Behcet's Disease or Behcet's Syndrome: Causes, Symptoms, Treatment, Investigations
Behcet's Syndrome is also known as Behcet's Disease.
Behcet's syndrome is a rarely caused disease. Behcet's syndrome is a condition which affects blood vessels present in all over the body making them chronically inflamed for a long period. The inflammation of the blood vessel is referred to as vasculitis. Vasculitis usually affects arteries and veins by damaging both of them. However, the accurate reason for Behcet's syndrome is still not known though this is suspected to be an autoimmune disorder which occurs when immune system of the body accidentally attacks its healthy cells. Behcet's disease may also occur due to environmental and genetic factors.
Treatment for Behcet's syndrome Usually concentrates on reducing its symptoms and preventing complications like blindness.
Epidemiology of Behcet's Disease or Behcet's Syndrome
Behcet's syndrome is very common in the Middle East and Mediterranean basin, about 300 cases out of 100,000 and about 15 cases out of 100,000 of Far East. In US, there are approximately 7 cases out of 100,000. Women and men both get affected with Behcet's syndrome. People between age group of 20 to 30 years often develop Behcet's syndrome.
Causes And Risk Factors of Behcet's Disease or Behcet's Syndrome
The exact cause of Behcet's syndrome is unknown. The suspected causes are environmental and genetic factors as many of the genes are suspected to be associated with Behcet's syndrome. Bacterium or virus may also result in Behcet's syndrome in individuals having certain genes making them more susceptible to the disease.
Factors That May Increase Your Risk of Behcet's Disease or Behcet's Syndrome Include
- Age: Behcet's syndrome is usually caused between 20 to 30 years of age. However, children and old people may also develop the disorder. Early age Behcet's syndrome may be even more severe in nature.
- Location: Behcet's syndrome is found all over the world. Individuals from countries like Turkey, China, Iraq, Middle East, Japan, Iran and Asia are also prone to the disease.
- Sex: Although men and women both get affected with Behcet's syndrome, it is more severe in men when compared to women.
- Genes: Certain genetic disorders can also increase the risk of Behcet's syndrome.
- Smoking: Cigarette smoke contains certain chemicals which damage the DNA increasing the risk of Behcet's syndrome.
Signs and Symptoms of Behcet's Disease or Behcet's Syndrome
The peculiar feature of Behcet's syndrome is that it may disappear and then again reoccur on its own without any intervention. Signs and symptoms of Behcet's syndrome typically depend upon the part of the body that gets affected. Given below are the commonly affected areas along with the symptoms:
- Mouth: Painful mouth sores or ulcers in the mouth that begin as raised round lesions which usually heal up in 7 to 21 days.
- Skin: Development of skin lesions may be caused in patients suffering from Behcet's syndrome. Patients may get these lesions in the form of tender red raised nodules or sores resembling acne.
- Genitals: Sores in the form of red, ulcerated lesions on the vulva or scrotum. These genital sores caused due to Behcet's syndrome may leave scars and are generally very painful.
- Eyes: Behcet's syndrome may lead to uveitis or inflammation of the middle layer of the eye. This may result in pain, blurred vision and redness in either or both eyes.
- Joints: Pain and swelling of the joint usually affects knees in individuals suffering from Behcet's syndrome. However this may also involve elbows, wrists and ankles.
- Vascular System: Large arteries and veins may get inflamed leading to swelling, pain and redness of legs or arms resulting from clotting of blood. Several symptoms are caused due to vasculitis which usually affects arteries and veins by damaging both of them. Large arteries when get inflamed result in complications like stenosis or narrowing or blockage of the vessel and aneurysms.
- Digestive System: Signs and symptoms of Behcet's syndrome affecting digestive system may include diarrhea, bleeding and abdominal pain.
- Brain: Behcet's syndrome may cause inflammation of the brain and nervous system leading to disorientation, headache, poor balance, fever and stroke.
Treatment of Behcet's Disease or Behcet's Syndrome
There is no proper cure available for treating Behcet's syndrome. Therefore, its treatments specifically focus on decreasing the inflammation and pain. In case of mild symptoms, medications are recommended for temporarily controlling flares of inflammation and pain. In case of more severe symptoms, systemic medicines are recommended for controlling the symptoms all over the body. Treatment also depends on factors like age and sex of the patient.
Application of topical medicines like gels, ointments and skin creams to skin sores or genital sores helps in reducing pain and inflammation. Generally, these medicines contain a corticosteroid drug which helps as an anesthetic to relieve pain and reduce inflammation.
- Mouth Rinses: Mouthwashes containing corticosteroids help in reducing the painful mouth sores by easing away discomfort.
- Eye Drops: In the case of mild inflammation, eye drops with anti-inflammatory medicines or corticosteroids help in relieving redness and pain in the eyes.
- The treatment of Behcet's syndrome in severe cases usually concentrates on controlling the damage caused due to the disorder during flares.
- Corticosteroids to Control Inflammation: Combination of corticosteroids like prednisone with other medications helps in reducing the inflammation. Only usage of corticosteroids may result in recurrence of the syndrome. Therefore, these are usually prescribed with the combination of other medicines suppressing the activities of immune system. Corticosteroids may cause side effects like high blood pressure, bone thinning, weight gain and persistent heartburn.
- Medications to Suppress Immune System: Immunosuppressive drugs help in suppressing the immune system in order to stop the immune system from attacking healthy tissues of the body. Immunosuppressive drugs helps in reducing the inflammation caused by the immune system. Immunosuppressive drugs may include cyclosporine, cyclophosphamide and azathioprine. Immunosuppressive drugs may include side effects like low blood counts, liver problems, high blood pressure, kidney problems and increased risk of infection.
- Medication to Regulate Immune System: Interferon alfa-2b (Intron A) is usually used for regulating the activities of immune system in order to control inflammation. This also helps in controlling eye inflammation, skin sores and joint pains in patients suffering from Behcet's syndrome. Interferon alfa-2b (Intron A) may include side effects like fatigue and muscle pain. Interferon alfa-2b may also be used with the combination of other medications. Other medications may include methotrexate and colchicine.
- Infliximab and Etanercept may also be helpful in treatment according to a recent study.
Investigations of Behcet's Disease or Behcet's Syndrome
- There is no particular test available for diagnosing Behcet's syndrome. A thorough subjective and physical examination is performed to diagnose Behcet's disease.
- Skin sores, genital sores, mouth sores and eye problems are usually diagnosed for confirming diagnosis of Behcet's syndrome.
- Blood tests and other laboratory tests may be conducted in order to rule out other diseases and conditions.
- Other tests may include pathergy test which is done by inserting sterile needle into the skin. A small red bump under the skin helps indicate overreaction of the immune system.