Advances and Challenges in Diagnosing Behçet’s Disease: A Comprehensive Review

Abstract

Behçet's Disease (BD) presents significant diagnostic and management challenges due to its complex and multifaceted nature. Initially described in 1937, BD primarily affects populations along the Silk Road, with a prevalence of 14.6 per 100,000 globally and 420 per 100,000 in Turkey. Its autoimmune nature and lack of definitive etiology make diagnosis reliant on clinical manifestations, with recurrent oral ulcers being a hallmark symptom. Recent research has shed light on the genetic underpinnings of BD, with HLA-B*51 being a significant susceptibility locus. Immunological dysregulation involving T and B lymphocytes, dendritic cells, and various cytokines contribute to the pathogenesis. Clinical manifestations vary widely, affecting multiple organ systems, with mucocutaneous and ocular symptoms being the most common. Diagnosis is primarily clinical, aided by classification criteria such as the ISG and ICBD criteria, although challenges persist due to overlapping features with other autoimmune disorders. Multidisciplinary collaboration among specialists is crucial for accurate diagnosis and optimal management.