What is Behcet’s disease?
Behcet’s Disease, also known as Silk Road disease, is a chronic (long-term) inflammatory condition, caused by vasculitis (inflammation of the blood vessels), which will affect and damage both arteries and veins.
Symptoms of Behcet’s disease
The main symptoms of Behçet’s disease include:
- Genital and mouth ulcers
- Red, painful eyes and blurred vision
- Acne-like spots
- Painful, stiff, and swollen joints
In severe cases, there is also the risk of serious and life-threatening problems, such as permanent vision loss and strokes. Most people with the condition experience episodes in which symptoms are severe (flare-ups or relapses), followed by periods in which symptoms disappear (remission). Over time, some of the symptoms may subside and become less bothersome, although they may never fully resolve.
Causes of Behcet’s disease
The cause of Behçet syndrome is unknown. The disease is comparatively rare, but it is more common and severe in patients from the eastern Mediterranean and Asia than in those of European descent. Both hereditary (genetic) and environmental factors, such as microbial contagions, are suspected to be contributing factors to the development of Behçet’s disease. Behçet’s disease is not known to be contagious.
Treatment of Behcet’s disease
Treatment for Behcet’s disease hinges on the harshness of your condition. Mild cases can be treated with anti-inflammatory medications, such as ibuprofen (Advil, Motrin). You may only need medicine when you are having an outbreak. You may not need to take any medications when the disease is in remission.
Topical ointments that contain corticosteroids may be helpful in treating skin sores. Corticosteroid mouthwashes can help reduce the pain of mouth sores and help them go away more quickly. Similarly, corticosteroid eye drops or other anti-inflammatory medications can ease your discomfort if your eyes are affected.
In severe cases, a strong anti-inflammatory drug called colchicine (Colcrys) is sometimes prescribed. Colchicine is generally prescribed to treat gout. It can be particularly helpful in relieving joint pain associated with Behcet’s disease. Colchicine and other strong anti-inflammatory medications may be needed between flare-ups to help limit the damage caused by symptoms.
Other medications that may be prescribed between flare-ups include immunosuppressant medications, which help prevent your immune system from attacking healthy tissue. Some examples of immunosuppressive medications include:
- Azathioprine (Azasan, Imuran)
- Cyclosporine (Sandimmune)
- Cyclophosphamide (Cytoxan, Near)
Diagnosis of Behcet’s disease
There is no exact test to diagnose Behcet’s disease. Rather, the diagnosis is based on the appearance of symptoms and signs consistent with the disease. The presence of certain features that are particularly characteristic (eg, oral or genital ulcerations), elimination of other possible causes of the patient’s symptoms, and, if possible, testing for vasculitis by biopsy of an affected organ, all would support Behcet’s diagnosis.
A positive pathergy test can support the diagnosis of Behcet, but it is not a diagnosis of the disease in itself. A pathergy test is a humble test in which the preparation is pricked with a small, sterile needle. A small red bump or pustule at the needle insertion site is a positive test. Note that although a positive pathergy test helps diagnose Behcet, only a minority of Behcet’s patients demonstrate the phenomenon of pathergy (i.e., they have positive tests). Patients in the Mediterranean region are more likely to demonstrate pathergy. Also, other circumstances can infrequently result in positive pathergy tests, so the test is not 100% specific.
Risk factors for Behcet’s disease
Factors that can increase your risk of getting Behcet to include:
- Years: Behcet’s disease commonly affects men and women in their 20s and 30s, although children and older adults can develop the condition as well.
- Where you live: People from countries in the Middle East and East Asia, including Turkey, Iran, Japan, and China, are more likely to develop Behcet.
- Gender: Although Behcet’s disease occurs in both men and women, the disease is usually more severe in men.
- Genes: Having certain genes is associated with an increased risk of developing Behcet.
Complications of Behcet’s disease
The complications of this disease depend on your signs and symptoms. For example, untreated uveitis can lead to decreased vision or blindness. People with eye signs and symptoms of Behcet’s disease should see an eye specialist (ophthalmologist) frequently because treatment can assistance prevent this complication.
It can be a chronic (long-lasting) condition. Symptoms sometimes decrease over time and disappear over several years.
There is no way to prevent Behcet’s disease.