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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, Middle East and East Asia. It is rarely reported in Europe. 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 the following:
Additional clinical manifestations include the involvement of:
skin and joints
gastrointestinal and genitourinary tracts
central nervous system
cardiovascular and respiratory systems
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:
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 9
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 7
The diagnosis of Behçet disease relies on fulfilling a number of criteria as no single diagnostic test is available 10,11.
The International criteria for Behcet’s disease assigns one or more ports to a number of criteria, and the likelihood of a diagnosis of Behçet disease increases as the score increases 11.
ocular lesions: 2 points
genital aphthosis: 2 points
oral aphthosis: 2 points
skin lesions: 1 point
neurological manifestations: 1 point
vascular manifestations: 1 point
positive pathergy test: 1 point
A simple cut off score of 4 or more is sufficient for the diagnosis in the correct clinical setting, however, a more probablistic interpretation of the score is also available 11.
0 or 1 = almost certainly not Behçet disease
2 = very unlikely
3 = possible but unlikely
4 = probable
5 = highly likely
≥6 = almost certainly Behçet disease
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), a Turkish dermatologist, who first described it in 1937.
On imaging consider: