Behçet disease is a multisystemic 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 has been reported in Turkey. Males are affected 2-5 times more often than females.
The classic clinical triad of Behçet disease consists of:
Additional clinical manifestations include skin, joints, gastrointestinal tract, genitourinary tract, central nervous system, cardiovascular system, and lung involvement. Vascular complications develop in ~30% (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.
Primarily involves four main systems:
- cardiovascular manifestations of Behçet disease
- affects 5-30% of cases
- thickening of the aorta and superior vena cava (vasculitis)
- inflammation of the veins leads to thrombosis while arterial involvement includes arterial narrowing and aneurysmal dilatation
- pericarditis is the most common cardiac manifestation occurring in 39% of cases
- 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 (most common pulmonary manifestation of Behçet disease)
- 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
- gastrointestinal 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 or arthralgia
Treatment and prognosis
The disease may recur, progress, or resolve (either spontaneously, within a few weeks to several months, or with corticosteroid therapy).
History and etymology
It is named after Hulusi Behçet (1889-1948), Turkish dermatologist.
On imaging consider: