Behcet’s syndrome is a disease that involves inflammation of the blood vessels. It causes problems in many parts of the body. The most common symptoms are:
- Sores in the mouth
- Sores on the sex organs
- Other skin sores
- Swelling of parts of the eye
- Pain, swelling and stiffness of the joints
More serious problems can include meningitis, blood clots, inflammation of the digestive system and blindness.
Doctors aren’t sure what causes Behcet’s. It is rare in the United States, but is common in the Middle East and Asia. It mainly affects people in their 20s and 30s. Diagnosing Behcet’s can take a long time, because symptoms may come and go, and it may take months or even years to have all of the symptoms. There is no cure. Treatment focuses on reducing pain and preventing serious problems. Most people can control symptoms with treatment.
Common Signs & Symptoms
Behcet’s Disease is common in the Middle East, Asia, and Japan. It is rare in the United States. In Middle Eastern and Asian countries, the disease affects more men than women. In the United States, it affects more women than men.
The exact cause of Behcet’s Disease is unknown. Behcet’s Disease tends to develop in people in their 20’s or 30’s, but all age groups may be affected. Behcet’s Disease is an autoinflammatory disease resulting in damage to blood vessels throughout the body, particularly veins. It is a form of vasculitis (an inflammation of the blood vessels). Inflammation is a characteristic reaction of the body to injury or disease and is marked by four signs: swelling, redness, heat, and pain.
Doctors think that an autoimmune reaction may cause the blood vessels to become inflamed, but they do not know what triggers this reaction. Under normal conditions, the immune system protects the body from diseases and infections by killing harmful “foreign” substances. In an autoimmune reaction, the immune system mistakenly attacks and harms the body’s own tissues.
Behcet’s Disease is not contagious; it does not spread from one person to another and affects each person differently. The four most common symptoms are mouth sores, genital sores, inflammation of the internal portions of the eye, and skin problems. Inflammation inside of the eye (uveitis, retinitis, and iritis) may cause blurred vision, pain and redness. Other symptoms of the disease may include arthritis, blood clots, and inflammation in the central nervous system and digestive organs.
Diagnosing Behçet’s disease is very difficult to diagnose because no specific tests confirm it. When a patient reports symptoms, the doctor must examine the patient and rule out other conditions with similar symptoms. Because it may take several months or even years for all the common symptoms to appear, the diagnosis may not be made for a long time and is often a retrospective diagnosis. A patient may even visit several different kinds of doctors before the diagnosis is made.
International Criteria for Behçet’s disease:
- Mouth sores (oral ulcers) at least three times in 12 months
- Any two of the following:
- Recurring genital sores/ulcers
- Eye inflammation with loss of vision
- Characteristic skin lesions
- ositive pathergy (skin prick test)
Besides finding these signs, the doctor must rule out other conditions with similar symptoms, such as Lupus, Crohn’s disease and Rheumatoid Arthritis. The doctor also may recommend that the patient see an ophthalmologist to identify possible complications related to eye inflammation. A dermatologist may perform a biopsy of mouth, genital, or skin lesions to help distinguish Behçet’s from other disorders.
There is no specific “Behçet’s’test”. Consequently, the diagnosis is based on the occurrence of symptoms and signs that are compatible with the disease, the presence of certain features that are particularly characteristic (e.g., oral or genital ulcerations), elimination of other possible causes of the patient’s presentation, and – whenever possible – proof of vasculitis by biopsy of an involved organ.
There is no cure for Behçet’s disease. Treatment typically focuses on reducing discomfort and preventing serious complications. Corticosteroids and other medications that suppress the immune system may be prescribed to treat inflammation. Behçet’s is a chronic disease that recurs. However, patients may have periods of time when symptoms go away temporarily (remission). The severity of the disease varies from patient to patient. Some patients may live somewhat normal lives, but others may become blind or severely disabled.
Behçet’s disease affects different parts of the body, therefore, a patient probably will see several different doctors. It may be helpful to both the doctors and the patient for one doctor to manage the complete treatment plan. This doctor can coordinate the treatments and monitor any side effects from the various medications that the patient takes.
A rheumatologist (a doctor specializing in arthritis and other inflammatory disorders) often manages a patient’s treatment and treats joint disease. The following specialists also treat other symptoms that affect the different body systems:
- Gynecologist-treats genital sores in women
- Urologist-treats genital sores in men
- Dermatologist-treats genital sores in men and women, and skin and mucous membrane problems
- Ophthalmologist-treats eye inflammation
- Gastroenterologist-treats digestive tract symptoms
- Hematologist-treats disorders of the blood
- Neurologist-treats central nervous system symptoms
Although there is no cure for Behçet’s disease, people usually can control symptoms with proper medication, rest, exercise, and a healthy lifestyle. The goal of treatment is to reduce discomfort and prevent serious complications such as disability from arthritis or blindness. The type of medicine and the length of treatment depend on the person’s symptoms and their severity.It is likely that a combination of treatments will be needed to relieve specific symptoms. Patients should tell each of their doctors about all of the medicines they are taking so that the doctors can coordinate treatment.