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 eastEast Asia. The highest incidence 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 ~30% (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-B51 ⁴
• factor V leidenLeiden mutation ⁴
• superficial thrombophlebitis ⁸

Distribution

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 leadleads 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
• 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/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.

Differential diagnosis

On imaging consider:

• Hughes-Stovin syndrome