Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare (R) cohort - Archive ouverte HAL Access content directly
Journal Articles Pediatric Pulmonology Year : 2018

Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare (R) cohort

Agnes Fina
  • Function : Author
Antoine Tran
  • Function : Author
Jocelyne Derelle
  • Function : Author
Philippe Reix
Michael Fayon
  • Function : Author
Laure Couderc
  • Function : Author
Marie-Dominique Donnou
  • Function : Author
Anne Pagnier
  • Function : Author
Sylvain Blanchon
  • Function : Author
Nathalie Faure
  • Function : Author
Laurent Mely
  • Function : Author
Marc Albertini
  • Function : Author
Jacques De Blic
  • Function : Author
Lisa Giovannini-Chami
  • Function : Author

Abstract

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 Results We collected information on pediatric EGPA disease presentation, management, and outcome. Cases met the Lanham criteria and/or American College of Rheumatology classification criteria. 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.
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Dates and versions

hal-01942443 , version 1 (03-12-2018)

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Agnes Fina, Jean-Christophe Dubus, Antoine Tran, Jocelyne Derelle, Philippe Reix, et al.. Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare (R) cohort. Pediatric Pulmonology, 2018, 53 (12), pp.1640-1650. ⟨10.1002/ppul.24089⟩. ⟨hal-01942443⟩

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