Description
Also known as: silk road disease, Behçet’s disease is a systemic inflammatory vasculitis, characterized by a course of remission and relapse of oral ulcers, genital ulcers, skin lesions and ocular lesions. It can affect the arteries and veins of almost any system and has been shown to involve the gastrointestinal and neurological systems. The cause of Behçet’s is unknown but it is believed to be triggered by an infectious or environmental agent in a genetically predisposed individual.
Symptoms
Causes
Complications
Clinical Utility
The HLA class I molecule HLA-B51 is the most strongly associated risk factor and has been shown to be associated with Behçet’s in Turkish and Asian patients, though the association in Caucasian patients is much weaker. Testing for HLA-B51 is a useful, though not diagnostic, tool for Behçet’s as the vast majority of HLA-B51 carriers do not develop Behçet’s. Diagnosis is instead based on the recurrence of symptoms including oral ulcers, genital ulcers, skin lesions and ocular lesions. Treatment options depend on organs involved.
Behcet’s syndrome has no specific cure. Treatment includes medication to control signs and symptoms.
Scientific References
- Ahmad T et al. Mapping the HLA association in Behçet’s disease: a role for tumor necrosis factor polymorphisms? Arthritis Rheum. 2003; 48:807.
- de Menthon M et al. HLA-B51/B5 and the risk of Behçet’s disease: a systematic review and meta-analysis of case-control genetic association studies. Arthritis Rheum. 2009; 61:1287.
- Kötter I et al. Comparative analysis of the association of HLA-B*51 suballeles with Behçet’s disease in patients of German and Turkish origin. Tissue Antigens. 2001; 58:166.
- Mahr A et al. Population-based prevalence study of Behçet’s disease: differences by ethnic origin and low variation by age at immigration. Arthritis Rheum. 2008; 58:3951.
- Yazici H et al. Behçet’s syndrome is not so rare: why do we need to know? Arthritis Rheum. 2008; 58:3640.
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