Behcet disease

Definition: Behçet disease is a chronic, relapsing, systemic disorder of unknown etiology, characterized by recurrent oral and genital ulcers, uveitis, and other clinical manifestations in multiple organ systems.

Although the diagnosis is made on the basis of the combination of typical clinical symptoms, radiologic findings of Behçet disease show characteristic features of its involvement in the gastrointestinal, neurologic, cardiovascular, and thoracic organ systems.

In the gastrointestinal tract, Behçet disease may produce various types of ulcers in the esophagus, stomach, and small and large intestines, as well as deeply penetrating ulcerations in the ileocecal region, with frequently accompanying enteric fistulas.

Neurologic involvement includes typical and atypical parenchymal neurobehcet disease, dural sinus thrombosis, cerebral arterial aneurysm, occlusion, dissection, and meningitis.

Vascular involvement is divided into three subsets including venous occlusion, arterial occlusion, and arterial aneurysm.

Cardiac manifestations include intracardiac thrombus, endomyocardial fibrosis, periaortic pseudoaneurysm, and rupture of the sinus of Valsalva.

Manifestations of Behçet disease in the thorax include pulmonary arterial aneurysm, pulmonary arterial thromboembolism, thrombosis in the superior vena cava, pulmonary infarction, hemorrhage, and vasculitis of the pleura and pericardium.

These various manifestations of Behçet disease respond to steroid treatment; however, one of the characteristics of Behçet disease is the high rate of complications and recurrence after surgery.

Synopsis

 neurologic lesions
 ophthalmic

• retinal

 cardiovascular
 pulmonary
 gastrointestinal
 visceral
 genitourinary
 mucous lesions

• oral ulcers

 synovitis

 cutaneous lesions

• cutaneous vasculitis

 venous occlusions

Associations

 myelodysplastic syndrome (#16213367#)
 antithrombin III deficiency (#16213367#)

Predisposition - Susceptibility

 Genome-wide association analysis identifies new susceptibility loci for Behçet’s disease and epistasis between HLA-B*51 and ERAP1. (doi:10.1038/ng.2520 )

References

 Lakhanpal S, Tani K, Lie JT, Katoh K, Ishigatsubo Y, Ohokubo T. Pathologic features of Behcet’s syndrome: a review of Japanese autopsy registry data. Hum Pathol. 1985 Aug;16(8):790-5. PMID: #4018777#