Behçet’s syndrome

Hasan Yazici, Sebahattin Yurdakul and Izzet Fresko

in Oxford Textbook of Medicine

Fifth edition

Published on behalf of Oxford University Press

ISBN: 9780199204854
Published online May 2012 | e-ISBN: 9780199570973 | DOI:

Series: Oxford Textbooks

Behçet’s syndrome

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Behçet’s syndrome is an inflammatory disorder of unknown aetiology that involves arteries and veins of all sizes. Most cases are from the countries around the Mediterranean basin, the Middle East and east Asia, with the highest prevalence in Turkey.

Clinical features—the disease typically presents in the second and third decades with recurrent oral ulcers (98% of cases), genital ulcers (85%), acneiform lesions (85%), pathergy reaction (60% in some countries), erythema nodosum (50%), uveitis (50%), arthritis (50%), thrombophlebitis (30%), and less commonly with arterial occlusion/aneurysm, central nervous system involvement or gastrointestinal lesions. A relapsing/remitting course is usual. Disease is more severe and mortality is higher in men. The diagnosis is clinical, laboratory findings are nonspecific and there is no specific diagnostic test for Behçet’s syndrome.

Management and prognosis—elderly people, and women with mild mucocutaneous lesions, can be managed symptomatically. Young people and men need a more aggressive treatment approach, typically as follows (1) mucocutaneous lesions—can be helped by colchicine, thalidomide, and local ointments (i.e. corticosteroids); (2) acute severe eye involvement—ciclosporin with or without steroids, an anti-TNF agent, or solo interferon is the first agent to use, often replaced by azathioprine to maintain remission; (3) thrombophlebitis—typically managed with aspirin and azathioprine; (4) severe vascular disease—cyclophosphamide and steroids is the preferred treatment; (5) parenchymal central nervous system disease—management remains problematic: steroids, immunosuppressives, interferon-α, and tumour necrosis factor α antagonists have all been tried. Major vessel disease and neurological involvement are the main causes of death. About 10–15% of male patients who have eye disease lose useful vision despite treatment.

Chapter.  3904 words.  Illustrated.

Subjects: Rheumatology

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