Overview
Behcet's disease (BD) is a chronic, multisystem inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions, uveitis, and variable involvement of other organs such as the gastrointestinal tract, vascular system, and central nervous system. The condition is classified as an autoimmune or autoinflammatory disease, though its exact etiology remains unclear. BD predominantly affects young adults, with a peak incidence between the ages of 20 and 40, and has a higher prevalence in regions along the ancient Silk Road, particularly in Turkey, Japan, and Middle Eastern countries 3. Understanding and managing BD is crucial in day-to-day practice due to its potential for significant morbidity and the need for long-term multidisciplinary care to prevent organ damage and improve quality of life 13.Pathophysiology
The pathophysiology of Behcet's disease involves complex interactions at molecular, cellular, and organ levels. Central to BD is an aberrant immune response, likely triggered by environmental factors in genetically predisposed individuals. Genetic studies suggest associations with HLA-B51, particularly in populations with higher disease prevalence, indicating a role for antigen presentation in disease initiation 3. At the cellular level, there is evidence of both innate and adaptive immune dysregulation. Neutrophilic activation and increased levels of pro-inflammatory cytokines, such as TNF-α, IL-1, and IL-6, contribute to the chronic inflammation observed in BD 1. This inflammatory milieu leads to endothelial dysfunction and vascular permeability, explaining the diverse organ involvements seen in BD, including vasculitis affecting small to medium-sized vessels 15. Additionally, dysregulation in lipid metabolism and plasma lipoprotein pathways may exacerbate inflammatory processes, further complicating the disease's progression 5.Epidemiology
Behcet's disease has a varying incidence and prevalence globally, with significant regional disparities. The prevalence ranges from 10 to 400 cases per 100,000 population, with higher rates reported in Turkey (approximately 86 cases per 100,000), Japan, and certain Middle Eastern countries 3. The disease predominantly affects young adults, with a male predominance in early onset but a more balanced sex ratio in later presentations. Geographic distribution suggests a possible genetic predisposition or environmental triggers specific to these regions. Over time, there has been an increasing recognition and diagnosis of BD, partly due to improved awareness and diagnostic criteria, though true incidence trends are challenging to discern without standardized global reporting 3.Clinical Presentation
The clinical presentation of Behcet's disease is characterized by a constellation of symptoms involving multiple organ systems. Classic manifestations include recurrent oral and genital ulcers, skin lesions (such as erythema nodosum and acneiform nodules), and uveitis leading to ocular complications like retinal vasculitis. Atypical presentations can involve gastrointestinal symptoms (such as abdominal pain, bowel perforation), vascular manifestations (aneurysms, thrombosis), and neurological involvement (headaches, stroke-like episodes). Red-flag features include severe ocular inflammation leading to vision loss, vascular occlusions, and gastrointestinal perforations, which necessitate urgent intervention 13.Diagnosis
Diagnosing Behcet's disease relies on a combination of clinical criteria and exclusion of other conditions. The International Study Group (ISG) criteria, widely accepted, require recurrent oral ulcers plus any two of the following: genital ulcers, skin lesions, uveitis, and positive pathergy test (a sterile inflammatory response to subcutaneous injection of saline). More recent updates emphasize the importance of clinical judgment and exclusion of other autoimmune diseases 1.Management
The management of Behcet's disease is multifaceted, aiming to control inflammation, prevent complications, and improve quality of life.First-Line Treatment
Second-Line Treatment
Refractory Cases / Specialist Escalation
Complications
Behcet's disease can lead to significant long-term complications, necessitating vigilant monitoring and timely intervention.Prognosis & Follow-up
The prognosis of Behcet's disease varies widely among patients, influenced by the extent and severity of organ involvement. Prognostic indicators include early onset of severe ocular or vascular complications. Regular follow-up is essential, typically every 3-6 months, focusing on:Special Populations
Pregnancy
Pregnancy in women with Behcet's disease requires careful management to prevent flare-ups and ensure maternal and fetal safety. Corticosteroids and colchicine are generally considered safe, while immunosuppressants like azathioprine and TNF inhibitors should be used cautiously or discontinued pre-conception and during pregnancy 1.Pediatrics
In pediatric patients, the disease course can be more aggressive, necessitating early and aggressive treatment. First-line therapies include colchicine and corticosteroids, with close monitoring for growth and development 1.Elderly
Elderly patients may have more comorbidities and are at higher risk for complications like vascular events. Management focuses on minimizing immunosuppression while controlling symptoms, often requiring a multidisciplinary approach 1.Key Recommendations
References
1 Wang Y, Chang X, Deng S, Tang S, Chen P. Functional material probes and advanced technologies in organ-on-a-chip characterization. Theranostics 2026. link 2 Li K, Payne T, Francis R, Hubbard RE, Gordon EH. A systematic review of frailty changes following solid organ transplantation: Is it all about the frailty tool?. Transplantation reviews (Orlando, Fla.) 2026. link 3 Bilsel Y, Bektas H, Tilki M. The impact of Western physicians on the modernization of Turkish surgery and medicine, 1827-1936. World journal of surgery 2010. link 4 Kalter ES, de By TM. Tissue banking programmes in Europe. British medical bulletin 1997. link 5 Cramp DG, Tickner TR, Wills MR. Controlled storage of biological energy: The role of plasma lipoproteins. Lancet (London, England) 1976. link92348-5)