About the Behçet’s Syndrome Working Group
Behçet’s syndrome is a rare disorder that affects several organs and organ systems. It is characterized by inflammation of the vessel wall and it can involve vessels of all sizes and both veins and arteries. It affects men and women, often before age 30, causes significant reduction in quality of life, can result in serious disability, and may be life-threatening. The disease course, severity, and organ involvement varies substantially between patients, and differences occur based on age at presentation and sex, making it quite difficult to recommend a single management strategy. The most common manifestations are mouth ulcers followed by genital ulcers, other skin lesions, and inflammation of the joints. These manifestations follow a relapsing and remitting course and impair the quality of life of patients with Behçet’s syndrome. When organ involvement is present in addition to the skin and joint inflammation, Behçet’s syndrome may be organ- or life-threatening. Eye involvement affects almost half of the patients and can cause blindness if not treated. The vascular system can be involved, usually in the form of blood clots of large veins. Arterial involvement causes enlargement of the arteries and can be fatal if the arteries rupture. Gastrointestinal system involvement results in ulcers in the bowels that can cause serious bleeding. Nervous system involvement can cause permanent damage and disability. Behçet’s syndrome usually starts between the ages of 20 and 30 years, with an almost equal frequency among men and women. However, the serious organ manifestations are more frequent among men and those whose disease start at an earlier age. Management of Behçet’s syndrome depends on the age and gender of the patient, the organs that are involved and the frequency and severity of each type of lesion.
OMERACT Endorsed Core Domain Set for Behçet’s Syndrome
Working Group Members: