Eosinophilic granulomatosis with polyangiitis in childhood: Retrospective experience from a tertiary referral centre in the UK

Abstract

Objective. To describe the presenting clinical features, treatment and outcome in children with eosinophilicgranulomatosis with polyangiitis (EGPA) and to define factors that predicted mortality.Methods. A retrospective case notes review of patients fulfilling the Chapel Hill Consensus Conferencedefinition and/or ACR criteria for EGPA seen at Great Ormond Street Hospital, London. Demographics,clinical features, histopathology, treatment and outcomes were recorded. Descriptive statistics were expressedas median and range. Fisher's exact test was used for group comparisons. The PaediatricVasculitis Activity Score and Paediatric Vasculitis Damage Index (PVDI) were calculated.Results. Thirteen children (38% female) aged at diagnosis 14.1 (4-15.6) years were identified. Themedian time to diagnosis was 2 (0-7.3) years. History of asthma was documented in 76%. Themost common presenting features were pulmonary (69%), skin (61%), gastrointestinal (46%), cardiacinvolvement (46%), paranasal sinus abnormality (38%), arthritis/arthralgia (38%) and neurological involvement(15%). Paediatric Vasculitis Activity Score at presentation was 8/63 (2-25/63); ANCA was negativein all 10/13 patients tested. Treatment included corticosteroids in all, combined with CYC in 38% or AZAin 23%. PVDI at 12 (3-48) months follow-up was 3/72 (0-13/72). Relapses were recorded in 46%.Mortality was 15%; cardiomyopathy and PVDI scores≥5 significantly associated with mortality risk(P = 0.012).Conclusion. EGPA in the paediatric population is a rare and potentially life-threatening vasculitis.Increased awareness is essential to secure a timely diagnosis and to promptly initiate treatment sinceour data emphasize a high mortality, particularly in those with cardiac involvement and significant accrueddamage.