Pronounced: Buh-settes
En Espaol (Spanish Version)Definition
Behcets disease is a rare, chronic disorder involving inflammation of blood vessels throughout the body. It is marked by recurrent oral and genital ulcers and eye inflammation.
Causes
The cause of Behcets disease is unknown. It is believed to be caused by an autoimmune reaction. This occurs when the bodys immune system attacks its own tissue by mistake. This autoimmune abnormality may be inherited. An environmental trigger, such as a specific virus or bacterium, may activate the disease in people susceptible to it.
Risk Factors
A risk factor is something that increases your chances of getting a disease or condition. Although the exact cause of Behcets disease is unknown, some groups of people are more likely to develop the condition than others. Risk factors include:
- Location: the Middle East, Asia, and Japan
- Sex:
- In the US, men are more likely than women to develop this condition.
- In the Middle East, Asia, and Japan, women are more likely than men to develop Behcets.
- Age: 20s and 30s
Symptoms
Symptoms of Behcets disease can vary from mild to very severe. Symptoms tend to appear, heal, and then recur (referred to as a flare) frequently over months or years. The most common symptoms of the disease are:
Oral sores Affect almost all people with the diseaseUsually are the first to appearUsually last for 10 to 14 daysCan be painfulCan cause scarring Ulcerated Gums 2008 Nucleus Medical Art, Inc. Genital sores In men: appear on the penis and/or scrotumIn women: appear on the vulva or within the vaginaCan be painfulCan cause scarring Uveitisinflammation of the middle part of the eye, including the iris Tends to cause eye redness, blurred vision, sensitivity to light, and watering eyesIf not treated, can cause partial vision loss or blindnessArthritis inflammation of joints Tends to be painfulUsually does not cause permanent joint damage Skin problems, such as sores May appear as red, raised sores or bumps, or may be flat (flush with the skin)Usually appear on the legs and upper torso Other symptoms of the disease may include: Severe fatigue during a flare Blood clots Caused by thrombophlebitis (inflammation of veins), usually in the legs Can cause severe complications if not treated quicklyAneurysms , or severely dilated blood vessels May rupture and cause severe consequences Heart problems, such as: Abnormal heart rhythmsMissed heartbeatsEarly heartbeats Inflammation of the heart muscle ( myocarditis ) Central nervous system problems For example, meningoencephalitis, which is inflammation of the brain and membrane that lines the brain May result in seizures , confusion, strokes , memory problems, headaches Stroke from a Cerebral Aneurysm 2008 Nucleus Medical Art, Inc.
Digestive problems Causes ulcers and inflammation of the digestive tract (rarely) Diagnosis Diagnosing Behcets disease is very difficult because: Symptoms are similar to those of many other diseases.Symptoms often appear very slowly, over months or years.There is no specific test to confirm the disease. A doctor may suspect Behcets disease if oral sores appear at least three times within a year, and at least two of the following recurring symptoms appear: Eye inflammationGenital soresSkin soresIf Behcets disease is suspected, your doctor will do tests to rule out other diseases with similar symptoms. Also, you may have a pathergy skin test. For this test, your skin is pricked with a small needle. If you have Behcets disease, a bump will develop at the site of the skin prick. However, this test is not conclusive. Many people with the disease do not have a reaction to the skin prick. TreatmentThere is no cure for Behcets disease. Treatment is aimed at limiting and preventing complications from symptoms. With proper treatment, the disease can usually be managed fairly well. Treatment includes medications, rest, and exercise. MedicationBoth topical and oral medications are used.
Topical medications include creams, lotions, and mouth rinses that contain corticosteroids (to cut down on inflammation) and/or anesthetics (to decrease pain). These are applied to sores to reduce inflammation and pain. Oral medications, include: Oral corticosteroidsto reduce inflammation and pain Immunosuppressive drugsto help control the overactive immune system. These may include: InterferonAzathioprineChlorambucilCyclosporineColchicineMethotrexateDapsoneInfliximabThalidomideMany of the drugs used to treat Behcets disease can cause severe side effects. If you are taking these medications your doctor must closely monitor you. Rest and Exercise General guidelines include: Rest when symptoms flare to help speed healing.Engage in moderate exercise when symptoms recede to help keep joints flexible and strong. PreventionThere are no guidelines for the prevention of Behcet's disease because the exact cause is unknown. RESOURCES: American Behcets Disease Associationhttp://www.behcets.com National Institute of Arthritis and Musculoskeletal and Skin Diseaseshttp://www.niams.nih.gov CANADIAN RESOURCES: BC Health Guidehttp://www.bchealthguide.org/ The Arthritis Societyhttp://www.arthritis.ca/custom%20home/default.asp?s=1
References: Abu El Asrar, ABoooud EB, ALbibhi H, Al-Arfaj A. Long-term safety and efficacy of infliximab therapy in refractory uveitis due to Behcet's disease. Int Ophthalmol . Sep 23, 2006. Cakir O, Eren N, Ulka R, Nazaroghi H. Bilateral subclavian arterial aneurysm and ruptured abdominal aorta pseudoaneurysm in Behcet's disease. Ann Vasc Surg. 2002,16:516-520 The Medical Letter. 1996;38:15. The Merck Manual of Diagnosis and Therapy . Simon & Schuster; 2001. National Organization of Rare Disorders, Inc. website. Available at: http://www.rarediseases.org/ . National Institute of Arthritis and Musculoskeletal and Skin Diseases website. Available at: http://www.niams.nih.gov . Okada AA. Behcet's disease: general concepts and recent advances. Curr Opin Ophthalmol . 2006;17:551-556. Last reviewed December 2007 by Jill Landis, MDPlease be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
CONTRIBUTE TO THIS STORY
