Behçet disease

Behçet disease (also known as Behçet syndrome, Adamantiades-Behçet disease, or Silk Road disease) is a chronic, relapsing, multisystem inflammatory disorder classified as a variable vessel vasculitis. It can affect blood vessels of all sizes — arteries and veins — throughout the body. The disease is characterized by a classic triad of recurrent oral aphthous ulcers, genital ulcers, and ocular inflammation (uveitis), though it can involve virtually any organ system. Behçet disease is most prevalent along the ancient Silk Road, with the highest rates in Turkey, Iran, Japan, Korea, and other parts of the Middle East and East Asia. The disease affects multiple body systems including the mucocutaneous (skin and mucous membranes), ocular, vascular, musculoskeletal, gastrointestinal, and central nervous systems. Oral ulcers are typically the earliest and most common manifestation, occurring in nearly all patients. Ocular involvement, particularly posterior uveitis and retinal vasculitis, can lead to significant visual impairment or blindness if untreated. Vascular manifestations include deep vein thrombosis, arterial aneurysms, and arterial occlusions. Neuro-Behçet disease, affecting the central nervous system, can cause meningoencephalitis, cerebral venous sinus thrombosis, and progressive neurological disability. Gastrointestinal involvement may mimic inflammatory bowel disease with ulcerations throughout the intestinal tract. There is no cure for Behçet disease, and treatment is tailored to the organ systems involved and disease severity. Colchicine is commonly used for mucocutaneous and joint manifestations. Corticosteroids are employed for acute flares, while immunosuppressive agents such as azathioprine, cyclosporine, and cyclophosphamide are used for severe organ involvement, particularly ocular and neurological disease. Biologic therapies, especially anti-TNF agents (infliximab, adalimumab) and interferon-alpha, have shown significant efficacy in refractory cases. Apremilast has been approved for oral ulcers associated with Behçet disease. Early and aggressive treatment of ocular and vascular disease is critical to prevent irreversible damage.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Behçet disease