title: Paediatric Behçet's disease: a UK tertiary centre experience.
abstract: There are currently limited data regarding paediatric Behçet's disease (BD), particularly in the UK. We describe the clinical spectrum, treatment and outcome of BD, and explore the relative sensitivities of the criteria for the diagnosis of BD in a UK paediatric cohort. Single retrospective case note review of children with a clinical diagnosis of BD presenting between 1987 and 2012. Demographics, clinical features, treatment and outcomes were recorded. The sensitivities of the International Study Group (ISG) and International Criteria for BD (ICBD) criteria were explored. BD disease activity was calculated using the Behçet's Disease Activity Index (BDAI). Forty-six patients (22 male) were identified. Median age of onset was 4.87 (0.04-15.71) years; median time to diagnosis was 3.74 (0.25-13.48) years. Clinical features were recurrent oral ulceration (97.8 %), recurrent genital ulceration (73.9 %), gastrointestinal (58.7 %), musculoskeletal (47.83 %), cutaneous (23.9 %) involvement and uveitis (2 %). Recurrent genital ulceration was more common in female patients (P = 0.044). Thirty-seven patients (80.4 %) fulfilled the ICBD criteria; only 12 patients (26.1 %) fulfilled the ISG criteria. BDAI score at diagnosis was 7/20 (0-10/20) and significantly decreased to 5/20 (0-9/20) (P < 0.0001) at latest follow-up. The commonest systemic treatment was colchicine (76.1 %); anti-TNFα treatment was reserved for severe cases (15.5 %). Paediatric BD in the UK may present very early in life, sometimes with a family history, and with a low incidence of ocular involvement. Diagnostic delay is common. The majority of our patients required systemic therapy; anti-TNFα was reserved for severe cases and has largely superseded the use of thalidomide.
authors.affiliation: Infection, Inflammation and Rheumatology Section, UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, 30 Guilford Street, London, WC1N 1EH, UK. s.nanthapisal@ucl.ac.uk.
authors.forename: Sira
authors.initials: S
authors.lastname: Nanthapisal
authors.affiliation: Infection, Inflammation and Rheumatology Section, UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, 30 Guilford Street, London, WC1N 1EH, UK.
authors.forename: Nigel J
authors.initials: NJ
authors.lastname: Klein
authors.affiliation: Department of Rheumatology, University College London Hospital NHS Foundation Trust, London, UK.
authors.forename: Nicola
authors.initials: N
authors.lastname: Ambrose
authors.affiliation: Infection, Inflammation and Rheumatology Section, UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, 30 Guilford Street, London, WC1N 1EH, UK.
authors.forename: Despina
authors.initials: D
authors.lastname: Eleftheriou
authors.affiliation: Infection, Inflammation and Rheumatology Section, UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, 30 Guilford Street, London, WC1N 1EH, UK.
authors.forename: Paul A
authors.initials: PA
authors.lastname: Brogan
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D001528:Behcet Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D002648:Child; D002675:Child, Preschool; D057210:Delayed Diagnosis; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome; D006113:United Kingdom
chemical_list: D007166:Immunosuppressive Agents
keywords: Behçet’s disease; Children; Clinical features; Diagnostic criteria; Vasculitis
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