What is Behcet disease?

Behcet disease is a type of autoimmune disease. It causes inflammation of blood vessels. It’s a rare disease in the U.S., but it's more common in the Middle East and Asia. It’s more common in people in their 20s to 40s, but it can happen at any age. The disease tends to be more severe in men, in younger people, and in people from the Middle East or Asia.

An autoimmune disease is caused by a problem with the immune system. The immune system’s job is to protect the body from disease. It does this by attacking things in the body, such as viruses, that may cause harm. When you have an autoimmune disease, your immune system attacks your own body.

In Behcet disease, the immune system attacks blood vessels in your body. This causes them to become swollen and inflamed. This may limit blood flow to vital organs and tissues. It can affect any size of blood vessels that carry blood in your body. The inflammation can cause many symptoms, such as sores on the mouth or genitals.

The disease is named after the Turkish dermatologist, Dr. Hulusi Behcet. He first described the disease in the 1930s.

What causes Behcet disease?

Researchers are still working to learn what causes Behcet disease. Changes in genes, problems in the immune response, and environmental factors are all being studied as causes. Behcet disease is not contagious. You can’t get it from someone else who has it. But it may run in some families.

Who is at risk for Behcet disease?

Having a gene called HLA-B51 may increase your chance of getting Behcet disease. Other genes may also increase your risk of the disease. You have an increased risk if other members of your family have had the disease.

What are the symptoms of Behcet disease?

Symptoms can vary, depending on which blood vessels are affected. They can range from mild to severe. The most common symptoms are:

• Mouth sores. They are often the first symptom. These may be painful and make it hard to eat. Several sores may appear at one time.

• Genital sores. These usually appear on the vulva in women or on the scrotum in men. They can be quite painful.

• Skin sores. These often look like red or pus-filled bumps. They often appear on the legs or upper torso.

• Arthritis. This causes pain, swelling, and stiffness. It’s most common in the knees, ankles, wrists, and elbows. The arthritis usually lasts a few weeks and doesn’t cause permanent damage to the joints

• Eye problems. Inflammation can affect your eye. It can cause blurred vision, pain, and redness. It may cause vision impairment or blindness.

Symptoms may last for a long time, or they might go away within a few weeks. In most cases, symptoms come and go over long periods of time. When the symptoms are active, this is called a disease flare.

Some less common problems that can be caused by Behcet disease include:

• Fever and fatigue

• Blood clots in the veins of the legs (thrombophlebitis)

• Obstructed blood vessels causing symptoms in other parts of the body

• Inflammation of the brain or meninges (encephalitis or meningitis)

• Aneurysms or weakening in various blood vessels

• Ulcers of the digestive tract, causing bleeding, belly pain, or diarrhea

• Kidney disease

• Lung problems

• Urinary or erectile problems

• Inner ear problems

• Inflammation around the heart (pericarditis)

How is Behcet disease diagnosed?

Your healthcare provider will ask about your health history and your symptoms. You’ll also have a physical exam. It can be difficult to diagnose Behcet disease. Some of its symptoms are similar to those of other diseases. There is no single test that can confirm the diagnosis. For these reasons, your healthcare provider may take a while to diagnose it. A variety of specialists may work together to diagnose Behcet disease. It may take months or longer for your symptoms to match a pattern that can help confirm the disease.

Some tests can help diagnose Behcet disease, such as:

• A skin prick test that is usually positive in people with Behcet disease

• Blood tests to look for inflammation

• Skin biopsy, which is taking a tiny sample of skin to see if sores are from other illnesses

• Imaging tests, like X-rays and scans

How is Behcet disease treated?

Currently, there is no cure for Behcet disease. But most people can control their symptoms with treatment. Your treatment may vary depending on your symptoms and how mild or severe they are. Treatment may include:

• Anesthetic cream. This can help to block pain from sores.

• Steroid cream. This can help to reduce pain and inflammation of sores.

• Oral steroid medicine. This is used to reduce inflammation and other symptoms. You may only need to use steroids for a short period of time to treat a disease flare-up.

• Immunosuppressive medicine. This can help control an overactive immune system. These medicines include colchicine, azathioprine, and cyclosporine. You may need to take immunosuppressive medicines over a longer period of time.

• Newer biologic medicines. Immune altering medicines, including etanercept, infliximab, apremilast, and intravenous immune globulin, have also shown promise.

• Other treatments as needed. You may also need additional treatments for other problems. For example, you may need blood thinner medicine if you have a blood clot in a major artery or vein.

Because the condition can affect so many parts of the body, you may need to see a range of healthcare providers to treat your condition. For example, you may need to see an eye healthcare provider if your eyes are affected.

What are possible complications of Behcet disease?

One possible complication of Behcet disease is blindness. Inflammation of the brain and meninges, the covering of the brain and spinal cord, may happen. Blocked blood vessels can lead to problems anywhere in the body.

Coping with Behcet disease

Keeping a healthy lifestyle is important to help manage the symptoms of Behcet disease. During a disease flare-up, make sure to:

• Get enough rest

• Eat healthy foods

• Get some exercise

When should I call the healthcare provider?

Call your healthcare provider right away if you have any of the following:

• Sudden severe symptoms, such as stiff neck and headache that may be meningitis

• Any eye problems

• Mild symptoms that may be signs of a disease flare-up

Key points about Behcet disease

• Behcet disease is a type of autoimmune disease. It causes abnormal inflammation in many blood vessels of your body. With treatment, most people can manage their symptoms.

• The exact cause of Behcet disease is unknown. Genes, environmental triggers, and an abnormal immune response may be possible causes.

• It causes symptoms, such as mouth sores, genital sores, arthritis, and vision problems.

• There is no single test for Behcet disease. It may take some time to be diagnosed.

• Treatments include anesthetic and steroid creams, and steroid and immunosuppressive medicines.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.

• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your healthcare provider if you have questions, especially after office hours or on weekends and holidays.