Patient education: Behçet syndrome (The Basics)

What is Behçet syndrome? — Behçet syndrome is a type of vasculitis, which is a general term that means inflammation of the blood vessels. Inflammation can make blood vessel walls thick, weak, narrow, or scarred. In Behçet syndrome, these changes can happen in blood vessels all over the body. This can lead to different types of symptoms.

Doctors don't know what causes Behçet syndrome. It happens most often in adults age 20 to 40.

Behçet syndrome is a lifelong disease. Symptoms can come and go over time.

What are the symptoms of Behçet syndrome? — The most common symptoms are:

●Open sores (called "ulcers") in the mouth – These sores can be very painful. They usually go away on their own, but then often come back later.

●Open sores on the genitals – These sores can be very painful. They usually happen on the scrotum (the skin sac between the legs) or on the vulva (the area around the vagina).

●Eye inflammation (called "uveitis") – This can cause eye redness or pain, or blurred vision. If the eye inflammation isn't treated, it can lead to blindness.

●Arthritis, which is joint pain and swelling – This commonly affects the knees, ankles, or wrists.

●Skin symptoms – These can include bumps, rashes, or acne-like symptoms.

●Digestive symptoms – These happen when ulcers form in the digestive tract (figure 1). Symptoms can include lack of appetite, nausea, belly pain, or diarrhea.

People with Behçet syndrome can have other symptoms, too. These can affect the brain, kidneys, heart, lungs, or other parts of the body.

Is there a test for Behçet syndrome? — There is no test that can tell for sure whether you have Behçet syndrome. To figure out whether you have it, your doctor or nurse will ask about your symptoms and do an exam. They might also order blood tests to make sure that your symptoms are not caused by another condition.

How is Behçet syndrome treated? — There is no cure for Behçet syndrome. But medicines can help ease and control your symptoms. They can also help prevent long-term problems from the disease. The right medicines for you will depend on your symptoms and how severe your disease is.

In general, doctors use the following medicines to treat Behçet syndrome:

●Steroid medicines, such as prednisone – These medicines reduce and control inflammation. Because steroid medicines can cause serious side effects, doctors try to give them for as short a time as possible.

●Colchicine – This medicine helps relieve pain and swelling in the joints. It also helps prevent mouth and genital sores. Another medicine called apremilast can also help prevent mouth sores.

●Medicines that calm down or "turn off" the body's infection-fighting system, called the "immune system"

Other treatment depends on the part of the body affected. For example, eye inflammation is also treated with eye drops or other medicines that go in the eye.

People with certain blood vessel problems might need treatment other than medicines. For example, if a blood vessel wall gets too thin and bulges out, it might need to be fixed with surgery.

What if I want to get pregnant? — If you want to get pregnant, talk with your doctor or nurse before you try. Some medicines for Behçet syndrome are not safe to take during pregnancy. You might need to get your symptoms under control or switch medicines before you try to get pregnant.

What will my life be like? — You will need regular exams and tests to check for the problems Behçet syndrome can cause. Take your medicines exactly as prescribed, and tell your doctor or nurse if you have any side effects. The right treatment can help you control your symptoms and stay as healthy as possible.

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