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Behcet's Disease

What is Behcet's Disease?

Behcet’s Disease (or Behcet’s Syndrome) is a chronic (long term) form of vasculitis, which is an inflammation of the blood vessels. Blood vessel inflammation is caused by blood clots that form and obstruct the flow of blood throughout the body, leading to dangerous complications in the brain such as aneurysm and stroke. There is currently no known cause of Behcet’s Disease, but there are multiple symptoms that may appear together that may seem unrelated to blood vessel inflammation. People with Behcet’s Disease develop sores on their mouth, inflammation around the eyes, and skin rashes and genital sores. These symptoms may be chronic, meaning they never go away, or may flare up at different times. This makes Behcet’s Disease difficult to diagnose before inflammation of the blood vessels leads to complications; so many of the symptoms may seem unrelated at first. he first physician to describe this disease was Dr. Hulusi Behcet of Turkey in the 1930s, who diagnosed it as a triad of inflammations involving the mouth, genitals, and eyes. 

 

Synonyms

  • Behcet disease
  • Behcet´s disease
  • Behcet´s syndrome
  • Behcet syndrome
  • Silk Road Disease

Behcet’s Disease (or Behcet’s Syndrome) is a chronic (long term) form of vasculitis, which is an inflammation of the blood vessels. Blood vessel inflammation is caused by blood clots that form and obstruct the flow of blood throughout the body, leading to dangerous complications in the brain such as aneurysm and stroke. There is currently no known cause of Behcet’s Disease, but there are multiple symptoms that may appear together that may seem unrelated to blood vessel inflammation. People with Behcet’s Disease develop sores on their mouth, inflammation around the eyes, and skin rashes and genital sores. These symptoms may be chronic, meaning they never go away, or may flare up at different times. This makes Behcet’s Disease difficult to diagnose before inflammation of the blood vessels leads to complications; so many of the symptoms may seem unrelated at first. he first physician to describe this disease was Dr. Hulusi Behcet of Turkey in the 1930s, who diagnosed it as a triad of inflammations involving the mouth, genitals, and eyes. 

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

The synonym “Silk Road Disease” for Behcet’s Disease originated from this disease’s prevalence along the region referred to as the “Old Silk Road”. This refers to a trade route from East Asia such as Japan and China to the Mediterranean, spanning countries such as Turkey and the Middle East.

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
Silk Road Disease

The direct cause of Behcet’s Disease is still unknown, but it is believed that there are a number of factors including genetic, immune, and environmental that combine to cause the inflammation and resulting skin sores. 

  • Genetic factors: There is no known gene that is responsible for Behcet’s Disease. However, there is a gene that produces a particular protein known as an antigen that is found in inflammatory tissues of people with Behcet’s Disease. This gene, known as HLA-B51, encodes the antigen B51, which can be found in inflamed tissues such as the eyes and skin. This antigen B51 can sometimes be used as a genetic marker of Behcet’s Disease, meaning that testing for high levels of it in the blood can indicate a diagnosis. However, it is unknown whether this gene and antigen cause the disease in any way or if they are just part of the inflammatory response.. 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. 

  • Autoinflammatory factors: Due to the symptoms of inflammation experienced by those with Behcet’s Disease, one of the causes could be autoinflammation. An autoinflammatory condition occurs when the body triggers inflammation when there is no challenge to the immune system, or when a bacterial or viral infection causes an immune response to target a certain part of the body. This is a defect that causes the body to activate an immune response without a way of shutting it down. 

  • Environmental: Environmental factors, such as food, drugs, or exposure to toxins, can impact the way genes are expressed in the body. A person’s DNA cannot be changed by such factors, but the way they are read and translated into proteins that have important functions in the body can change. If DNA is the instructions, gene expression carried out by transcription factors is the ability to interpret instructions. External factors can turn these transcription factors on or off, and change sites on the DNA that these transcription factors recognize to start reading certain genes.

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.

While there is no definitive test to diagnose Behcet’s Disease, several tests can be employed to evaluate the inflammation to blood vessels and the impacts to the brain and spinal cord:

  • Blood test

  • Urine test

  • Scans: x-ray, CT, MRI

  • Skin biopsy: removal of a small amount of tissue to evaluate the health of the cells

  • Pathergy test: skin prick with a needle to evaluate development of a sore; individuals with Behcet’s Disease often have hypersensitive skin

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.

There are 3 sites of NHS Excellence devoted to the study and diagnosis of Behcet’s Disease. They are all located in England: London, Birmingham, and Liverpool. More information can be found on their website: https://www.behcets.nhs.uk/ 

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
The Rare World of Rare Diseases 12/17/2022
Mental health: Mum with rare disease says support is lacking 02/26/2023
Soligenix Receives FDA IND Clearance for Phase 2 Clinical Trial of Dusquetide in the Treatment of Aphthous Ulcers in Behçet's Disease 12/03/2023
Community Resources
Title Description Date Link
American Behcet's Foundation

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

03/20/2017

Clinical Trials


Cords registry

CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.

Enrolling is easy.

  1. Complete the screening form.
  2. Review the informed consent.
  3. Answer the permission and data sharing questions.

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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I'm here because I have Bechets Disease.

I am the mother of the patient. Due to neurological changes she is unable to manage any aspect of her life. Doctors say previous brain lesions caused brain atrophy and brain damage that may not...

I am a person with Behcet's Syndrome/Disease. Plenty to share. Love to read stories of others with rare, auto inflammatory and autoimmune diseases. It's time for a cure.
retired high school teacher, two kids, (girl 31, boy 29) have had BD for decades but it was in remission for 20 years or so.

 

BD has screwed my life up big time, right now I'm emerging from a...
I am the President and Founder of StopCAIDnow,Inc. (www.stopcaidnow.com), in process of becoming a 501 (3) non-profit dedicated to educate, awareness diverse genetic testing, genomics and...
I have a rare disease which may be Henoch Schoenlein Purpura or Behcet's Disease. It has plagued me for more than 2 years now and is gradually eroding my health.

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Newbie...

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

video

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


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