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Updated: Nov 16, 2025

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Published on: June 16, 2020
[Characteristics of cardiac involvement in eosinophilic granulomatosis with polyangiitis]
A Belhassen1, S Toujani1, A El Ouni1
1Service de médecine interne, centre hospitalier universitaire Mongi-Slim, 2070 La Marsa, Tunisie.
Insights
Cardiac involvement, including cardiomyopathy, frequently reveals eosinophilic granulomatosis with polyangiitis (EGPA), particularly in women. Late-onset asthma and hypereosinophilia are key diagnostic clues for this ANCA-negative vasculitis.
Area of Science:
- Rheumatology
- Cardiology
- Internal Medicine
Background:
- Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis.
- Cardiac involvement is a significant cause of morbidity and mortality in EGPA.
- Understanding the cardiac manifestations of EGPA is crucial for timely diagnosis and management.
Purpose of the Study:
- To identify the specific characteristics of cardiac involvement in patients with EGPA.
- To analyze the clinical presentation, diagnostic findings, and outcomes of cardiac manifestations in EGPA.
Main Methods:
- Retrospective analysis of EGPA cases diagnosed between 2000 and 2019.
- Inclusion criteria based on 1990 American College of Rheumatology and 2012 Chapel Hill Consensus criteria.
- Evaluation of cardiac manifestations using clinical data and cardiac magnetic resonance imaging (MRI).
Main Results:
- Eleven EGPA cases were included, with 64% female patients; cardiac damage revealed EGPA in 55% of cases.
- Common cardiac manifestations included myocarditis, ischemic cardiomyopathy, cardiac tamponade, and intracardiac thrombus.
- Cardiac MRI revealed subendocardial changes, impaired left ventricle function, and micro-infarctions; 83.3% of cases were ANCA-negative.
Conclusions:
- Cardiomyopathy is a frequent and often presenting feature of EGPA.
- Late-onset asthma and hypereosinophilia are important indicators for EGPA diagnosis.
- Histological confirmation is recommended due to frequent ANCA negativity; the role of cardiac MRI requires further definition.
Aim Of The Study:
Our study aimed to identify the characteristics of cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA).
Methods:
We conducted a retrospective analytic study including EGPA cases diagnosed between 2000 and 2019 in an internal medicine department. Diagnosis was made according to the 1990 American College of Rheumatology criteria and the 2012 Chapel Hill Concensus.
Results:
Eleven EGPA cases were included, 64% of patients were female. Median age at diagnosis was 52 years [42-58]. Heart damage revealed EGPA in 55% of cases with a significant predominance of women (p=0.015). The main cardiac manifestations were myocarditis, ischemic cardiomyopathy due to small vessel vasculitis, cardiac tamponade and intracardiac thrombus. Cardiac magnetic resonance imaging (MRI) mainly showed subendocardial hyposignal in early infusion and late enhancement in the same areas, nodular by locations, associated with impaired left ventricle function and micro-infarctions by distal vasculitis. Cardiac damage was associated to ANCA negativity in 83.3% of cases. The median Birmingham Vasculitis Activity Score version3 (BVAS v3) was 16 [10-17]. Under conventional treatment, no relapses had occurred. The median vasculitis damage index (VDI) was 2 [1-2.3] and the mortality rate was zero after a mean follow-up of 43 months.
Conclusion:
Cardiomyopathy is a frequent revealing mode of EGPA. A late onset asthma and hypereosinophilia should guide the diagnosis. As ANCA research often turns out to be negative, histological evidence is recommended in this context. The contribution of cardiac MRI in the diagnosis of EGPA remains to be defined.
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