Behçet’s disease (BD) is a rare immune disorder that causes inflammation of the blood vessels. It can affect both arteries and veins of any size. The most common symptoms are sores in the mouth and/or genitals but it can also affect the skin, eyes (causing the conditions iritis or uveitis), joints (arthritis) and other organs.
Behçet’s disease belongs to a family of arthritic diseases called vasculitis. The word vasculitis means inflammation of blood vessels.
It can be tough to diagnose Behçet’s disease because of how its symptoms vary from person to person. It is usually best identified by a rheumatologist.
It is very important that BD is treated as early and aggressively as possible to prevent serious damage to blood vessels and the tissues that they in turn supply with blood.
BD is typically diagnosed in young adults between the ages of 20 to 40. It affects men and women equally. It is more prevalent in people whose descendants are from the “Asian silk route” from the Far East through to the Middle East and the Mediterranean. It is most common in Turkey. A gene called HLA-B51 has been associated with the disease.
Behçet’s disease tends to flare: there may be periods where symptoms are well controlled and other times they flare up and get worse. In many patients, BD becomes less severe over time.
Behçet’s disease (BD) presents a wide variety of potential symptoms and can be very different from person to person.
It also tends to flare up and down. That means that many people with BD experience periods where their symptoms are well controlled and other times they flare up again.
Like many other types of vasculitic (vasculitis family) diseases, the first symptoms that most people experience are similar to the flu. They might start feeling unwell and commonly have a fever, achiness, and perhaps weight loss. These symptoms can last for some time. As these symptoms are not specific, a diagnosis can be difficult in the early stages.
The most frequent symptom in people with established BD are sores in the mouth. They may be present all the time, or they may come and go. It’s typical for a person with the disease to have around 3 attacks of these sores per year.
Sores on the genitals are also very common. In men, the sores could appear on the scrotum or glans (top part) of the penis. In women, sores might be found on the vulva or in the vagina.
Skin rashes are also common and elevated bumps on the skin called pustules may appear. This can look a lot like acne.
The eyes can also be affected by BD. A condition called iritis or uvetitis may be present, which is a type of inflammation of the inner parts of the eye.
Arthritis can be a symptom of Behçet’s disease. This can result in pain and swelling of he affected joint. The arthritis can be intermittent and come when the disease flares.
Behçet’s disease can cause inflammation of the large arteries such as the pulmonary artery, which brings blood from the heart to the lungs. If these vessels are involved, patients might experience shortness of breath, a cough or chest pain. Inflammation can affect veins of any size. When this happens, there is an increased risk of blood clots.
Other Organs and Tissues
Behçet’s can cause various other symptoms depending on which blood vessels are affected, and which organs and/or tissues they supply blood to.
Behçet’s disease less frequently affects the nerves, gastrointestinal system (stomach & bowels), kidneys and heart.
Behçet’s disease (BD) is best diagnosed by a rheumatologist, a type of doctor that specializes in arthritis, autoimmune, and autoinflammatory diseases. BD can be tricky to diagnose and having someone experienced is very useful.
To diagnose BD, a rheumatologist will take a careful and complete history and perform a thorough physical examination. The doctor will be seeking to confirm that their patient meets a “checklist” of characteristic signs and symptoms associated with Behçet’s disease. Among other considerations, they will look for frequent sores in the mouth or genitals, lesions on the eyes or skin, and may conduct a pathergy test (“skin prick test”) to see if there is a positive result.
Next, they will usually order blood tests, x-rays, and other types of tests to confirm their diagnosis and rule out other possible conditions.
Common Tests for Behçet’s Disease
Pathergy (“Skin Prick”) test for a common sign of BD: This test involves 3 skin pricks. If an irritation in the form of a skin lesion or pustule (ulcer) develops at the sites of the needle prick (this is called pathergy), then the test is positive. This result is sometimes seen in people who have Behçet’s disease.
Looking for inflammation: BD is an autoinflammatory disease so these tests are expected to have abnormal results. Common tests include a Complete Blood Count (CBC), Erythrocyte Sedimentation Rate (ESR), and C-Reactive Protein (CRP)
To rule out other forms of vasculitis: The Anti-neutrophil cytoplasmic antibody (ANCA) test can help rule out other forms of vasculitis.
Looking for a genetic marker: A positive genetic test for a gene called HLA-B51 can support a diagnosis of Behçet’s disease because it is more common in people who have this gene.
X-rays: A baseline (start) chest X-ray is often ordered so doctors can look for changes to the pulmonary arteries and monitor the progression of the disease over time.
Looking for nerve involvement: Nerve conduction studies can be performed to look for nerve involvement
Scientists don’t know what causes Behçet’s disease, but it is thought to be an autoinflammatory disease. An autoinflammatory disease is caused by a malfunction with the innate immune system. An autoimmune disease is different as is caused by a malfunction of the adaptive immune system.
It is suspected that something in the environment triggers the inflammation underlying Behçet’s disease in people who have the right mix of genes.
A cause related to genetics is supported by the fact that BD is most common in Turkey and is prevalent along the “Asian silk route”, a part of the world that stretches from the Far East through to the Middle East and the Mediterranean.
Behçet’s disease should be treated early and aggressively to avoid permanent damage to blood vessels, organs, and other tissues.
A rheumatologist, a type of doctor that specializes in arthritis and autoimmune disease, is one of the best people to help people with BD manage their condition. Depending on a patient’s individual symptoms, they may need to see other doctors as well, such as a lung specialist (respirologist). Patients might also want to consider attending a vasculitis clinic to receive services and support from a team of experts in vasculitis diseases.
There are many medications that can be used to treat Behçet’s disease. The choice of medications depends on the specific symptoms that a person with BD is experiencing.
In the short-term, prednisone works very well to rapidly control common symptoms such as mouth and genital sores, eye inflammation and skin rashes. In some cases it may be used for longer periods of time, but this increases the risk of side effects. Patients should to discuss the risks and benefits of using prednisone with their rheumatologist.
Mouth or genital sores
Topical corticosteroid medications can be applied directly to sores in the mouth or genitals as prescribed by a doctor. Another option is a medicine called Colcrys (colchicine), which can be taken orally.
Prednisone can be used when topical medications and Colcrys (colchicine) are not effective. Other immune modulating agents such as Imuran (azathioprine), Neoral (cyclosporine), thalidomide, and a class of drugs called anti-TNF biologics can be used when prolonged high doses of prednisone are required.
For skin rashes, topical corticosteroid medications can be applied directly to the affected area. Another option is a medicine called colchicine, which can be taken orally. Prednisone is often used with colchicine for severe skin rashes. Other immune modulating agents such as azathioprine (Imuran) can also be used.
If the eyes are affected, steroid drops (that control inflammation) and dilating drops (that open or expand the pupils) will be prescribed. In some cases oral steroids like prednisone may be required. If the disease is further back in the eye (posterior) other immune modulating medications are used.
Swollen joints may be treated with medications called Non-Steroidal Anti-Inflammatory Drugs (NSAIDs). Luckily there are about 20 different anti-inflammatory medications available. So if one doesn’t work for you, try another. Other systemic therapies used for arthritis include prednisone, colchicine, azathioprine (Imuran), methotrexate, and anti-TNF medications.
Vascular disease and other major organ involvement
Prednisone is used for any serious organ involvement including vascular disease.
Other immune modulating medications such as Cytoxan (cyclophosphamide), Neoral (cyclosporine) or Imuran (azathioprine) can also be used to control symptoms and prevent long-term damage. These medications are effective, but may take a few months before the effects are felt.
In more severe cases where other medications are not successful, a newer group of medications called anti-TNF biologics may be prescribed. Examples include Remicade (infliximab), Humira (adalimumab) and Enbrel (etanercept). A medication called thalidomide also has anti-TNF effects.
When other organs are involved, combinations of the above medications are often used. For example, DMARDs can be used in combination with prednisone. Biologics are often given in combination with DMARDs or prednisone. Combination therapy has been shown to work better than treatment with either medication alone. Using biologics can also help reduce the dose of prednisone that is used over the long-term.
Analgesic (pain relief) medications can be used to help control pain. They do nothing to control BD or to prevent damage to tissues such as joints. Analgesics can range from simple things like acetaminophen (paracetamol, Tylenol) to more potent narcotics like morphine.
Living with BD
The pain and stiffness caused by BD can sometimes limit people’s normal activities, including work. There are many things people can do to lessen the impact of BD on their work and daily routine.
We recommend adjusting features of the workplace to help make working with BD easier. For example, people that sit for much of the day can adjust the position of chairs and desks for proper posture. Vehicle seats can be adjusted to make driving more comfortable and reduce stress on joints and affected tissues.
Those who find out they have BD while they’re still young can consider choosing work that isn’t physically demanding on their joints and back.
Exercise is important to overall health. It also helps keep the joints moving properly, and helps protect them by strengthening the muscles around them.
The level and amount of exercise people with BD can do depends on the activity of their disease.
A trained arthritis physiotherapist is the best person to help design an exercise program tailored to the needs of patients with BD.
The following are some useful articles on exercising with arthritis:
Exercise and Arthritis: An article by arthritis physiotherapist Marlene Thompson
Exercising in a Flare: Another excellent article by Marlene Thompson on how to cope with flares through your exercise routine.
Alcoholic beverages are not an effective treatment for BD, and they can interact with many medications.
People with BD that plan on drinking should ask their doctor about possible negative interactions with the medications they take.
Some examples of medication that mixes poorly with alcohol includes, and is not limited to: the DMARDs methotrexate, Arava (leflunomide), and Imuran (azathioprine).
Smoking is not healthy for people with BD. It can make symptoms worse and harder to treat.
Research has shown that some arthritis medications do not work as well in people who smoke.
We recommend that smokers quit immediately to reduce the impact of their symptoms, improve the effectiveness of their medications, and improve their overall health.
Smoking significantly increases the risk of cardiovascular disease. The chronic (long-term) inflammation caused by rheumatic diseases like BD already increases this risk without a big extra boost from smoking.
Travel is still possible when you have BD.
It is best for people with this disease to get organized well in advance of a trip to ensure a smooth, comfortable, and enjoyable time.
Check out our Travel Checklist page.
Though BD does not particularly cause a loss of sex drive, it can cause pain, fatigue and emotional hardships. These hardships risk creating barriers to sexual needs, ability, and satisfaction.
People with BD can take comfort knowing that sex and intimacy can be maintained. In many cases, it can help draw partners closer together, especially through improved communication.
For more information on intimacy and arthritis, a great book is: Rheumatoid Arthritis: Plan to Win by Cheryl Koehn, Taysha Palmer and John Esdaile.
Behçet’s Disease (BD) has no effect on fertility. The chances of getting pregnant for people with BD are the same as with people that don’t have it. BD also has no known effects on the developing fetus or the newborn.
The two main issues doctors worry about with BD and pregnancy are:
Patients with BD might be treated with a number of medications that can affect pregancies including NSAIDs, anti-TNF biologics, and occasionally DMARDs. It is important for people with BD who want to become pregnant to discuss their medications with their doctor and create a pre-pregnancy plan. A plan is important for a number of reasons. For example, discontinuing a particular medication for safety of the newborn might cause BD to flare, so its wise to have a plan in place to handle these possibilities.
Any type of arthritis that affects the pelvis or the hips can make vaginal delivery difficult. It is important for those planning pregnancy to discuss delivery options with an obstetrician.
Following the basics of healthy eating can help improve health and well-being in everyone, including those with BD. Keeping a healthy weight helps reduce the load on weight-bearing joints including the spine, hips, and knees, making it easier to live with the disease.
Unfortuantely, no special diet has ever been proven to significantly alter the course of BD or any other types of arthritis.
In addition, no known natural remedies or complementary therapies have been proven to help BD in any significant way.
People with BD should consult with their doctor to make sure that any supplements or alternative therapies they consume will not negatively interact with their medications.
Its very important for people with BD to attend regularly scheduled appointments with their rheumatologist, and promptly get any tests that are ordered by their doctors.
It is often very important that medications are taken exactly as prescribed. Patients should always talk to their doctor about any issues they experience, or if they would like to change anything.
Behçet’s Disease is a chronic disease and a rheumatologist is the best partner in helping patients achieve the best quality of life possible.