DESTIN, Fla. — Behçet syndrome — which is classified as a vasculitis — is marked by distinct features that differentiate it from other autoimmune and autoinflammatory diseases, according to Yusuf Yazici, MD, clinical associate professor of medicine and director of the Behçet's Syndrome Center, New York University School of Medicine.
In a presentation here at the Congress of Clinical Rheumatology, Yazici outlined demographics, diagnostic criteria, natural history and treatment of the disease.
“Behçet syndrome is an interesting disease in that it’s not autoinflammatory, it’s not autoimmune, it does not fit a lot of things, and if you look at the Behçet classification, it’s just by itself with Cogan syndrome among vasculitis conditions, which I’ve never seen, but is not very common,” he said.
Behçet's syndrome is marked by distinct features that differentiate it from other autoimmune and autoinflammatory diseases, according to Yazici.
Behçet syndrome is endemic in the Mediterranean basin, Korea and Japan; it rarely presents before puberty or after age 50; and occurs equally in men and women, although the disease is worse in men.
There is no gold standard and no test that can confirm Behçet syndrome — diagnosis is based solely on clinical features. However, the ISG Criteria for Behçet, which were developed in 1990, are quite standard. To meet criteria, patients must have oral ulcers, and at least two of these four clinical features: genital ulcers, skin lesions, eye lesions or pathergy. The second-most used criteria were created in Japan in 2003. According to these criteria, to have the complete form of the disease patients must have four “major symptoms:” genital ulcers, oral ulcers, skin lesions and ocular lesions.
With time, all disease manifestations occur less frequently, except for CNS disease and major vascular pathology, he explained. In this way, the disease course is unlike rheumatoid arthritis or systemic lupus erythematosus.
“Disease burden gets better with time — even if you don’t treat patients, the number of attacks and severity of attacks over 20 years get milder in most patients.,” Yazici said. “That’s very different than a lot of our rheumatic diseases. We don’t know why it happens in Behçet's, but it also effects how we treat those patients.”
In terms of treatment, colchicine is commonly used because of the low danger it possesses. According to Yazici, if patients can tolerate colchicine and remain on treatment, clinicians will know within 4 to 6 weeks whether the patient is responding. Another treatment option, azathioprine, is to Behçet's syndrome what methotrexate is to RA, he said. Additional options include TNF-alpha antagonists, IFN-alpha and apremilast (Otezla, Celgene), which have been shown to be effective for oral ulcers. Lastly, IL-1 inhibition has been effective in some patients, but does not work very well, according to Yazici.
“Most patients — at worst, two out of three — do well with time and treatment, so I think we owe it to our patients to give them that opportunity to aggressively treat them so we can get them into remission,” he said. “We have several new agents on the horizon, so I think our treatment options are going to better optimized for each patient that we see.” – by Stacey L. Adams
Yazici Y. Update in Behçet syndrome; May 2-5, 2019; Destin, Florida.
Disclosures : Yazici reports professional relationships with Bristol-Myers Squibb, Celgene, Genentech and Samumed.