Behçet’s disease, also known as Behçet’s syndrome, is a long-term auto-inflammatory disorder that affects the body’s blood vessels.
It is a relapsing and remitting condition, which means that sometimes there may be no symptoms, but, during a flare, symptoms worsen for a while.
Fast facts about Behçet’s disease
- Behçet’s disease is a rare condition that is most commonly seen in Turkey but occurs in other countries throughout the world.
- According to the American Behçet’s Disease Association, the prevalence of Behcet’s disease in Turkey is as high as 400 cases per 100,000 people.
- Behçet’s disease affects one person in every 170,000 in the United States.
- The condition is not contagious and cannot be spread from person to person
- Behçet’s disease affects everyone differently, and symptoms may intensify and weaken over a period of weeks or longer.
There is no cure for Behçet’s disease, but some therapies and lifestyle choices can help relieve symptoms.
The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) suggest rest and exercise to help reduce pain and prevent further complications.
Once a person has a diagnosis of Behçet’s disease, a team of health professionals team will make recommendations about treatment options.
The team may include an oral health specialist, a dermatologist, ophthalmologist and a rheumatologist, among others.
According to the United Kingdom’s National Health Service (NHS), medications include:
Corticosteroids: These reduce inflammation and may be used as a systemic treatment, affecting the whole body, or in topical applications, for example, to treat mouth sores.
Immunosuppressants: These reduce are systemic medications the excessive activity of the immune system, which underlies most of the symptoms of Behçet’s.
Biological therapies: This is a newer, systemic therapy. It targets some of the specific biological processes that are involved in causing symptoms. For example, tumour necrosis factor alpha inhibitors (TNFa-inhibitors) affect the antibodies that lead to inflammation.
This is applied to the surfaces of the body. It may include the use of pain-relieving therapy, including corticosteroid rinses, gels, eye drops, and ointments. Examples include triamcinolone acetonide, betamethasone, and dexamethasone.
At times, it may be necessary to undergo treatment with drugs that work throughout the body. These drugs include:
- colchicine, a medication used to prevent gout
- medications to suppress the immune system such as azathioprine, cyclosporine, and cyclophosphamide
- medications that change how the body’s immune cells work
Additional medications may be recommended based on the symptoms that develop. Patients should discuss treatment options with their healthcare provider.
Behçet’s does not appear to be linked to pregnancy complications, but the medications used can be harmful to the unborn baby.
For this reason, it is best for any pregnancy to be planned and discussed first with a health provider.
Sometimes a baby is born with neonatal Behçet’s disease. This is very rare and usually resolves itself within 6 to 8 weeks.
There is no evidence that a specific diet will improve symptoms of Behçet’s disease, but eating healthfully will boost overall help and reduce the risk of further problems.
Eating a balanced and varied diet with plenty of fruit and vegetables, and avoiding food that contains too much fat or sugar is best for overall health.
Some people with the condition have suggested that a sensitivity to certain foods may trigger a flare.
In one study, researchers found that the following items made Behçet’s-related mouth ulcers worse in 32 to 35 percent of respondents to a questionnaire:
- Emmental and other cheeses
- nuts generally, almonds, and peanuts
Temporarily cutting out individual items such as pineapple, nuts, or cheese, may help identify any items that make the symptoms worse.
Any supplements or complementary therapies should first be discussed with a health provider, as these may worsen symptoms or interact with treatment.
The exact cause of Behçet’s disease is unknown, but it is thought to be an autoimmune disease.
In this type of condition, the immune system mistakenly reacts to a normal substance or process in the body, leading to symptoms of inflammation.
Certain groups of people have a higher risk of developing the disease than others.
Age: All ages and sexes are at risk of developing Behçet’s disease, but it most commonly affects people in their 20s and 30s. Men typically experience more severe symptoms than women.
Ethnicity and geographic location: These may play a role in how likely a person is to develop Behçet’s disease. The condition is most common in men from the Middle East and Asia, and women from the United States, other Western Countries, Japan, and Korea.
Genetic and hereditary factors: There may be a genetic or inherited component to the disease. Behçet’s disease could also be linked to bacteria, viruses, or environmental factors. More research is needed, however, before these suggestions can be confirmed.
Behçet’s disease affects everyone differently. People with the disease may experience symptom flares, in which the symptoms wax and wane over a period of weeks or longer. The symptoms of Behçet’s disease may also go away without treatment.
The condition causes a wide range of symptoms affecting different areas of the body. These symptoms include the following.
Typically, painful sores in the mouth are the first symptom of Behçet’s disease. The disease can cause a single ulcer to develop or more than one sore.
These sores can from anywhere in the mouth, Specific areas include the tongue, lips, gums, tonsils, lining of the cheeks, roof of the mouth, and the back of the throat.
Mouth sores caused by Behçet’s disease can be:
- shallow or deep
- round or oval
- white or yellow base with a red halo surrounding the sore
- ranging in size from 1 to 20 millimeters
Sores in the mouth often go away within 10 to 20 days with occasional scarring.
According to NIAMS, over half of those living with Behçet’s disease will develop genital lesions. This figure includes both males and females.
Men may have ulcers on the scrotum and penile shaft or head. At times, these lesions are associated with another condition called epididymitis, inflammation of the tubes that carry sperm.
Women may experience lesions on the vulva, vagina, and cervix
These sores typically present as:
- painful red, open sores
- may be large and deep
When these sores heal, scarring often affects the area.
People with Behçet’s disease may experience skin problems called erythema nodosum. This inflammatory skin response causes the skin to develop red and tender nodules that are often ulcerated.
These lesions may bear a resemblance to pus-filled bumps or bruises. Other lesions associated with Behçet’s disease include acneiform nodules, pseudofolliculitis, and papulopustular lesions.
Those living with Behçet’s disease often experience inflammation of the middle layer of the eyes. This condition is known as uveitis.
Anterior uveitis affects the front of the eye. Common symptoms include pain, blurry vision, sensitivity to light, and excessive tear production. A layer of pus may be seen within the eye.
Posterior uveitis affects the back of the eye and retina. Common symptoms include blurry vision, floaters, pain, redness, and sensitivity to light.
Eye problems caused by Behçet’s disease can cause serious complications if they go untreated, including blindness or partial loss of vision.
Inflammation of the veins and arteries can lead to serious complications such as blood clots, aneurysm, and narrowed or blocked vessels.
People with Behçet’s disease may experience a condition called thrombophlebitis, with symptoms such as redness, pain, warmth, and swelling of an extremity due to the presence of a blood clot. Chest murmurs have also been reported.
One or more joints may be affected by joint inflammation related to Behçet’s disease. The pain, swelling, and stiffness typically go away over the course of a few weeks.
The joints most commonly affected include the knees, ankles, wrists, and elbows. Some people affected by Behçet’s disease will experience low back or buttock pain caused by inflammation to a joint in the pelvis.
No permanent joint damage is experienced with Behçet’s-related arthritis
At times, ulcers may form anywhere in the digestive system from the mouth to the anus. Patients affected in this area can experience symptoms such as lack of appetite, pain, vomiting, diarrhea, and rectal bleeding.
A rare condition known as Budd-Chiari syndrome may develop in some patients. In this condition, the vein that carries blood away from the liver is blocked.
Although rare, the lungs may be affected by Behçet’s disease. The condition can cause symptoms such as coughing, shortness of breath, and aneurysms in the pulmonary artery.
Central nervous system problems
Behçet’s disease can lead to inflammation of the brain and brain stem. This inflammation can cause symptoms such as headaches, confusion, strokes, personality changes, disorientation, fever, poor balance, and memory loss.
Symptoms that patients should report to the healthcare team at once include:
- stiff neck
- difficulty with coordination
These symptoms can indicate inflammation of the brain stem. Untreated symptoms can result in a stroke.
In some cases, cardiac or kidney disease may occur.
Additionally, some patients may develop mouth and genital ulcers with inflamed cartilage, referred to as MAGIC syndrome.
This symptom is most likely to occur in patients with both Behçet’s disease and another rare disorder called relapsing polychondritis.
This condition causes cartilage and other connective tissue in the body to become inflamed.
Because there is not a single test to diagnose Behçet’s disease, doctors need to rule out any conditions that mimic the disease.
The International Clinical Criteria for Behçet’s disease diagnosis require that certain symptoms must be present for a diagnosis to be made.
A diagnosis requires:
- The presence of recurring mouth ulcers at least three times in one single year
In addition to the above, at least two of the criteria below must also be met:
- recurring genital ulcers
- eye inflammation (uveitis) confirmed by an eye exam
- skin sores in adults who are not taking corticosteroids
- a positive pathergy test reading within 24-48 hours of the test
In a pathergy test, a doctor inserts a small, clean needle into the skin of the forearm. A positive result is given if a small, red bump forms 1 to 2 days after the needle has been inserted.