Behçet Syndrome

Suspected autoimmune vasculitis with a genetic predisposition.

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Behçet Disease.

First described in 1937 by Dr. Helusi Behçet, a Turkish dermatologist.

Prevalence is highest in Turkey (80-320:100,000 in the general population) and Asia (2-30:100,000 in the general population), but patients from many parts of the world have been described. Male gender is predominant, especially in severe Behçet disease.

The majority of cases are sporadic, but familial forms are described. Behçet disease has been associated with HLA-B51 in 45 to 60% of cases, but the significance of this finding for the pathogenesis of Behçet disease is not established.

The cornerstone of the disease is a multisystemic vasculitis involving large and small vessels in various degrees. The underlying cause has not been defined, but an autoimmune pathogenesis, including an abnormal T-cell response to microbial antigens and cross-reaction toward body tissue, is suspected. Characteristically, skin pathergy is present or so-called positive, that is, an exaggerated reaction with formation of a papule or a pustula after minor trauma such as a needle puncture occurs. Although helpful (but not pathognomonic) for the diagnosis of Behçet disease, the majority of patients do not show this reaction.

No specific tests are available. The diagnosis relies on the clinical picture, and criteria for establishing the diagnosis have been published by the International Study Group for Behçet's Disease.

Recurrent oral and genital ulcerations and uveitis or retinal vasculitis are the characteristic findings in Behçet disease. Systemic manifestation may affect joints, gut, kidney, lung, and the cardiovascular system. Lung involvement presents with pulmonary infarction, hemoptysis, recurrent pneumonia, bronchiolitis obliterans, organizing pneumonia, and pleurisy. Cardiovascular manifestations include pulmonary artery aneurysms, intracardiac thrombus formation with right-sided predominance, and deep venous thrombosis of large veins. Aneurysmatic changes and thrombosis have been described in many other sites, including gut and brain. The clinical course of Behçet disease is characterized by alternating periods of disease activity and remission. Therapy mainly consists of antiinflammatory and/or immunosuppressive therapy, usually including steroids with or without cyclophosphamide. Because of the risks and benefits, the therapeutic approach to clot formation must be evaluated individually. Mortality is low and usually is the consequence of a thrombotic or hemorrhagic complication.

The possibility of systemic involvement has a significant impact on anesthesia. Chest radiography should be ordered to assess the extent of pulmonary manifestations. Cardiac and large-vessel involvement can be evaluated sonographically and should include the potential sites of central venous catheter insertion. Laboratory ...