title: Eosinophilic granulomatosis with polyangiitis in childhood: retrospective experience from a tertiary referral centre in the UK.
abstract: To describe the presenting clinical features, treatment and outcome in children with eosinophilic granulomatosis with polyangiitis (EGPA) and to define factors that predicted mortality.
  
  
  
  A retrospective case notes review of patients fulfilling the Chapel Hill Consensus Conference definition 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 expressed as median and range. Fisher's exact test was used for group comparisons. The Paediatric Vasculitis Activity Score and Paediatric Vasculitis Damage Index (PVDI) were calculated.
  
  
  
  Thirteen children (38% female) aged at diagnosis 14.1 (4-15.6) years were identified. The median time to diagnosis was 2 (0-7.3) years. History of asthma was documented in 76%. The most common presenting features were pulmonary (69%), skin (61%), gastrointestinal (46%), cardiac involvement (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 negative in all 10/13 patients tested. Treatment included corticosteroids in all, combined with CYC in 38% or AZA in 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).
  
  
  
  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 since our data emphasize a high mortality, particularly in those with cardiac involvement and significant accrued damage.
authors.affiliation: Paediatric rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, Institute of Child Health and ARUK centre for adolescent rheumatology, UCL, London d.eleftheriou@ucl.ac.uk.
authors.forename: Despina
authors.initials: D
authors.lastname: Eleftheriou
authors.affiliation: College of Medicine and Vetinary Medicine, Edinburgh.
authors.forename: Hugo
authors.initials: H
authors.lastname: Gale
authors.affiliation: Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust.
authors.forename: Clarissa
authors.initials: C
authors.lastname: Pilkington
authors.affiliation: Paediatric Cardiology, Great Ormond Street Hospital for Children NHS Foundation Trust.
authors.forename: Matthew
authors.initials: M
authors.lastname: Fenton
authors.affiliation: Histopathology, Great Ormond Street Hospital for Children NHS Foundation Trust.
authors.forename: Neil J
authors.initials: NJ
authors.lastname: Sebire
authors.affiliation: Paediatric rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, Institute of Child Health, UCL, London.
authors.forename: Paul A
authors.initials: PA
authors.lastname: Brogan
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D009202:Cardiomyopathies / Q000209:etiology / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D004803:Eosinophilic Granuloma / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D006801:Humans; D008131:London; D008297:Male; D012008:Recurrence; D012189:Retrospective Studies; D012720:Severity of Illness Index; D062606:Tertiary Care Centers; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones
keywords: asthma; children; eosinophilia; eosinophilic granulomatosis with polyangiitis