Diagnosis, treatment and monitoring of pediatric Behçet's disease: Systematic literature review informing the ISSAID/PRES recommendations.
Autoimmun RevJul 11, 2026 (epub)
Maria Vincenza Mastrolia, Micol Romano, David Piskin et al.
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In 30 seconds
This systematic review analyzed 52 studies involving 2929 pediatric patients with Behçet's disease (BD), highlighting its distinct clinical features and treatment variability. The study found that 50% of patients were HLA-B51 positive, and mucocutaneous manifestations were present in 97.8% of cases. The findings aim to inform recommendations by ISSAID and PReS.
Key findings
- 50% of pediatric BD patients were HLA-B51 positive (95% CI, 0.5-0.6).
- Mucocutaneous manifestations were present in 97.8% of patients.
- Musculoskeletal and ocular manifestations occurred in 36% and 35% of patients, respectively.
- The lowest median age of onset was 1 year, with diagnosis at 2 years.
Why it matters
Understanding the clinical features and treatment approaches for pediatric Behçet's disease is crucial for improving diagnosis and management. This review provides insights into the disease's variability, which can guide clinicians in tailoring care for affected children.