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

Area of Science:

• Rheumatology
• Pediatrics
• Immunology

Background:

• Limited data exists on pediatric Behçet's disease (BD) in the UK.
• Understanding the clinical spectrum, treatment, and outcomes is crucial for this rare condition.

Purpose of the Study:

• To describe the clinical features, treatment, and outcomes of pediatric BD in a UK cohort.
• To evaluate the diagnostic sensitivity of the International Study Group (ISG) and International Criteria for BD (ICBD) criteria.
• To analyze disease activity using the Behçet's Disease Activity Index (BDAI).

Main Methods:

• Retrospective review of medical records of children diagnosed with BD between 1987 and 2012.
• Data collected included demographics, clinical manifestations, treatments, and outcomes.
• Analysis of ISG and ICBD criteria sensitivity and BDAI scores.

Main Results:

• Forty-six pediatric patients were identified with a median age of onset of 4.87 years.
• Recurrent oral ulceration (97.8%) was the most common feature; gastrointestinal and musculoskeletal involvement were also frequent.
• The ICBD criteria (80.4%) were more sensitive than ISG criteria (26.1%) for diagnosis. Median BDAI scores decreased significantly from diagnosis to follow-up.
• Colchicine was the most common systemic treatment (76.1%), with anti-TNFα reserved for severe cases.

Conclusions:

• Pediatric BD in the UK can present early, often with diagnostic delays.
• The ICBD criteria demonstrate higher sensitivity in this pediatric cohort compared to ISG criteria.
• Systemic therapy is frequently required, with anti-TNFα agents playing a role in severe disease management.