Show Summary Details
Page of

Behçet’s syndrome 

Behçet’s syndrome

Chapter:
Behçet’s syndrome
Author(s):

Hasan Yazici

, Sebahattin Yurdakul

, and Izzet Fresko

DOI:
10.1093/med/9780199204854.003.191105_update_002

Update:

Aetiology—description of new susceptibility loci.

Updated on 27 Feb 2014. The previous version of this content can be found here.
Page of

PRINTED FROM OXFORD MEDICINE ONLINE (www.oxfordmedicine.com). © Oxford University Press, 2015. All Rights Reserved. Under the terms of the licence agreement, an individual user may print out a PDF of a single chapter of a title in Oxford Medicine Online for personal use (for details see Privacy Policy).

date: 25 March 2017

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.

Access to the complete content on Oxford Medicine Online requires a subscription or purchase. Public users are able to search the site and view the abstracts for each book and chapter without a subscription.

Please subscribe or login to access full text content.

If you have purchased a print title that contains an access token, please see the token for information about how to register your code.

For questions on access or troubleshooting, please check our FAQs, and if you can''t find the answer there, please contact us.