Behçet’s Syndrome

Abstract

Behçet's syndrome is a systemic vasculitis with an unknown etiology affecting the small and large vessels of the venous and arterial systems. At least two clusters of disease expression have been described. The first includes superficial vein thrombosis, deep vein thrombosis, and dural sinus thrombi. The second includes acne, arthritis, and enthesitis. The presence of these clusters suggests there may be more than one disease mechanism operative in this complex disorder. Recent European League Against Rheumatism guidelines are useful for the management of the disease in organ systems distinct from the vascular, neurological, and gastrointestinal systems. This is because of a lack of controlled studies evaluating such vascular, neurological, and gastrointestinal complications.

Treatment of Different Types of Vasculitis

“…Behçet disease is a multisystem inflammatory disease with an unknown etiology that affects all types and sizes of blood vessels (Calamia KT & Kaklamanis PG, 2008;Langford CA, 2010;Yazici Y et al, 2010). Treatment of Behçet disease is adjusted depending on the type and severity of symptoms, sex, and age (Yazici Y et al, 2010).…”

“…Treatment of Behçet disease is adjusted depending on the type and severity of symptoms, sex, and age (Yazici Y et al, 2010). Colchicine, 1.0-2.0 mg/day, can be effective for genital ulcers, erythema nodosum, and arthritis among women, although merely for arthritis in men (Yurdakul S et al, 2001).…”

“…It was reported that tumor necrosis factor TNF α) antagonists, which are infliximab, etanercept, and adalimumab, improve symptoms in patients with eye, mucocutaneous, and gastrointestinal involvement, as well as neurological disease, and even pulmonary artery aneurysms (Sfikakis PP et al, 2007). Corticosteroids are widely used in the treatment of Behçet disease (Yazici Y et al, 2010).…”

“…For patients in the early disease stages of pulmonary and peripheral arterial aneurysms, cyclophosphamide pulse therapy and corticosteroids are especially effective (Yazici Y et al, 2010).…”

The Role of Biological Therapies in the Management of Systemic Vasculitis

“…Behçet's disease is a chronic multisystemic vasculitis affecting small and large arterial and venous branches (Yazici et al, 2010). The condition presents a distinctive geographical pattern, with higher incidence rates in Turkey, other Mediterranean countries and Middle and Far East countries, following the ancient "silk route" until Japan.…”