Familial aggregation of Behcet's disease in Turkey

Abstract

Objective-Familial aggregation of Behçet's disease has been reported previously. The current study aimed at investigating the sibling recurrence risk ratio ( s) for Behçet's disease, which is of value in the estimation of the magnitude of genetic factors in the pathogenesis of Behçet's disease. Methods-170consecutive unrelated index cases (98 male, 72 female) were interviewed with a detailed questionnaire to ascertain their family trees and the manifestations of Behçet's disease in their relatives. Subsequently, the immediately older sibling, or if an older sibling was not available, the immediately younger sibling, was selected as the second sibling for the evaluation. These siblings were contacted by telephone, and all subjects with recurrent oral ulcers were invited for examination. Results-31 of the 170 index cases had 51 relatives fulfilling the International Study Group criteria. Among 166 second siblings, seven had Behçet's disease (six male, one female) and 22 siblings (eight male, 14 female) with recurrent oral ulcers were identified. Sibling recurrence rate-defined as the ratio of the risk of being aVected among the siblings of patients and the risk of being aVected in the general population-was found to be 4.2%, which gives a s value for Behçet's disease of between 11.4 and 52.5 in Turkey. Conclusions-A high s value supports a strong genetic background for Behçet's disease which will be helpful in designing genetic linkage studies.

Ocular involvement is associated with HLA-B51 in Adamantiades–Behçet's disease

“…The prevalence of familial ABD is in the range of 1-18%. 8,9,[13][14][15][16][17][18][19] In a study from Turkey, the risk of disease in sisters was 11.4-52.5. 19 Among different genetic markers, the class I HLA-B5 and -B51 gene on chromosome 6p21 is in association with ABD.…”

“…8,9,[13][14][15][16][17][18][19] In a study from Turkey, the risk of disease in sisters was 11.4-52.5. 19 Among different genetic markers, the class I HLA-B5 and -B51 gene on chromosome 6p21 is in association with ABD. 20,21 The relative risk of ABD in HLA-B51-positive patients is 13.3 for Turkey, 6.7 for Japan, and 1.3 for the United States of America.…”

Investigations of genetic polymorphisms related to Behcet\s disease in Turkish population

“…Bunun yanında ailesel olgular (4,5) ve hastalığın HLA-B*51 ile ilişkili olması hastalığın patojenezinde genetik faktörlerin önemli rolü olduğunu ortaya koymuştur (6). Bununla birlikte Behçet hastalarının %60'tan fazla bir oranda HLA-B51 ilişkili olmasına rağmen bu ilişkinin hastalığa olan genetik yatkınlıktaki rolünün %20 civarında olması HLAB-51'in hastalığın mekanizmasını tek başına açıklayamayacağını ve BH'ye yatkınlıkta bazı başka genlerin de sorumlu olabileceğini düşündürmüştür (7)(8)(9).…”

Is Behçet's disease a ‘class 1-opathy’? The role of HLA-B*51 in the pathogenesis of Behçet's disease

“…This was quantified for a Turkish population by G€ ul et al [17], showing a sibling recurrence risk ratio (ks) of between 11Á4 and 52Á5 in different populations within Turkey. Hence, although the inheritance of BD does not follow a simple Mendelian pattern, there appears to be a genetic link with a ks higher than some other genetically complex diseases, such as rheumatoid arthritis (ks 5 6) [18] and type I diabetes mellitus (ks 5 15) [19].…”