A RECENT monocentre study has highlighted the prevalence and severity of cardiac complications in patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA), a rare systemic vasculitis marked by hypereosinophilia. The findings underscore the need for early diagnosis and aggressive management to improve outcomes.
The retrospective study evaluated 103 previously untreated EGPA patients. Detailed multidisciplinary assessments, including ECG, echocardiography, cardiac MRI, invasive coronary angiography, and histopathology, were performed to identify cardiac manifestations associated with the disease.
Common and Severe Cardiac Manifestations in EGPA
Cardiac involvement was diagnosed in 36 patients (35%), revealing it as a common and serious component of EGPA. Among these patients, the most frequent diagnoses included pericarditis (77%), cardiomyopathy with heart failure (55%), and definite myocarditis (36%). Severe complications were not uncommon: 14% experienced cardiogenic shock, while 6% suffered cardiac arrest. Notably, vasospastic coronary arteries leading to myocardial infarction were observed in 8% of patients with cardiac involvement, representing a rare but life-threatening manifestation.
Patients with cardiac involvement (EGPA-CI) demonstrated significantly higher systemic disease activity, including elevated eosinophil counts and C-reactive protein levels, indicating a more aggressive disease course. These patients also exhibited worse overall prognosis compared with those without cardiac complications, emphasising the clinical importance of early cardiac assessment.
The study concludes that cardiac involvement is not only a frequent initial manifestation of EGPA but also a key determinant of severity and outcome. While pericarditis and myocarditis remain the most common cardiac complications, coronary vasospasms were identified as a rare but potentially fatal presentation that can mimic acute myocardial infarction, complicating diagnosis.
Implications for Improving Outcomes in High-Risk EGPA Patients
Overall, the findings highlight the urgent need for prompt recognition and intervention. Early cardiac evaluation and management in EGPA patients may prevent severe outcomes and improve survival. Clinicians are encouraged to maintain a high index of suspicion for cardiac involvement, even at initial presentation, to ensure timely treatment and mitigate the risk of advanced cardiac impairment.
Reference
Schramm M et al. Coronary vasospasms and other cardiac manifestations in Eosinophilic Granulomatosis with Polyangiitis: Clinical impact and frequency in a monocentre study of 103 patients. RMD Open. 2025;11(4):e005816.
