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Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort
Author(s) -
Fina Agnès,
Dubus JeanChristophe,
Tran Antoine,
Derelle Jocelyne,
Reix Philippe,
Fayon Michael,
Couderc Laure,
Donnou MarieDominique,
Pagnier Anne,
Blanchon Sylvain,
Faure Nathalie,
Mely Laurent,
Albertini Marc,
de Blic Jacques,
GiovanniniChami Lisa
Publication year - 2018
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.24089
Subject(s) - medicine , granulomatosis with polyangiitis , cohort , pediatrics , eosinophilic , vasculitis , surgery , disease , pathology
Objectives To describe the characteristics of pediatric cases of eosinophilic granulomatosis with polyangiitis (EGPA), a systemic necrotizing vasculitis rarely diagnosed in children, retrieved from the French Reference Center for rare pediatric lung diseases and compared with adult cases included in the French Vasculitis Study Group cohort. Methods We collected information on pediatric EGPA disease presentation, management, and outcome. Cases met the Lanham criteria and/or American College of Rheumatology classification criteria. Results Fourteen cases of pediatric EGPA were included, from 1980 to 2012, with a median follow‐up of 58.5 months. Median age at diagnosis was 12.3 years. All cases had respiratory involvement. The organ systems most frequently involved were the upper airway (85%), skin (71%), digestive tract (64%), and heart (57%). Neurological and renal involvement were rare. Four of the fourteen children were positive for ANCA (30.7%). During follow‐up, three children required intensive care and one child died. The relapse rate was 64%. In comparison with an adult cohort, we found more ENT, heart, and digestive‐tract involvement, and fewer neurological manifestations. In children, the delay between asthma onset and diagnosis was shorter, and biopsies showed fewer features of vasculitis. Conclusion 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.