The 2019 Congress of Clinical Rheumatology held May 2nd through the 5th in Destin, Florida featured a talk by Yusuf Yazici, the Director of the Behçet Syndrome Center at the New York University School of Medicine. He discussed the diagnosis, treatment, and ongoing research for the condition.
Behçet Syndrome
Behçet’s syndrome is a form of vasculitis. It is a rare and chronic condition that is most common in the Middle East, Asia, and Japan. The disease typically presents when patients are in their 20s or 30s. It affects all sexes but is generally worse in males.
Symptoms of the condition include ulcers in the mouth, genitals, eyes, and skin. It can also cause arthritis, uveitis, and skin lesions. The most severe forms of the disease include blood clots, ulcers within the digestive system, and inflammation in the brain. The symptoms vary from person to person and patients typically experience flare ups followed by periods of remission.
Its features make it very different from any other autoinflammatory or autoimmune disease such as systemic lupus or rheumatoid arthritis.
Unfortunately, there is no clinical test that can diagnose this disease. Instead, physicians rely on an evaluation of patient symptoms. This means that misdiagnosis is very common. Diagnosis is typically provided only after all other diagnoses are ruled out.
In 1990, criteria for a Behçet’s syndrome diagnosis were created. These criteria have become standard. They say that first, oral ulcers must be present and second, patients must have at least two of the following features- pathergy, eye lesions, skin lesions, or genital ulcers. If physicians don’t utilize this criteria, they are most likely using the criteria created in 2003 by the Japanese. This set of criteria require patients to have all four of the following symptoms- oral ulcers, genital ulcers, ocular lesions, and skin lesions.
Treatment Options
Thankfully, Behçet’s syndrome patients tend to experience improvement as they age. Even though it’s not fully understood why this condition gets better over time (in terms of severity and frequency of attacks), it’s obviously a positive thing and does effect the treatment that patients are prescribed. Typically, all manifestations of the disease occur less frequently. Unfortunately, major vascular pathology and CNS disease is not typically reduced.
What’s astounding is that these improvements are made over time even without treatment. However, researchers make it clear that to reduce the burden of disease, they still owe their patients the most effective treatment as soon as possible. This way, they can be put into remission as fast as they can.
Common treatments for this condition are topical medicines, corticosteroids, immunosuppressive drugs, and biologics as well as rest, exercise, and a healthy diet. Colchicine is a typical prescription because it is considered low risk. Azathioprine, TNF-alpha antagonists (apremilast and IFN-alpha), and IL-1 inhibition are also commonly prescribed therapies. However, research is ongoing to find new options for patients, and scientists are excited about the promise of many up and coming therapies. Ultimately, the goal is to find a cure.
“Our treatment options are going to better optimized for each patient that we see.”
You can read more about Behçet’s syndrome here.
