Behçet’s Disease

Behçet’s (beh-CHETS) disease is a rare illness that affects the body’s blood vessels. It was discovered by a doctor named Hulusi Behçet in 1937. People with this disease often get painful sores in their mouth and genital areas. It can also cause inflammation in other parts of the body, such as the blood vessels, joints, intestines, skin, lungs, and eyes. We don’t know exactly what causes Behçet’s disease, but there are certain genes that seem to be involved. It commonly affects men and women in their 20s-30s, but older people and children can also be affected. People from the Middle East, East Asia (China, Japan), Turkey, and Iran are more likely to develop this condition.

How Is Behçet’s Disease Diagnosed?

There is no specific test for Behçet’s disease, but rheumatology providers can diagnose it by asking about a person’s medical history, doing a physical exam, and doing blood tests. Nearly all patients with Behçet’s disease develop mouth ulcers. Healthcare providers also look for other symptoms like recurring genital sores, eye inflammation, and skin rashes. Sometimes they check for a specific gene marker, the HLA B*51, but it doesn’t confirm the diagnosis on its own.

What Are Common Treatments?

The treatment of Behçet’s disease depends on the symptoms a person has. If there’s a rash on the skin, creams and gels can be used. Mouth rinses can help with the pain of mouth ulcers, and eye drops can help reduce inflammation in the eyes. Additionally, your rheumatology provider may use alternative medications such as colchicine (“colcrys”, “mitigare”) and apremilast (“Otezla”) to help treat oral and genital ulcers. Medications that lower your immune system may be used to help treat inflammation. Rarely, biologics such as infliximab (“Remicade”) and adalimumab (“Humira”) have been used to treat severe cases.

The Rheumatology Provider Role in Treatment of Behçet’s Disease

Behçet’s disease can affect different parts of the body, so a rheumatology provider, who specializes in diseases like this, will oversee the treatment. They will monitor how the disease is affecting different organs and recommend the right medications and doses. They will also watch for any side effects. Sometimes, if needed, they may suggest imaging tests or refer the patient to another specialist.

Living with Behçet’s Disease

Living with Behçet’s disease can be challenging, but with the right education and communication with your healthcare providers, it is possible to live a fulfilling life. It’s important to take medications as prescribed and follow the recommendations of the rheumatology provider. During flare ups, it is a good idea to rest, reduce stress, and inform the rheumatology provider if a medication change is needed. Moderate exercise like walking or swimming can help with symptoms, reduce stress, and improve mood. Behçet’s disease is rare, and it can cause a feeling of isolation, so joining local support groups or online communities can help connect with others and reduce the burden of the disease.

Written April 2023 by Mohammad Ursani, MD, and reviewed by the American College of Rheumatology Committee on Communications and Marketing.

This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.