechanical circulatory support (15.4%). Troponin and NT–proBNP were markedly elevated. At discharge, only 45.8% recovered LVEF ≥50%. Eosinophil counts and CRP did not differ between groups. ANCA–positive patients were less often male and less likely to present with CI. EMB (performed in 52.2% of CI) showed eosinophilic myocarditis in 76%. CMR confirmed myocarditis in 83%, with frequent late gadolinium enhancement (90.9%), mainly subendocardial (Figure 2), and intracavitary thrombi in 27.3%. Follow–up was longer in nCI (Figure 1). At last follow–up, LVEF remained lower in CI, while mortality was low and not significantly different between groups. Conclusions CI in EGPA represents a severe clinical phenotype with distinctive CMR and supportive EMB findings. Cardiac symptoms often drive initial hospitalization and diagnosis. Despite low mortality with contemporary management, residual systolic dysfunction is common, supporting routine cardiac screening and long–term follow–up, especially in symptomatic patients.Figure 1 Figure 2
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Ghidini et al. (2026) conducted a cohort in Eosinophilic granulomatosis with polyangiitis (EGPA) (n=53). Cardiac involvement vs. Non-cardiac involvement was evaluated on Pericardial effusion (p=0.011). Cardiac involvement in EGPA was associated with significantly lower LVEF (42.8% vs 59.7%; p=0.002) and more frequent pericardial effusion (61.5% vs 0%; p=0.011) compared to non-cardiac involvement.
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