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. 2000 Aug;59(8):622–625. doi: 10.1136/ard.59.8.622

Familial aggregation of Behçet's disease in Turkey

A Gul 1, M Inanc 1, L Ocal 1, O Aral 1, M Konice 1
PMCID: PMC1753203  PMID: 10913059

Abstract

OBJECTIVE—Familial aggregation of Behçet's disease has been reported previously. The current study aimed at investigating the sibling recurrence risk ratio (λs) 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—170 consecutive 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 affected among the siblings of patients and the risk of being affected in the general population— was found to be 4.2%, which gives a λs value for Behçet's disease of between 11.4 and 52.5 in Turkey.
CONCLUSIONS—A high λs value supports a strong genetic background for Behçet's disease which will be helpful in designing genetic linkage studies.



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Selected References

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  1. Akpolat T., Koç Y., Yeniay I., Akpek G., Güllü I., Kansu E., Kiraz S., Ersoy F., Batman F., Kansu T. Familial Behçet's disease. Eur J Med. 1992 Nov;1(7):391–395. [PubMed] [Google Scholar]
  2. Arber N., Klein T., Meiner Z., Pras E., Weinberger A. Close association of HLA-B51 and B52 in Israeli patients with Behçet's syndrome. Ann Rheum Dis. 1991 Jun;50(6):351–353. doi: 10.1136/ard.50.6.351. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Bird Stewart J. A. Genetic analysis of families of patients with Behçet's syndrome: data incompatible with autosomal recessive inheritance. Ann Rheum Dis. 1986 Apr;45(4):265–268. doi: 10.1136/ard.45.4.265. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Brown M. A., Pile K. D., Kennedy L. G., Campbell D., Andrew L., March R., Shatford J. L., Weeks D. E., Calin A., Wordsworth B. P. A genome-wide screen for susceptibility loci in ankylosing spondylitis. Arthritis Rheum. 1998 Apr;41(4):588–595. doi: 10.1002/1529-0131(199804)41:4<588::AID-ART5>3.0.CO;2-0. [DOI] [PubMed] [Google Scholar]
  5. Chamberlain M. A. A family study of Behcet's syndrome. Ann Rheum Dis. 1978 Oct;37(5):459–465. doi: 10.1136/ard.37.5.459. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Dündar S. V., Gençalp U., Simşek H. Familial cases of Behcet's disease. Br J Dermatol. 1985 Sep;113(3):319–321. doi: 10.1111/j.1365-2133.1985.tb02084.x. [DOI] [PubMed] [Google Scholar]
  7. Fresko I., Soy M., Hamuryudan V., Yurdakul S., Yavuz S., Tümer Z., Yazici H. Genetic anticipation in Behçet's syndrome. Ann Rheum Dis. 1998 Jan;57(1):45–48. doi: 10.1136/ard.57.1.45. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Guo S. W. Inflation of sibling recurrence-risk ratio, due to ascertainment bias and/or overreporting. Am J Hum Genet. 1998 Jul;63(1):252–258. doi: 10.1086/301928. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Huang Y., Hall I. H. Synthesis and hypolipidemic evaluation of beta-alkylaminopropiophenone and beta-alkylaminopropio-2'-naphthone derivatives in rodents. Pharmazie. 1996 Apr;51(4):199–206. [PubMed] [Google Scholar]
  10. Keane J. R. Neuro-ophthalmologic signs and symptoms of cysticercosis. Arch Ophthalmol. 1982 Sep;100(9):1445–1448. doi: 10.1001/archopht.1982.01030040423010. [DOI] [PubMed] [Google Scholar]
  11. Koné-Paut I., Geisler I., Wechsler B., Ozen S., Ozdogan H., Rozenbaum M., Touitou I. Familial aggregation in Behçet's disease: high frequency in siblings and parents of pediatric probands. J Pediatr. 1999 Jul;135(1):89–93. doi: 10.1016/s0022-3476(99)70333-1. [DOI] [PubMed] [Google Scholar]
  12. Mizuki N., Inoko H., Ohno S. Molecular genetics (HLA) of Behçet's disease. Yonsei Med J. 1997 Dec;38(6):333–349. doi: 10.3349/ymj.1997.38.6.333. [DOI] [PubMed] [Google Scholar]
  13. Nishiura K., Kotake S., Ichiishi A., Matsuda H. Familial occurrence of Behçet's disease. Jpn J Ophthalmol. 1996;40(2):255–259. [PubMed] [Google Scholar]
  14. Risch N. Assessing the role of HLA-linked and unlinked determinants of disease. Am J Hum Genet. 1987 Jan;40(1):1–14. [PMC free article] [PubMed] [Google Scholar]
  15. Sakane T., Suzuki N., Nagafuchi H. Etiopathology of Behçet's disease: immunological aspects. Yonsei Med J. 1997 Dec;38(6):350–358. doi: 10.3349/ymj.1997.38.6.350. [DOI] [PubMed] [Google Scholar]
  16. Tüzün Y., Yurdakul S., Cem Mat M., Ozyazgan Y., Hamuryudan V., Tüzün B., Yazici H. Epidemiology of Behçet's syndrome in Turkey. Int J Dermatol. 1996 Sep;35(9):618–620. doi: 10.1111/j.1365-4362.1996.tb03683.x. [DOI] [PubMed] [Google Scholar]
  17. Weeks D. E., Lathrop G. M. Polygenic disease: methods for mapping complex disease traits. Trends Genet. 1995 Dec;11(12):513–519. doi: 10.1016/s0168-9525(00)89163-5. [DOI] [PubMed] [Google Scholar]
  18. Zouboulis C. C., Kötter I., Djawari D., Kirch W., Kohl P. K., Ochsendorf F. R., Keitel W., Stadler R., Wollina U., Proksch E. Epidemiological features of Adamantiades-Behçet's disease in Germany and in Europe. Yonsei Med J. 1997 Dec;38(6):411–422. doi: 10.3349/ymj.1997.38.6.411. [DOI] [PubMed] [Google Scholar]

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