Pediatric Behçet’s disease

Behçet’s disease (BD) is a vasculitis that affects vessels of any size. It is more frequent along the ancient Silk Road, extending from the Far East to the Mediterranean basin. Its etiopathogenesis is complex, and both the innate and adaptive immune systems play a role in recurrent hyperinflammation. The significant association between human leukocyte antigen B-51 and BD indicated a strong genetic background in pathogenesis. Although mucocutaneous involvement is the most common finding, it may present with a broad spectrum of clinical signs and symptoms involving the ocular, vascular, musculoskeletal, neurologic, and gastrointestinal systems. Pediatric cases may present with an incomplete clinical picture of the BD, making diagnosis difficult for the physicians. Several classification criteria have been published so far. In 2015, a classification criteria set for pediatric BD (PEDBD) was established for the first time. The treatment strategies vary depending on the severity and type of organ involvement. The treatment should be arranged with a multidisciplinary approach according to the organs involved. Also, the possibility of developing morbidity and mortality requires early diagnosis, appropriate treatment, and close follow-up. In this review, we aimed to discuss the etiopathogenesis, clinical findings, diagnostic criteria, and treatment approach of pediatric BD based on current data.