A pediatric case of eosinophilic granulomatosis with polyangiitis accompanied by heart failure mimicking an asthma attack
10.4168/aard.2019.7.4.212
- Author:
Min Jung KIM
1
;
Bo Ra LEE
;
Ji Soo PARK
;
Yun Jung CHOI
;
Mi Kyoung SONG
;
Soyoung LEE
;
Dong In SUH
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. dongins0@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Eosinophilic granulomatosis with polyangiitis;
Churg-Strauss syndrome;
Child
- MeSH:
Adolescent;
Asthma;
Cardiomyopathies;
Child;
Churg-Strauss Syndrome;
Cytoplasm;
Death, Sudden, Cardiac;
Electrocardiography;
Eosinophilia;
Eosinophils;
Female;
Granulomatosis with Polyangiitis;
Heart Failure;
Heart;
Humans;
Myocarditis;
Pericardial Effusion;
Pericarditis;
Prognosis;
Steroids;
Systemic Vasculitis
- From:Allergy, Asthma & Respiratory Disease
2019;7(4):212-217
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Eosinophilic granulomatosis with polyangiitis (EGPA, also known as the Churg-Strauss syndrome) is a disorder characterized by asthma, peripheral eosinophilia and systemic vasculitis. It rarely occurs in children, so that physicians may frequently mistake it for a simple uncontrolled asthma. Since a subsequent cardiac involvement is critical for the prognosis, it is important to suspect EGPA in children with severe, uncontrolled asthma. The cardiac manifestations in EGPA are variable from asymptomatic electrocardiogram abnormalities to pericarditis with pericardial effusion, myocarditis with cardiomyopathy, heart failure, and sudden cardiac death. Although delayed treatment may lead to fatal cardiac complications in EGPA, adequate immune suppression can reverse cardiac impairment. We report a 14-year-old girl with persistent asthma refractory to steroids who was eventually diagnosed with an anti-neutrophil cytoplasmic antibody-negative EGPA.
