Cardiac Involvement and Absence of Asthma – What is Phenotype Specificity of EGPA: A Case Report

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. Two recently defined 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.