Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA) typically is characterized by asthma, blood eosinophilia, and extrapulmonary manifestations. Asthma is a major EGPA symptom affecting almost all patients, but cardiac involvement is one of the most serious manifestations, responsible for 31% of deaths. The recently defined two phenotypes of EGPA, according to the patient’s antineutrophil cytoplasmic antibody (ANCA) status, differ significantly in clinical features and prognosis. We share a case of EGPA characterized by atypical manifestation without any evidence of asthma, in whom extensive cardiac involvement was the dominant manifestation of vasculitis. This case demonstrates the difficulties associated with phenotyping EGPA and highlights the importance of cardiac magnetic resonance imaging (CMRI) in definitive diagnosis.
Footnotes
Disclosures: The authors have declared no financial support or conflicts of interest relevant to this work. Written informed consent was obtained from the patient for the publication of this case report.
- Received April 20, 2021.
- Revision received February 12, 2022.
- Accepted March 22, 2022.
