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Behcet's Disease

What is Behcet's Disease?

Behçet’s disease is an autoimmune inflammatory condition affecting many parts of the body, most commonly including the mouth, skin, eyes, joints and genitals. Health problems associated with Behçet’s disease are caused by widespread inflammation of blood vessels (vasculitis). In some people, the disease also results in arthritis, skin problems, and inflammation of the digestive tract, brain, and spinal cord. This syndrome can be fatal due to ruptured vascular aneurysms (from enlargement and weakening of artery walls) or severe neurological complications.

 

Synonyms

  • Behcet disease
  • Behcet´s disease
  • Behcet´s syndrome
  • Behcet syndrome

Behçet’s disease is an autoimmune inflammatory condition affecting many parts of the body, most commonly including the mouth, skin, eyes, joints and genitals. Health problems associated with Behçet’s disease are caused by widespread inflammation of blood vessels (vasculitis). In some people, the disease also results in arthritis, skin problems, and inflammation of the digestive tract, brain, and spinal cord. This syndrome can be fatal due to ruptured vascular aneurysms (from enlargement and weakening of artery walls) or severe neurological complications.

Acknowledgement of Behcet's Disease has not been added yet.

Behçet’s disease is a rare disorder occurring more frequently in the Middle East and Asia, along the Silk Road. Turkey has the highest prevalence rate (80 to 370 cases per 100,000). Japan, Korea, China, Iran, and Saudi Arabia also have high prevalence rates. In the United States and Australia, the syndrome is more common in women than men, and the symptoms tend to be less severe. Men may be more affected in Middle Eastern countries, and usually have more severe symptoms. Involvement of the central nervous system is more common among native populations of northern Europe and the United States. In northern European countries and the United States, this disease generally affects fewer than 1 in 100,000 people.

Name Abbreviation
Behcet disease BD
Behcet´s disease BD
Behcet´s syndrome BD
Behcet syndrome BD

The cause of Behçet’s disease is unknown. The condition probably results from a combination of genetic and environmental factors, most of which have not been identified. However, a particular variation in the HLA-B gene has been strongly associated with the risk of developing Behçet’s disease. This gene provides instructions for making a protein that plays an important role in the immune system. It has many different normal variations, allowing each person’s immune system to react to a wide range of foreign proteins. A variation called HLA-B51 increases the risk of developing Behçet’s disease. Although many people with Behçet’s disease have the HLA-B51 variation, most people with this version of the HLA-B gene never develop the disorder. It is unknown how HLA-B51 increases the risk of developing this disease. It appears likely that environmental factors, such as certain bacterial or viral infections, play a role in triggering the disease in people who are at risk. Most cases of Behçet’s disease are sporadic; they occur in people with no history of the disorder in their family. This condition does not have a clear pattern of inheritance although a small percentage of all cases have been reported to run in families.

The first signs of Behçet’s disease include painful mouth sores called aphthous ulcers. These occur on the lips, on the tongue, and inside the cheeks. They look like common canker sores and typically heal within one to two weeks. About 75% of people with Behçet’s disease develop similar ulcers on the genitals.

Behçet’s disease can also cause painful bumps and sores on the skin. Most affected individuals develop pus-filled bumps that resemble acne that can occur anywhere. Some also have red, tender nodules called erythema nodosum. These usually develop on the legs, but also occur on the face, neck, and arms.

In more than half of people with Behçet’s disease, there is an inflammation of the eye called uveitis. This is more common in younger people and men and results in blurry vision and an extreme sensitivity to light (photophobia). If untreated, this symptom can lead to blindness.

Less commonly, Behçet’s disease can affect the joints, gastrointestinal tract, large blood vessels, and the central nervous system (brain and spinal cord). Central nervous system abnormalities are among the most serious complications.

The symptoms usually begin from ages twenty to forty, although it can appear at any age. Some affected people have relatively mild symptoms that are limited to sores in the mouth and on the genitals. Others have more severe symptoms affecting many parts of the body, including the central nervous system. The features of Behçet’s disease typically come and go over a period of months or years. In most affected individuals, the health problems associated with this disorder improve with age.

Name Description
Mouth ulcers Mouth ulcers
Arthritis Arthritis
Stomach or bowel inflammation Stomach or bowel inflammation
Inflammatory problems Inflammatory problems usually occuring in the chest and lungs
Extreme exhaustion Extreme exhaustion
Personality changes Personality changes
Loss of balance Loss of balance
Hearing problems Hearing problems

The diagnosis of Behçet’s syndrome is made based on the clinical judgment of a physician. According to the International Study Group guidelines, for a patient to be diagnosed with Behçet's disease, he/she must have oral ulcers (any shape, size, or number at least 3 times in any 12 months period) along with 2 out of the 4 following symptoms: genital ulcers, skin lesions, eye inflammation, pathergy reaction. However, there are cases where not all the criteria can be met and therefore a diagnosis cannot readily be made.

Diagnostic tests include a pathergy test. During a pathergy test, a physician pricks an individual with a sterile needle. A positive outcome occurs if a reddish spot (nodule or pustule) forms 48 hours after the prick.

There is no cure for Behçet's disease, but people can usually control symptoms with proper medication, rest, exercise, and a healthy lifestyle. The goal of treatment is to reduce discomfort and prevent serious complications such as disability from arthritis or blindness. The type of medicine and length of treatment depend on the person’s symptoms and their severity. A combination of treatments will likely be needed to relieve specific symptoms. Doctors may prescribe rinses, gels, or ointments. The medicine usually contains corticosteroids (which reduce inflammation), other anti-inflammatory drugs, or an anesthetic. Other drugs that may be prescribed in severe cases include immunosuppressive drugs (Azathioprine, Chlorambucil or Cyclophosphamide, Cyclosporine, Colchicine, or a combination of these treatments) and Methotrexate.

In the absence of treatment, there is potential for a severe prognosis causing blindness. There is also the risk of lethal arterial rupture and neurological symptoms potentially causing brain related disorders that may lead to a loss of autonomy. Intensive ophthalmological (eye) care coupled with immunosuppressive treatment has been shown to reduce morbidity greatly.

Exercise can improve the conditions of joints. Exercise such as yoga or Pilates may also help to reduce stress, which can sometimes trigger a flare-up of symptoms. A balanced diet is beneficial to general health.

Behçet’s disease is not contagious. However, long-term conditions can affect the mood, emotions, and confidence of the individual. It can also have an impact on work, social life, and relationships. Talking with a friend, relative, or doctor could help. In addition, contacting support groups is a good way to meet others with Behçet’s disease.

Newbie... Created by Imannie
Last updated 8 Feb 2012, 07:33 AM

Posted by Courtnee22
8 Feb 2012, 07:33 AM

Hi! I was diagnosed about a year and a half ago but I have had symptoms since I was 10 years old and I am now 23, turning 24 this year. It was actually a relief to finally get a diagnosis because it was hard having flare ups all of the time with no answers and confused doctors. What kinds of symptoms do you have? I have been on three different medications, Colchicine and Imuran, in which I had some very unpleasant side effects, I was throwing up all the time and had horrible nausea and abdominal pain, so I have stopped taking those, I am now taking plaquenil and it's so far so good. What kinds of questions do you have? I am happy to offer any information that I have gathered

Posted by Imannie
8 Feb 2012, 12:36 AM

Hi, I am newly diagnosed with Behcet's...It is all new to me so I am actively seeking info, etc. I hope to be in touch with others going through this disease. Thanks, ~Iamannie

video Created by stopcaidnow
Last updated 3 Mar 2011, 04:22 AM

Posted by stopcaidnow
3 Mar 2011, 04:22 AM

If anyone is interested in sharing your child's photo or story please email me at StopCAIDnow@aol.com Mike Quick(5x pro bowler for the NFL) and I are producing a video for the Foundations homepage. We welcome your VOICE and pictures to be added to the video. We will also use the pictures or videos at the Gala. The Gala will be covered by the media, so if you do not want the exposure please do not send your picture. We will not list any names or personal information on either the video for the homepage or the Gala. If you send in a video of yourself talking and choose to say who you are and what your child has, that will be the only way we will share your personal information. We want to share your story, your pictures the way you want it to be shared with the world. Thank you, StopCAIDnow,Inc.

Community External News Link
Title Date Link
Community Resources
Title Description Date Link
American Behcet's Foundation

An online resource for Behçet's sufferers, caregivers, and loved-ones.

03/20/2017

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After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.

Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.

Visit sanfordresearch.org/CoRDS to enroll.

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Created by Imannie | Last updated 8 Feb 2012, 07:33 AM

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