Behçet's disease (patient information)

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 * Associate Editors-In-Chief: Jinhui Wu, M.D.; Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]

Overview
Behçet’s disease is rare in America but relatively common in Middle East and Japan. It is a chronic, relapsing-remitting vasculitis affecting multiple organ systems in the body. The cause is not clear and researches demonstrate that the presence of the gene HLA–B51 is a risk factor for Behçet's disease. Many patients show multiple organs damages. Usual signs and symptoms include painful mouth sores, skin lesions, uveitis, abdominal pain, diarrhea, headache, fever, disorientation, stroke, or joint swelling and joint pain. There is no specific test for the diagnosis of Behçet's disease. Miltiple organs damages and images excluding other diseases may help diagnose Behçet's disease. Treatment of Behcet's disease depends on the severity and the location of the symotoms in an individual patient. It includes medications and surgery. The disease usually runs a protracted course and the mortality is low.

What are the symptoms of Behcet's disease?
Signs and symptoms of Behcet's disease vary from person to person, depending on the blood vessels affected. Many patients show multiple organ damages. Like other autoimmue diseases, signs and symptoms come and go.
 * Painful mouth sores: These are the most common sign of Behcet's disease. Mouth sores turn quickly from lesion to ulcers and heal in 7~21 days.
 * Skin lesions: Skin lesions in patients with Behçet's disease vary, from acne-like sores to red tender nodules.
 * Brain: When Behcet's disease affect brain, it leads to headache, fever, disorientation, poor balance, even stroke.
 * Digestive system: Patients with Behcet's disease may show symptoms such as abdominal pain, diarrhea or bleeding.
 * Eyes: Behcet's disease may cause inflammation in the eye, such as uveitis.
 * Joints: Patients with Behçet's disease may have joint swelling and joint pain when joints are affected with inflammination.
 * Vascular system: Inflammination may occur on veins and large arteries, leading redness, pain and swelling in the arms or legs, even aneurysms and narrowing or blockage of the vessel.
 * Genitals: Patients with Behcet's disease may develop sores on their genitals, feeling pain and leaving scars.

Diseases with similar symptoms
 * Erythema nodosum
 * Inflammatory bowel disease
 * Peptic ulcer
 * Acute febrile neutrophilic
 * Lupus erythematosus

What causes Behcet's disease?
The exact cause of Behçet's disease is unknown. Most symptoms of the disease are caused by 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 autoinflammatory 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, such as germs, that enter the body. In an autoimmune reaction, the immune system mistakenly attacks and harms the body's own tissues.

Behçet's disease is not contagious; it is not spread from one person to another. Researchers think that two factors are important for a person to get Behçet's disease. First, it is believed that abnormalities of the immune system make some people susceptible to the disease. Scientists think that this susceptibility may be inherited; that is, it may be due to one or more specific genes. Second, something in the environment, possibly a bacterium or virus, might trigger or activate the disease in susceptible people.

Who is at highest risk?
The underlying cause of Behçet’s disease is not clear. It is suggested that the following factors may be associated with the disease.
 * Genetic predisposition: Researches demonstrate that the presence of the gene HLA–B51 is a risk factor for Behçet's disease.
 * Age: Behçet's disease can affect people in any age, but the most common age is 20~30 years old.
 * Gender: Behcet's disease most commonly affects men than women.
 * Race: Behçet's disease is more common in Middle East and Japan then in other race. It is rare in America.

When to seek urgent medical care?
Call your health care provider if symptoms of Behçet's disease develop. If one emerges the following symptoms, seeking urgent medical care as soon as possible:
 * Sever headache
 * Massive hemorrhage of gastrointestinal tract

Diagnosis
To know you have Behçet's disease the physician may take help from the following
 * Mdical history and physical examination
 * Pathergy test: During this procedure, the nurse pricks the skin of the forearm with a sterile needle. After 24~48 hours, a red nodule or pustule greater than two millimeters in diameter suggests positive and Behcet's syndrome.
 * Skin biopsy: Skin biopsy is used to diagnose a skin condition such as a rash and exclude skin cancer. During the procedure, the doctor takes a thin slice off the top of the skin and remove the lesions. Then, the pathologist can check the lesion under a microscope and identify the diagnosis.
 * Lumbar puncture: When your appear severe headache and head CT shows normal, the doctor may order this test to exclude other center nervous diseases of headache. During this procedure, the patient lies on the side, with knees pulled up toward the chest, and chin tucked downward. After injecting the anesthetic into the lower spine, the doctor insert a spinal needle into the lower back area to measure CSF pressure and collect a sample.
 * Brain MRI scan: A head MRI uses magnetic fields but it is a different type of image than what is produced by computed tomography (CT) and produces detailed images of the body. Like computed tomography (CT), a contrast agent may be injected into a patient’s vein to create a better picture. The doctor orders brain MRI to exclude other causes of headache of Behçet's disease.
 * Colonoscopy and biopsy: When the patient appear symptoms of digeative system, the doctor may order solonoscopy to exclude intestinal cancer. After you are sedated, a thin, flexible, lighted tube called endoscope is passed down your anus. Then the doctor can view the lining of your rectum and each section of your colon, up to ileocecal junction. If abnormal areas are noted, biopsies (tissue samples) can be obtained through the endoscope. The tissue samples will be checked by the pathologists under a microscope to see whether cancer is present or not.

Treatment options
Treatment of Behcet's disease depends on the severity and the location of the symotoms in an individual patient. Treatment opinion includes medications and surgery.
 * Medications
 * Colchicine: Colchicine can also be recommended to control inflammination and relieve pain, especially in patients with peptic uncler. Usual side effects of colchicine include nausea, vomiting and diarrhea.
 * Non-steroidal anti-inflammatory drugs (NSAIDs): NSAIDs may also be used to control inflammation and pain in patients with Behçet's disease. Usual drugs include ibuprofen, naproxen and indomethacin. Usual side effects are pain, bleeding even ulcers in upper gastrointestinal tract.
 * Corticosteroids: Patients with acute anterior uveitis may reponse well to corticosteroid eye drops. Systemic corticosteroids are helpful in treating acute signs and symptoms.
 * Immunosuppressants: Immunosuppressive drugs, such as chlorambucil, azathioprine, and cyclophosphamide, may be used to treat severe condition of the arteries, eyes, and brain of Behçet's disease. Cyclosporine, either alone or in combination with corticosteroids, is effective for the ocular lesions. Its side effect includes nephrotoxicity. Chlorambucil can also be used alone or in combination with corticosteroids.


 * Surgery: Surgery may help treat patients of Behçet's disease with severe complications, such as gastrointestinal perforation or ocular inflammatory diseases.

Where to find medical care for Behcet's disease?
Directions to Hospitals Treating Behçet's disease

What to expect (Outlook/Prognosis)?
Behçet's disease usually runs a protracted course and the mortality is low. Prognosis depends on:
 * Gender: Men with Behçet's disease has poorer prognosis than women.
 * Whether the patient appear complications.

Possible complications
When treatment is effective, flares usually become less frequent. Many patients eventually enter a period of remission (a disappearance of symptoms). In some people, treatment does not relieve symptoms, and gradually more serious symptoms such as eye disease may occur. Serious symptoms may appear months or years after the first signs of Behçet's disease.