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Chapter-20 Behçet's Disease

BOOK TITLE: Uveitis: Text and Imaging

Author
1. Tutkun Ilknur Tugal
ISBN
9788184484915
DOI
10.5005/jp/books/11002_20
Edition
1/e
Publishing Year
2009
Pages
17
Author Affiliations
1. Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey
Chapter keywords
Behçet’s disease, Mucocutaneous manifestations, retinal periphlebitis, Behçet uveitis, fluorescein angiography, haemorrhagic vasculitis, macular ischaemia, cystoid macular oedema, retinal vessels, Gliotic sheathing, inflammatory cells

Abstract

Behçet’s disease (BD) is a multisystem inflammatory disorder named after Dr Hulusi Behçet, a Turkish professor of dermatology, who described the triplesymptom complex of recurrent oral ulcers, genital ulcers and iritis as a distinct entity in 1937. Although Behçet’s disease is a universal disorder it is much more common in the Mediterranean area, the Middle East and the Far East. Behçet’s disease has been shown to be associated with the HLA-B51 antigen especially in the ethnic groups along the ancient silk trading route extending from the Far East to the Mediterranean basin. Dissemination of this antigen by the nomadic tribes has been blamed for the introduction of the disease in the Asian and Eurasian populations. Behçet’s disease primarily affects young adults. The typical age of onset is in the third decade of life. Behçet patients may present with various symptoms depending on the type and severity of the organ systems involved. Because the disease is characterised by a spontaneously relapsing and remitting course, a comprehensive history should be obtained in order to reveal all previous manifestations. Mucocutaneous manifestations are the predominant clinical feature of the disease. The eye is the most commonly involved vital organ in Behçet’s disease and the typical form of involvement is a relapsing remitting uveitis. The natural course of the disease is characterised by explosive uveitis attacks and spontaneous remissions. Uveitis in Behçet’s disease is always nongranulomatous and eventually involves both anterior and posterior segments of the eye bilaterally in most of the patients. Maculopathy is the most common cause of visual morbidity in Behçet’s disease resulting from diffuse or cystoid macular oedema, infiltration with inflammatory cells, haemorrhagic vasculitis, or macular ischaemia. Gliotic sheathing of retinal vessels is a typical sequela of retinal periphlebitis. As retinal vasculitic changes constitute the main pathology in ocular involvement of Behçet’s disease, fluorescein angiography is a useful tool in the early detection, follow-up, and monitoring therapy of Behçet uveitis.

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