Name: Behcet's Disease
SKU: 59
Price: 135.00 USD
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Description

A rare chronic inflammatory disease of the blood vessels.

Extremely rare (Fewer than 1,000 cases per year in US)

Treatments can help manage condition, no known cure

Requires lab test or imaging

Can last several years or be lifelong

Common for ages 18-35

May be dangerous or life threatening

Family history may increase likelihood

Urgent medical attention recommended

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

Mouth and genital sores
Rashes and inflamed eyes
Pain in the eyes or joints
Rashes on the skin and the appearance of acne
Red and tender lumps on the skin
Visual problems such as sensitivity to light, blurred vision
Eye redness

Causes

The cause of Behcet’s syndrome is not known, but it is thought to be an autoimmune disorder where the body immune system attacks its own normal cells
Genetic and environmental factors may play a role
Some researches claim than Behcet’s syndrome can be triggered by a virus or bacterium in people with genes that may cause a predisposition to Behcet’s syndrome

Complications

Uveitis, a symptom of Behcet’s syndrome can lead to decreased vision or blindness
Vascular problems such as stroke can occur due to decreased blood flow to the brain
Formation of blood clots due to inflammation of arteries and veins

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.