Year: 2001
Type: article
Abstract: HistoryBehçet's disease is a chronic inflammatory disorder of unknown aetiology characterised by recurrent attacks.Although the triple symptom complex of oral and genital ulcerations with uveitis was reported by Hippocrates and other authors who attributed the symptom triad to major infections, Hulusi Behçet, a Turkish dermatologist, discarded the association with other illnesses and was the first to delineate the disease that now bears his name. 1 Clinical manifestations additional to this triad were described later including involvement of the skin, joints, large vessels, lung, brain, gastrointestinal and genitourinary tracts (table 1).[2][3][4][5][6] It is now recognised as a multisystem disease with vasculitis as the main pathological finding.[6][7][8][9][10] Diagnosis Since Behçet's disease does not have pathognomonic symptoms or laboratory findings, the diagnosis is made on the basis of the criteria proposed by the International Study Group for Behçet's disease in 1990 (table 2).11 According to the criteria, recurrent oral ulceration must be present and at least two of the following: recurrent genital ulceration, eye lesions, skin lesions, or a positive pathergy test (development of a papule or pustule following a needle prick to the skin, as in table 2). EpidemiologyAlthough Behçet's disease has a worldwide distribution, most cases cluster along the ancient Silk Road which extends from far eastern Asia to the Mediterranean basin (table 3).[12][13][14][15][16][17][18] The highest prevalence rate was reported from Turkey as 80-370 per 100 000.The prevalence ranges from two to 30 cases per 100 000 in other Asian countries, with lower figures in Europe and the USA.The age of disease onset is usually in the second or third decade of life and the male to female ratio is reported to be almost equal.However, the disease runs a more severe course in men and in those with an onset before 25 years of age.[7][8][9][10] Genetic associationSeveral studies have found a strong association between the risk of developing Behçet's disease and HLA-B51.19 20 The relative risk of the disease among carriers of this allele is up to 13.3 in Turkey compared with 1.3 in the USA. 10 21 The association of HLA-B51 positivity with eye disease and a more severe clinical course is controversial.22 Pulmonary involvementMore than 200 cases of Behçet's disease with pulmonary involvement have been reported in the literature.The pulmonary arteries are
Source: Thorax
DOI: 10.1136/thx.56.7.572
Institution: Istanbul University
Language: English
Subfield: Ophthalmology
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W4237163390