Behçet’s disease can create issues in your mouth and genital area, in the form of recurrent sores. This condition is mostly associated with inflammation of both the blood vessels and the eyes, though it often causes a lot of different issues throughout the body.
Behçet's disease, also known as Silk Road disease, is a chronic (long-term) inflammatory condition, caused by vasculitis (blood vessel inflammation), which will affect and damage both your arteries and veins.
Behçet's disease is seen all over the world. But it’s most common in Northern Turkey (up to 420 cases per 100,000 people), the Mediterranean basin and Middle East (up to 300 cases per 100,000 people), and the Far East (about 15 cases per 100,000 people). It’s not as common in the United States (about 7 cases per 100,000 people). Anyone can develop this disease at any age, though symptoms usually begin to appear between the ages of 20 and 30. Men and women are equally affected.
The main symptoms include:
The brain, nerves, lungs (rare), intestinal tract and kidneys can also be affected.
This disease affects everybody differently. Some of the most commonly affected places it strikes and key features include:
Scientists are still not exactly sure what causes this disease. It may be an autoimmune disorder, meaning the body’s immune system mistakenly attacks healthy tissue with an inflammatory response. Both HLA-B5 and HLA-B51 are gene markers that are sometimes present in patients with Behçet’s disease. But there are some people who have this gene marker who don’t have Behçet’s disease. Researchers are studying other genes related to immune function and think that infections (either bacteria or a virus) may play a role in triggering the disease in some people who have genetic markers that predispose them to Behçet’s.
Genetic and environmental factors are both likely factors in the development of Behçet’s disease.
There are a few different groups of people that are more likely to develop this disease than others, including:
There is no single laboratory test that can diagnose Behçet’s disease. The diagnosis is usually made based on your symptoms, including how often (typically at least three times a year) oral ulcers come back. Plus, at least two more of the following:
To make the correct diagnosis, other illnesses that produce mouth sores and closely resemble Behçet's disease need to be ruled out. Your doctor may order a blood test, which can help rule out some of these other conditions, including systemic lupus, Crohn's disease (an intestinal inflammatory condition) and other forms of vasculitis.
There’s no clear evidence to suggest Behçet’s disease is an inherited disease. Most cases develop randomly without any family link. While it’s been reported that a small percentage of cases occur in the same family, but there’s no clear inheritance pattern.
While there is no known cure right now for this disease, there are some different drugs that can be used to help manage symptoms:
Depending on the severity of your condition, your doctor may also suggest using certain eye drops, mouthwashes and skin ointments to control this disease and its symptoms, before deciding to use medication.
Behçet’s is a chronic (long-term) disease that can disappear and reappear, no matter the treatment. This can make it difficult to tell how well treatment is working. Most patients with Behçet’s will likely deal with symptoms, on and off, for their entire lives. But, most are able to live a full life.
Both rest and activity are helpful coping mechanisms. A consistent exercise regimen will make your body feel better, thus easing the pain on your joints and other problem areas. Alternatively, taking some time to rest when you begin to feel harsh pain will also improve your overall mood and lessen the effect of some of the symptoms. Another way to cope is through shared experience and community, which means finding others who also have this condition. This can sometimes be difficult, due to the rarity of this disease, but your doctor can help you find others who may be having similar experiences.
Death occurs in about 5% of patients. Causes of death are most often intestinal perforation (hole), strokes and a rupture of enlarged, weakened blood vessels (aneurysms).
While Behçet's disease itself is not exactly fatal, it can lead to a number of health and medical problems all over the body, most of which will cause pain. But successfully managing and living with this disease is very possible, especially if you can find ways to constantly exercise, rest and talk about your experiences with others who are also suffering from this rare disease.
Last reviewed by a Cleveland Clinic medical professional on 06/02/2020.
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