Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort

Abstract

This French cohort is the biggest pediatric EGPA series described to date, with a long follow-up period. The findings confirm that pediatric EGPA has specific clinical, radiological, and histological characteristics that differ from adult EGPA. Development of systemic symptoms, and consequently diagnosis, occur with a shorter delay in children, mainly during the eosinophilic phase and leading to a specific presentation.

A Pediatric Case of Relapsing Eosinophilic Granulomatosis with Polyangiitis Successfully Treated with Mepolizumab

“…EGPA is a rare disease that usually manifests in adulthood with an estimated incidence of approximately 0.1 to 2.7 new cases per 1 million people per year (2). According to the review of Fina et al (10) in 2018, only 38 articles describing pediatric cases of EGPA are listed in the PubMed database, with 92 cases cited (mostly individual case reports). In pediatric patients with EGPA, eosinophilia with respiratory involvement is frequently observed, and ANCA titers are often negative (8)(9)(10), as in our patient.…”

“…EGPA is uncommon in childhood (8)(9)(10). In a nationwide Japanese cohort, the mean age of EGPA patients was 58 years old (11).…”

“…Pediatric EGPA is a potentially life-threatening vasculitis (9). Systemic symptoms develop earlier than in adult patients, mainly during the eosinophilic phase (10). Corticosteroids are the mainstay initial therapy for pediatric EGPA patients, but steroid-related side effects regularly occur (8)(9)(10).…”

“…Systemic symptoms develop earlier than in adult patients, mainly during the eosinophilic phase (10). Corticosteroids are the mainstay initial therapy for pediatric EGPA patients, but steroid-related side effects regularly occur (8)(9)(10). Recently, targeted therapies using omalizumab and rituximab have been successfully used to treat pediatric patients with refractory EGPA (12,13).…”

Pediatric Pulmonology Year in Review 2018: Rare lung disease, neuromuscular disease, and diagnostic testing

“…Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis, rarely seen in children, that typically presents in patients with a history of asthma . Fina et al reported on 14 pediatric patients with EGPA diagnosed between 1980 and 2012, from the French national database for rare lung disease. The median age at diagnosis was 12.3 years, and all but two had a history of asthma of at least 2‐year duration before developing EGPA.…”