Paediatric Behçet's disease: a UK tertiary centre experience.

Sira Nanthapisal,Nicola Ambrose,Nigel J Klein,Paul A. Brogan,Despina Eleftheriou

doi:10.1007/s10067-016-3187-z

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 The majority of our patients required systemic therapy; anti-TNFα was reserved for severe cases and has largely superseded the use of thalidomide.Electronic supplementary materialThe online version of this article (doi:10.1007/s10067-016-3187-z) contains supplementary material, which is available to authorized users.

Highlights

Behçet’s disease (BD) [1] is a chronic multisystem vasculitis characterized by recurrent oral and genital ulceration, uveitis, cutaneous, articular, neurological and gastrointestinal involvement [2,3,4,5,6,7,8]
We describe the largest single-centre United Kingdom (UK) paediatric cohort of BD which suggests differences compared with studies involving Asian or Turkish children (Table 4)
For the first time, we retrospectively examined the diagnostic sensitivity of the International Criteria for BD (ICBD) criteria in children

Summary

Introduction

Behçet’s disease (BD) [1] is a chronic multisystem vasculitis characterized by recurrent oral and genital ulceration, uveitis, cutaneous, articular, neurological and gastrointestinal involvement [2,3,4,5,6,7,8]. Clin Rheumatol (2016) 35:2509–2516 for 7.7–26.4 % of the reported prevalent cases of BD outside the UK [1, 3]. In one UK study, only one paediatric BD case was identified in a population of 1.1 million children [13]. Due to the rarity of the condition and its non-specific presentation, the diagnosis of BD is challenging for paediatricians, and diagnostic delay of several years is common. This may lead to irreversible complications such as blindness, neurological injury and other end-organ damage

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Conclusion