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Behcet's Syndrome: What It Is and How It Can Be Treated Nancy Rosenberg, Medical Writer Published Publishing Information Published by CBSHealthWatch.com Behcet's Syndrome: What It Is and How It Can Be Treated Susan Stewart, an accountant who lives in Springfield, Pennsylvania, was 36 when she began having what she describes as "bizarre" symptoms: headaches, lethargy, and, most disturbing of all, painful oral ulcers that made eating and drinking difficult. "I felt like I had the flu all the time," says Stewart. "I suffered with symptoms on and off for three years. Finally, at the advice of my family doctor, I made an appointment to see a rheumatologist, who diagnosed Behcet's in five minutes." Understanding Behcet's Behcet's Syndrome is named after Hulusi Behcet (pronounced Beh SHETT or Beh SHAY), a Turkish physician who first identified the disease in 1937. While the cause of Behcet's Syndrome is not known, researchers suspect a genetic predisposition combined with viral exposure. In the United States, Behcet's affects approximately 20,000, usually women who are in their 20s or 30s. Because the syndrome is so difficult to diagnose, the actual number of those affected may be considerably higher. People with "Silk Route" bloodlines, or those from the Mediterranean Basin, Middle East, or Far East, are most likely to develop Behcet's. In Silk Route countries, however, the disease predominantly afflicts men, and in those countries Behcet's is a leading cause of blindness. "Behcet's is a clinical syndrome, but there are no definitive tests to determine whether or not a patient actually has the disease," says Raphael J. DeHoratius, M.D., a rheumatologist at Thomas Jefferson University Hospital in Philadelphia. "This can make Behcet's particularly difficult to diagnose." A Multi-Symptom Disorder "Symptoms often develop over time," says Dr. DeHoratius. "For example, oral ulcers may appear, then, years later, arthritis develops, then the symptoms may go into spontaneous remission for a period of four or five years. Many patients go from doctor to doctor, depending on the symptom they are experiencing. At some point a doctor puts all the pieces of the puzzle together, and a diagnosis of Behcet's Syndrome can be made." This is a multi-symptom disorder, says Dr. DeHoratius. It is an autoimmune disease, connective tissue disease, vascular disease, arthritic disease. It affects the skin, eyes, blood vessels, and joints. "Basically, Behcet's is the result of a hyperactive immune system," says Dr. DeHoratius. It causes blood vessels throughout the body become inflamed, and the central nervous system, heart, lungs, and intestinal tract can all be affected. "Behcet's is a form of generalized occlusive vasculitis," says Phillip Calenda, M.D., a Board-certified ophthamologist in Westchester County, New York. "It leads to a condition known as ischemia, which basically means that blood flow in the body is restricted, which leads to the death of tissue, which leads to ulcerations and a whole host of other symptoms." Symptoms of Behcet's The most frequently reported symptoms of Behcet's include mouth ulcers--sometimes in crops of up to 100, genital ulcers, skin bumps that resemble acne or boils, and vision difficulties, which can be among the most serious complications of the syndrome. Other symptoms of Behcet's include joint pain and swelling, bowel inflammation, fever, a stiff neck, headaches, and phlebitis, or a swelling of veins. In addition, as with any chronic illness, fatigue can be severe and debilitating, notes Dr. DeHoratius. Behcet's is usually not terminal, though it is a chronic, cyclical disorder. Symptom flares can last days, weeks, or months, but they are usually followed by periods of remission. Behcet's may be difficult to distinguish from its other, better-known cousins. Crohn's disease, which also causes bowel inflammation, rarely affects the eyes. Behcet's may be mistaken for rheumatoid arthritis, but the rheumatoid factor is not present in the blood. Lupus, another autoimmune disorder, shares many of the same symptoms as Behcet's but usually does not cause recurring ulcers. In most cases the presence of recurring oral ulcers is the defining symptom of Behcet's, according to the American Behcet's Disease Association. How Behcet's Affects Vision "There are several ways Behcet's can affect the eyes," says Dr. Calenda. "Typically, Behcet's causes uveitis, or an inflammation of a portion of the eye. "Behcet's stimulates the production of white blood cells, which circulate through the liquid in the eye and can even settle in the eye. The inflammation can clog the flow of fluid and can raise pressure in the eye. In the back part of the eye the inflammation can affect the macula, which can severely distort vision. "Behcet's can affect the blood vessels in the eye as well. If it's severe enough it can knock out blood flow to the optic nerve, which can lead to total blindness or a severe decrease in vision. A few years after developing Behcet's many people lose either part or all of their eyesight," says Dr. Calenda. Living With Behcet's Susan Stewart has now been "medically controlled" for five years. "I have minor arthritis-type problems, and I occasionally get a mild ulcer that usually fizzles out. I've learned to pay attention to my body. For example, I know that certain foods make my colitis flare, so I avoid them. I've also learned to limit my physical exertion. If I push myself too far, I know I'll pay for it with recurring symptoms, so I've learned to pace myself." Now Stewart sees her family doctor regularly and sees a rheumatologist and ophthamologist once a year. Stewart also credits the American Behcet's Disease Association hotline: "It has been a resource that I couldn't live without." "I'm one of the lucky ones," Stewart continues. "I work full time; I lead a normal life. The disease is manageable, and the medication has most definitely given me the ability to live life fully." Editor's note: The American Behcet's Disease Association can be contacted by calling 1-800-7Behcets, or visit their website at www.behcets.com. Box: Treating Behcet's "Treatment of Behcet's requires a multidisciplined approach," says Dr. Calenda. "A patient's rheumatologist, ophthamologist, and dermatologist may all confer on a regular basis, to make sure that treatment is aggressive enough but not so aggressive as to cause serious complications." The first line of treatment for Behcet's are usually corticosteroids such as prednisone, an immune suppressant, although that alone is usually not enough to control the disease, says Dr. DeHoratius. Because of severe side effects, prednisone should only be used intermittently. The second line of treatment may include cytotoxic drugs such as imuran, or an anti-gout medicine called colchicine. Anti-malarial drugs are sometimes used, and some patients are having success with thalidomide, although in women it should only be used on those who are past the age of childbearing, notes DeHoratius. "For some reason the body is attacking itself, so treatment focuses on trying to get the body to stop the attack," notes Dr. Calenda. "Other types of drugs, such as cyclophosphamide or cyclosporine, may be effective, but they are very powerful drugs and can have potentially serious side effects, such as toxicity to the kidneys and high blood pressure. "To quiet the disease down can sometimes take a year or more, so the goal is to use a course of treatment with the most mild side effects, because the patient will most likely be on the drugs for an extended period of time," says Dr. Calenda. |
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