Behcet’s 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.It is an autoimmune response where the immune system turns on the body, causes inflammation of parts of the body. In particular, small blood vessels around the body become inflamed, a condition known as vasculitis.
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The cause of Behcet’s remains unknown, but it’s often preceded by a viral infection, for example, which may trigger the autoimmune process, causing the body to attack its own blood vessels and making them inflamed. Experts in the field of Behcet’s research agree the causes may be genetic.
Life for people with Behcet’s is made more difficult because of misunderstandings about the illness. With the appearance of ulcers on the mouth and genitals, it’s often incorrectly assumed the condition is infectious and sexually transmitted – it’s not either of these.
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The disease was first described in 1937 by Dr. Hulusi Behçet, a dermatologist in Turkey. Behçet’s disease is now recognized as a chronic condition that causes canker sores or ulcers in the mouth and on the genitals, and inflammation in parts of the eye. In some people, the disease also results in arthritis (swollen, painful, stiff joints), skin problems, and inflammation of the digestive tract, brain, and spinal cord.
Sign & Symptoms;
Integumentary system (Skin and mucosa):
Nearly all patients present with some form of painful oral mucocutaneous ulcerations in form of aphthous ulcers or non-scarring oral lesions. The oral lesions are similar to those found in inflammatory bowel disease and can be relapsing. Painful genital ulcerations usually develop on the vulva and the scrotum and cause scarring in 75% of the patients. Additionally, patients may present with erythema nodosum, cutaneous pustular vasculitis, and lesions similar to pyoderma gangrenosum.
Ocular system (eyes):…..
Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20% of the cases. Ocular involvement can be in form of posterior uveitis, anterior uveitis, or retinal vasculitis. Anterior uveitis presents with painful eyes, conjuctival redness, hypopyon, and decreased visual acuity, while posterior uveitis presents with painless decreased visual acuity and visual field floaters. A rare form of ocular (eye) involvement in this syndrome is retinal vasculitis which presents with painless decrease of vision with possibility of floaters or visual field defects.
Gastrointestinal tract (bowels)
GI manifestations include abdominal pain, nausea, diarrhea with or without blood and often involves the ileocecal valve.
Lung involvement is typically in form of hemoptysis, pleuritis, cough, fever, and in severe cases can be life threatening if the outlet pulmonary artery develops an aneurysm which ruptures causing severe vascular collapse and death from bleeding in the lungs.
Musculoskeletal system (muscle, joint)
Arthralgia is seen in up to half of patients, and is usually a non-erosive poly or oligoarthritis of primarily the large joints of the lower extremities.
Neurological involvements range from aseptic meningitis, to vascular thrombosis such as dural sinus thrombosis and or organic brain syndrome manifesting with confusion, seizures, and memory loss. They oftern appear late in the progression of the disease but are associated with a poor prognosis.
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.
There is no specific pathological testing or technique available for the diagnosis of the disease, although the International Study Group criteria for the disease are highly sensitive and specific, involving clinical criteria and a pathergy test. Behçet’s disease has a high degree of resemblance to diseases that cause mucocutaneous lesions such as Herpes simplex labialis, and therefore clinical suspicion should be maintained until all the common causes of oral lesions are ruled out from the differential diagnosis.
International Study Group diagnostic guidelines:
According to the International Study Group guidelines, for a patient to be diagnosed with Behçet’s disease, the patient must have oral (aphthous) ulcers (any shape, size or number at least 3 times in any 12 months period)along with 2 out of the next 4 “hallmark” symptoms:
*genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men)
*skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in post-adolescents not on corticosteroids)
*eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous)
*pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick). The pathery test has a specificity of 95% to 100%, but the results are often negative in American and European patients
Despite the inclusive criteria set forth by the International Study Group, there are cases where not all the criteria can be met and therefore a diagnosis can not readily be made. There is however a set of clinical findings that a physician can rely upon in making a tenative diagnosis of the disease; essentially Behçet’s disease does not always follow the International Study Group guidelines and so a high degree of suspicion for a patient who presents having any number of the following findings, is necessary:
*nervous system symptoms
*stomach and/or bowel inflammation
*deep vein thrombosis
*cardio-vascular problems of an inflammatory origin
*inflammatory problems in chest and lungs
*problems with hearing and/or balance
*changes of personality, psychoses
*any other members of the family with a diagnosis of Behçet disease.
The etiology is not well-defined, but it is primarily characterized by auto-inflammation of the blood vessels. Although sometimes erroneously referred to as a “diagnosis of exclusion,” the diagnosis can sometimes be reached by pathologic examination of the affected areas.
The primary mechanism of the damage is an overactive immune system that seems to target the patient’s own body. The primary cause is not well known. In fact, as of now, no one knows why the immune system starts to behave this way in Behçet’s disease. There does however seem to be a genetic component involved as first degree relatives of the affected patients are often affected in more than expected proportion for the general population.
There’s no cure for Behcet’s yet, but research continues and treatment is available to keep inflammation and symptoms at bay.
Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. High dose Corticosteroid therapy (1 mg/kg/d oral prednisone) is indicated for severe disease manifestations. Anti-TNF therapy such as infliximab has shown promise in treating the uveitis associated with the disease. Another Anti-TNF agent, Etanercept, may be useful in patients with mainly skin and mucosal symptoms.
Interferon alfa-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers as well as ocular lesions. Azathioprine, when used in combination with interferon alfa-2b also shows promise, and Colchicine can be useful for treating some genital ulcers, erythema nodosum, and arthritis.
Thalidomide has also been used due to its immune-modifying effect. Dapsone and rebamipide have been shown, in small studies, to have beneficial results for mucocutaneous lesions.
Rest and Exercise:
Although rest is important during flares, doctors usually recommend moderate exercise, such as swimming or walking, when the symptoms have improved or disappeared. Exercise can help people with Behçet’s disease keep their joints strong and flexible.
HLA-B51 is strongly associated with Behçet’s disease Behçet disease is considered more prevalent in the areas surrounding the old silk trading routes in the Middle East and in Central Asia. Thus, it is sometimes known as Silk Road Disease. However, this disease is not restricted to people from these regions. A large number of serological studies show a linkage between the disease and HLA-B51. HLA-B51 is more frequently found from the Middle East to South Eastern Siberia, but the incidence of B51 in some studies was 3 fold higher than the normal population. However, B51 tends not to be found in disease when a certain SUMO4 gene variant is involved, and symptoms appear to be milder when HLA-B27 is present. At the current time, a similar infectious origin has not yet been confirmed that leads to Behçet’s disease, but certain strains of Streptococcus sanguinis has been found to have a homologous antigenicity.
Most people with Behçet’s disease can lead productive lives and control symptoms with proper medicine, rest, and exercise. Doctors can use many medicines to relieve pain, treat symptoms, and prevent 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.
A risk factor is something that increases your chances of getting a disease or condition. Although the exact cause of Behcet’s 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
*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 Behcet’s.
*Age: 20s and 30s
Researchers are exploring possible genetic, bacterial, and viral causes of Behçet’s disease as well as improved drug treatment. For example, genetic studies show strong association of the gene HLA-B51 with the disease, but the exact role of this gene in the development of Behçet’s is uncertain. Researchers hope to identify genes that increase a person’s risk for developing Behçet’s disease. Studies of these genes and how they work may provide new understanding of the disease and possibly new treatments.
Researchers are also investigating factors in the environment, such as bacteria or viruses, that may trigger Behçet’s disease. They are particularly interested in whether Streptococcus, the bacterium that causes strep throat, is associated with Behçet’s disease. Many people with Behçet’s disease have had several strep infections. In addition, researchers suspect that herpesvirus type 1, a virus that causes cold sores, may be associated with Behçet’s disease.
Finally, researchers are identifying other medicines to better treat Behçet’s disease. TNF inhibitors are a class of drugs that reduce joint inflammation by blocking the action of a substance called tumor necrosis factor (TNF). Although serious side effects have been reported for TNF inhibitors, they have shown some promise in treating Behçet’s disease. Examples of TNF inhibitors include etanercept and infliximab. TNF inhibitors belong to a family of drugs called biologics, which target the immune response. Also, interferon alpha, a protein that helps fight infection, has shown promise in treating Behçet’s disease. Thalidomide, which is believed to be a TNF inhibitor, appears effective in treating severe mouth sores, but its use is experimental and side effects are a concern. Thalidomide is not used to treat women of childbearing age because it causes severe birth defects.
Where Can People Get More Information About Behçet’s Disease?
•National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
National Institutes of Health
1 AMS Circle
Bethesda, MD 20892-3675
Toll Free: 877-22-NIAMS (226-4267)
•National Institute of Dental and Craniofacial Research (NIDCR)
National Institutes of Health
45 Center Drive, MSC 6400
Building 45, Room 4AS-25
Bethesda, MD 20892-2510
•National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
National Institutes of Health
1 Information Way
Bethesda, MD 20892-3560
Toll Free: 800-860-8747
•National Eye Institute (NEI)
National Institutes of Health
31 Center Drive MSC 2510
Bethesda, MD 20892-2510
•National Institute of Neurological Disorders and Stroke (NINDS)
NIH Neurological Institute
P.O. Box 5801
Bethesda, MD 20824
Toll Free: 800–352–9424
•American Academy of Dermatology (AAD)
P.O. Box 4014
Schaumberg, IL 60618-4014
Toll Free: 866-503-SKIN (7546)
•American College of Rheumatology (ACR)
2200 Lake Boulevard NE
Atlanta, GA 30319
1560 Sherman Avenue, Suite 870
Evanston, IL 60201-4808
(This organization is “research only.” Contact should be made by U.S. mail or e-mail.)
•American Behçet’s Disease Association
P.O. Box 869
Smithtown, NY 11787-0869
Toll Free: 800-7-BEHCET (723-4238)
•American Skin Association (ASA)
346 Park Avenue S., 4th floor
New York, NY 10010
Toll Free: 800-499-SKIN
P.O. Box 7669
Atlanta, GA 30357-0669
Toll Free: 800-283-7800
•National Organization For Rare Disorders (NORD)
55 Kenosia Avenue, P.O. Box 1968
Danbury, CT 06813-1968
Toll Free: 800-999-6673
Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.