Abstract: Behçet’s disease is a complex systemic vasculitis characterized by recurrent oral and genital ulcers, uveitis, and skin lesions. This article provides an in-depth review of Behçet’s disease, including its epidemiology, pathogenesis, clinical manifestations, diagnostic criteria, treatment approaches, and recent advancements. Understanding the multi-system involvement and evolving management strategies is essential for optimal care of patients with Behçet’s disease.

Introduction: Behçet’s disease, also known as Behçet syndrome, is a chronic relapsing inflammatory disorder with systemic manifestations. Although its etiology remains incompletely understood, genetic predisposition, environmental factors, and immune dysregulation are thought to contribute to its pathogenesis.

Epidemiology: Behçet’s disease primarily affects individuals of Mediterranean, Middle Eastern, and Asian descent, with a higher prevalence along the ancient Silk Road. The peak age of onset is between the second and fourth decades of life, with a slight male predominance.

Pathogenesis: Behçet’s disease is characterized by immune-mediated vasculitis affecting blood vessels of all sizes. Aberrant T-cell and neutrophil activation, along with pro-inflammatory cytokine release, contribute to vascular inflammation and tissue damage.

Clinical Manifestations: Behçet’s disease manifests with a wide spectrum of symptoms, including recurrent oral and genital ulcers, uveitis, skin lesions (e.g., erythema nodosum, pseudofolliculitis), arthritis, gastrointestinal involvement, and central nervous system manifestations (e.g., aseptic meningitis, cerebral venous thrombosis).

Diagnostic Criteria: The diagnosis of Behçet’s disease is based on clinical criteria, with the International Criteria for Behçet’s Disease (ICBD) being commonly used. Criteria include recurrent oral ulceration plus two of the following: recurrent genital ulceration, eye lesions, skin lesions, or a positive pathergy test.

Treatment Approaches: Management of Behçet’s disease aims to control inflammation, prevent relapses, and minimize organ damage. Treatment modalities include topical and systemic corticosteroids, immunosuppressive agents (e.g., azathioprine, cyclosporine), biologic therapies (e.g., anti-TNF agents), and supportive measures tailored to individual patient needs.

Recent Advancements: Recent advancements in the treatment of Behçet’s disease include the use of biologic therapies targeting specific inflammatory pathways, such as interleukin-17 inhibition. Additionally, ongoing research is exploring novel therapeutic agents and strategies aimed at achieving sustained remission and improving long-term outcomes.

Conclusion: Behçet’s disease is a complex multisystem disorder with diverse clinical manifestations and variable disease course. Through a multidisciplinary approach involving rheumatologists, ophthalmologists, dermatologists, and other specialists, patients with Behçet’s disease can receive comprehensive care aimed at controlling inflammation and improving quality of life.

For further reading and reference:

• International Society for Behçet’s Disease (ISBD): https://www.behcetsdisease.org/
• Behçet’s Syndrome Society (UK): https://behcets.org.uk/