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 The majority of our patients required systemic therapy; anti-TNFα was reserved for severe cases and has largely superseded the use of thalidomide.Electronic supplementary materialThe online version of this article (doi:10.1007/s10067-016-3187-z) contains supplementary material,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

Mucocutaneous Findings in Behçet’s Disease

“…The lowest mean age of 4.8 years old was reported by Nanthapisal et al [134]. Similarly to clinical symptoms of adulthood BD, OU was present in most of the children and also constituted the initial symptom in most of the cases [68,[131][132][133][134][135][136][137][138][139]. The localization and morphology of OU in children were also similar to the characteristics of adulthood OU [136,137,140].…”

“…Distinctively from adult cases, the presence of GU has been reported significantly lesser than adults [131,132]. However, Treudler et al did not reveal any differences in the ratio of GU between childhood and adulthood BD [68], and Nanthapisal et al detected recurrent GU more frequently in females [134]. The characteristics of GU were also found similar to the ones seen in adults [136,137].…”

“…The onset of BD has been reported at any age between 0 and 16 years [131,136,137]. The lowest mean age of 4.8 years old was reported by Nanthapisal et al [134]. Similarly to clinical symptoms of adulthood BD, OU was present in most of the children and also constituted the initial symptom in most of the cases [68,[131][132][133][134][135][136][137][138][139].…”

Recurrent oral ulcerations following heart transplant in a pediatric patient: A diagnostic dilemma

“…Behçet's disease usually presents with oral ulcers and can be a diagnostic challenge as it has a variety of manifestations including recurrent oral or genital ulcers, gastrointestinal complaints, arthralgia/arthritis, fever, skin, neurologic, and ocular involvement . Behçet's disease is relatively rare in northern Europe and North America and rarer still in pediatric populations .…”

“…Steroid‐sparing agents, such as azathioprine, are frequently used, and for the most severe cases, TNF‐α inhibitors can be effective. The natural history of Behçet's is difficult to predict in children with symptoms worsening in the first few years after diagnosis though generally disappearing throughout adulthood …”