Behçet’s Disease

Behçet’s disease (BD) is a systemic vasculitis characterized by recurrent oral and genital ulcers, and ocular inflammation, but it may also involve the joints, skin, central nervous system, and gastrointestinal tract. Although it has a worldwide distribution, it is more prevalent in the countries along the Silk Road. The etiology of the disease remains unknown. The diagnosis is based on clinical grounds, and classification criteria proposed by the International Study Group perform well in a clinical context. However, they present two main limitations. Firstly, the exclusion of major organ involvement, such as vascular, neurologic, and gastrointestinal involvement and, secondly, the low positivity of the pathergy test in Caucasians, reducing its diagnostic value in this group. To solve these problems, in 2014, international criteria for BD were designed based on a scoring system and including vascular and neurological involvement. The treatment of BD remains largely empirical and considerable differences exist in practical approaches to treatment. The primary goals of management are symptom control, early suppression of inflammation, and prevention of end-organ damage. Regarding prognosis, the main associates of mortality in BD are major vessel pathology and neurologic involvement.