Revision 29 for 'Behçet disease'All Revisions
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.
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 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
- 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 / arthralgia 7
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