Behçet disease is a multi-systemic and chronic inflammatory vasculitis of unknown etiology.
The mean age at which Behçet disease occurs is 20-30 years. The disease is most prevalent in the Mediterranean region, Middle East and east Asia. The highest incidence is has been reported in Turkey. Males are affected 2-5 times more often than females.
Classic clinical triad of Behçet disease consists of :
- oral ulceration
- genital ulceration
- ocular manifestations
Additional clinical manifestations include skin, joints, gastrointestinal tract, genitourinary tract, central nervous system, cardiovascular system, and lung involvement.
Vascular complications develop in about 20-40% of patients with Behçet disease.
The underlying pathologic process in Behçet disease is vasculitis and perivascular inflammatory infiltrates affecting vessels of differing sizes in various organs.
- factor V leiden mutation 4
Primarily involves four main systems.
- cardiovascular manifestations of Behçet disease - affects 5-30% of cases. Thickening of the aorta and SVC (vasculitis). Inflammation of the veins lead to thrombosis while arterial involvement includes arterial narrowing and aneurysmal dilatation.
- thoracic manifestations of Behçet disease - affects 1-8% of cases. Pulmonary arterial aneurysm: fusiform to saccular, commonly multiple and bilateral, located in the lower lobe or main pulmonary arteries. Subpleural alveolar infiltrates, and wedge-shaped or rounded areas of increased density, which represent focal vasculitis and thrombosis resulting in infarction, hemorrhage, and focal atelectasis
- gastro-intestinal manifestations of Behçet disease - affects 5-60% of cases
- CNS manifestations of Behçet disease - affects 10-50% of cases
- musculoskeletal manifestations of Behçet disease - >50% develop articular disorders/arthralgia7
Treatment and prognosis
The disease may recur, progress, or resolve (either spontaneously, within a few weeks to several months, or with corticosteroid therapy).
Named after Hulusi Behçet (1889-1948), Turkish dermatologist .