Behçet disease

Behçet disease is a multi-systemic and chronic inflammatory vasculitis of unknown aetiology.

Epidemiology

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.

Clinical presentation

Classic clinical triad of Behçet disease consists of :

1. oral ulceration
2. genital ulceration
3. 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.

Pathology

The underlying pathologic process in Behçet disease is vasculitis and perivascular inflammatory infiltrates affecting vessels of differing sizes in various organs.

Associations

• HLA-B51⁴
• factor V leiden mutation ⁴

Distribution

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, haemorrhage, 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).

EtymologyHistory and etymology

NamedIt is named after Hulusi Behçet (1889-1948), Turkish dermatologist .

Differential diagnosis

• Hughes-Stovin syndrome