Behçet's syndrome is a chronic disorder in which many body systems including the eyes, the joints, the blood vessels, the central nervous system, the digestive system and the genitals become swollen and tender (inflamed).
Because this disorder affects many organs, Behçet's syndrome has many symptoms, including:
- Painful ulcers in the mouth. These are usually the first symptom of Behçet's. They resemble canker sores.
- Ulcers on the genitals. When they occur on the penis and scrotum, they can be quite painful. In women, there may be no symptoms.
- Diseases of the eye. Appearing in most patients who have Behçet's syndrome, these cause pain, sensitivity to light and hazy vision. Without treatment, they can cause blindness.
- Skin sores or swelling, redness and tenderness due to minor injuries such as a needle puncture
- Mild arthritis of the knees and other large joints. This develops in about half of all patients with Behçet's.
- Inflamed veins and blood clots. If the inflammation and clot affect the two large veins that return blood to the heart (the inferior and the superior vena cava), there can be life-threatening complications.
- Central nervous system disorders. This range from chronic swelling and inflammation of the brain and the membranes that cover it (meningoencephalitis) to increased pressure inside the skull to life-threatening brain stem and spinal cord lesions.
- Disorders of the stomach and intestines. This can range from discomfort to symptoms that resemble Crohn's disease.
- Swelling and pain in a blood vessel anywhere in the body. This can lead to aneurysms (weak places in the vessel that thin and bulge out) or blood clots. The tiny blood vessels of the kidneys may be affected without symptoms being apparent (asymptomatic focal glomerulonephritis).
Causes and Risk Factors
It is not yet known what causes Behçet's. Cases in Japan and the Mediterranean area suggest that viruses may cause it. There may be genetic factors involved. Another possibility is that the body's defense system (immune system) attacks its own tissues (an autoimmune disorder).
The syndrome generally begins when a person is in his or her thirties. Men are twice as likely as women to develop it. People whose families once lived along the ancient silk route in Mediterranean and some Asian countries, especially Japan, have a greater risk of developing Behçet's syndrome.
There are no findings that can confirm Behçet's and rule out other similar conditions. It may take months to rule out other conditions such as Reiter's syndrome, lupus, Crohn's disease, ulcerative colitis, ankylosing spondylitis and herpes simplex infection.
Behçet's symptoms typically appear and disappear over time and involve many organs. Laboratory tests usually show signs of inflammatory disease.
Symptoms of Behçet's syndrome may appear for weeks, years or even decades and then disappear for weeks, years or even decades. If the symptoms are severe enough to cause blindness, block the vena cava or cause paralysis, Behçet's can be difficult to manage. Symptoms that affect the nerves, blood vessels or digestive system can become life threatening.
Treatment usually focuses on eliminating symptoms. Drugs may be used to make mouth or genital ulcers less painful and less frequent. Corticosteroids in the mouth or eye may temporarily relieve some symptoms, although they don't prevent symptoms from coming back.
Because the body of a person who has Behçet's reacts so intensely to minor injuries such as needle sticks, they should be avoided when possible. Occasional patients with severe symptoms respond to high-dose corticosteroids.