Behçet’s disease: diagnostic and therapeutic challenges in the context of a rare multisystem disorder

Abstract

Background. Behçet’s Disease (BD) is a chronic, recurrent, multisystem vasculitis of unknown etiology, primarily affecting small- and medium-sized vessels. It is more commonly encountered in regions along the former “Silk Road,” particularly in Turkey, the Middle East, and East Asia, but remains a rare in Europe. Objective(s). To present the main clinical manifestations, diagnostic criteria and current therapeutic approaches in Behçet’s Disease, with a focus on the importance of multidisciplinary evaluation and treatment. Materials and methods. This work is based on a review of recent specialized literature (from the past 10 years), including international guidelines (EULAR), original articles, metaanalyses, and case studies. The international classification criteria (ICBD) are described, and the main therapeutic options are analyzed based on disease severity and organ involvement. Results. The clinical manifestations of the disease are varied: recurrent oral ulcers (95%) and genital ulcers (68%), skin lesions (erythema nodosum, pseudo-folliculitis) (65%), ocular involvement – uveitis (40%), neurological (20%), articular (50%), gastrointestinal (15%), and cardiovascular (<10%) involvement. Diagnosis is clinical, supported by standardized scoring systems. Treatment involves immunomodulatory and immunobiologic agents, tailored according to the severity and the organs involved. The prognosis depends on the disease onset, the response to conventional immunosuppressive treatment, and the patient’s quality of life. Conclusion(s). Behçet’s Disease presents both diagnostic and therapeutic challenges due to its heterogeneous nature and the lack of specific biological markers. Early diagnosis and individualized therapeutic approaches, coordinated by a multidisciplinary team, are essential for reducing complication risks.