PE Şenol, DG Yıldırım, RC Kardaş, EN Sunar Yayla, Ç Yıldız, N Karaçayır, MA Öztürk, SA Bakkaloğlu

Abstract

Background: Behçet's disease (BD) is a multifactorial auto-inflammatory disorder that affects various organ systems. This study aims to provide a comprehensive analysis of the clinical features, diagnostic approaches, and treatment strategies employed in BD. Methods: We conducted a thorough review of the literature to gather pertinent information on the clinical manifestations, diagnostic criteria, and therapeutic modalities for BD. We meticulously examined the findings from relevant studies and guidelines, synthesising key insights. Results: BD is characterised by recurring oral aphthae, genital ulcers, ocular involvement, and cutaneous manifestations. Despite primarily affecting individuals in the 20-40 year age range, a notable percentage of cases exhibit paediatric onset. Because there are no definitive laboratory tests, the diagnosis of BD relies primarily on clinical criteria. Proposals for multiple diagnostic frameworks exist, and the disease phenotype shows significant heterogeneity across ethnicities, geographical regions, and age cohorts. Genetic factors, particularly HLA-B51 positivity, confer increased susceptibility to BD. Different types of treatments are available depending on how many organs are affected. These may include colchicine, corticosteroids, traditional and biologic disease-modifying anti-rheumatic drugs, and targeted therapies like TNF-α inhibitors, anti-interleukin-1, and anti-interleukin-6 agents. However, determining the optimal therapeutic approach remains an area of active investigation and necessitates personalised patient management. Conclusion: Patients with BD diagnosed at different ages or genders may exhibit different clinical findings. Especially, more severe organ involvement and a higher disease activity-related BD may exist in adults than in children with BD. Adult patients needed more intense immunosuppressive treatment.