Behçet's Disease Epidemiology and Clinical Management
Summary
Behçet’s disease is a chronic, relapsing multisystem vasculitis with a striking geographical gradient, being most common along the historic Silk Road but reported worldwide. Its aetiology is multifactorial, involving a strong genetic predisposition—most notably HLA-B51—interacting with environmental triggers such as microbial agents. The clinical spectrum ranges from recurrent oral and genital ulcers and cutaneous lesions to sight-threatening uveitis, vascular inflammation with thrombosis, neurological involvement and gastrointestinal ulceration. Disease severity and expression vary by ethnicity, sex and age, making early recognition and stratification essential.
Management has evolved from broad immunosuppression to a more targeted, organ-specific approach. Conventional agents such as colchicine, azathioprine and ciclosporin remain first-line for mucocutaneous and joint manifestations, while high-dose systemic corticosteroids are reserved for major organ flares. Over the past decade, biologic therapies—particularly TNF-alpha inhibitors—have proven transformative for refractory ocular, neurological and vascular disease. Long-term care emphasises treat-to-target strategies, regular monitoring for organ damage and minimisation of cumulative steroid exposure.
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Behçet's Disease Epidemiology and Clinical Management publication trend
The graph below shows the total number of articles in behçet's disease epidemiology and clinical management across all publications each year (not limited to Nature Index journals).
Technical terms
Vasculitis: Inflammation of blood vessel walls.
Mucocutaneous: Relating to mucous membranes and skin.
Uveitis: Inflammation of the uveal tract of the eye.
TNF-alpha: Pro-inflammatory cytokine targeted by biologic therapies.
HLA-B51: Genetic marker strongly associated with disease susceptibility.
References
- Behcet’s Disease: Is There Geographical Variation? A Review Far from the Silk Road. International Journal of Rheumatology (2015).
- Behçet’s Disease: An Overview of Etiopathogenesis. Frontiers in Immunology (2019).
- Efficacy and safety of infliximab or adalimumab in severe mucocutaneous Behçet's syndrome refractory to traditional immunosuppressants: a 6-month, multicentre, randomised controlled, prospective, parallel group, single-blind trial. Annals of the Rheumatic Diseases (2024).
- Decoding Behcet’s Uveitis: an In-depth review of pathogenesis and therapeutic advances. Journal of Neuroinflammation (2024).
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