TÜRK BEHÇET HASTALARININ CİNSİYET, HLA-B51 VE PATERJİ TESTİNE GÖRE KLİNİK ÖZELLİKLERİNİN DEĞERLENDİRİLMESİ

Amaç: Behçet Hastalığı’nın (BH) klinik özellikleri ülkeye ve ırkagöre farklılık gösterebilmektedir. Cinsiyet, paterji testi ve HLA B51de BH’nin klinik özelliklerini etkileyebilmektedir. Bu çalışmanınamacı; cinsiyet, HLA B51 ve paterji testinin Türk Behçet hastalarınınkliniğine etkisini değerlendirmektir.Gereç ve Yöntem: Uluslararası Çalışma Grubu kriterlerinegöre BH tanısı almış 97 Türk hasta (59 erkek, 38 kadın) retrospektifolarak değerlendirildi.Bulgular: Ortalama yaş 36,2 ± 11,1 yıl idi. Medyan(ÇAA:çeyreklerarası aralık) tanı yaşı, hastalık süresi ve tanıdagecikme yaşı; 26 (21-35), 7 (3-11) ve 4 (1-9) yıl idi. Oral aftözülserler en sık gözlenen bulguydu (%100). Oral ülserleri, genitalülserler (%67), cilt bulguları (%54,6), oküler (%51,5) ve vasküler(%24,7) tutulum, artrit (%23,7) ve nörolojik tutulum (%14,4)izlemekteydi. HLA B51 ve paterji testi, hastaların %70,1’inde ve%39,2’sinde pozitifti. Genital ülser, cilt bulguları, artiküler tutulumve HLA B51 pozitifliği erkek ve kadın cinsiyette benzerdi. Paterjipozitifliği, oküler ve vasküler tutulum erkeklerde daha fazlaydı. Ciltbulguları, paterji pozitifhastalarda daha azgözlendi.Sonuç:Paterji pozitifliği, vasküler ve oküler tutulum cinsiyete göre,cilt bulguları ise paterji pozitifliğine göre farklılık gösterebilmektedir.HLA B51 pozitifliğinin klinik özellikler üzerinde belirgin etkisibulunmamaktadır.

CLINICAL EVALUATION OF TURKISH PATIENTS WITH BEHÇET DISEASE ACCORDING TO SEX, HLA B51 POSITIVITY AND PATHERGY TEST

PURPOSE: The clinical features of Behçet disease (BD) differ according to race and country. Gender, pathergy test and HLA B51 also have impact on clinical manifestations of BD. The aim of this study is to evaluate the effect of gender, HLA B51 and pathergy test on the clinical features of Turkish patients with BD. MaterIal and Method: Ninety-seven Turkish patients (59 males, 38 females) fulfilling the International Study Group criteria for Behçet Disease were evaluated retrospectively. Results: The mean age was 36.2 ± 11.1 years. The median (IQR: interquartile range) age at diagnosis, duration of disease and delay in diagnosis were 26 (21-35), 7 (3-11) and 4 (1-9) years, respectively. Oral aphtous ulcers were the most common manifestation (100%), followed by genital ulcers (%67), skin findings (54.6%), ocular (51.5%) and vascular (24.7%) involvement, arthritis (23.7%) and neurologic involvement (14.4%). HLA B51 and pathergy test were positive in 70.1% and 39.2% of patients, respectively. Genital ulcer, skin findings and articular involvement and HLAB51 positivity were similar between male and female gender. Pathergy positivity, ocular and vascular involvement were more common in male gender. Skin findings were less common in pathergy positive patients. ConclusIon: Pathergy positivity, vascular and ocular involvement may differ according to gender, while skin findings may differ according to pathergy positivity. HLA B51 positivity had no significant impact on clinical features.

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1.)Tursen U, Gurler A, Boyvat A. Evaluation of clinical findings according to sex in 2313 Turkish patients with Behcet's disease. Int J Dermatol. 2003;42(5):346-51.
2.)Verity DH, Marr JE, Ohno S, Wallace GR, Stanford MR. Behcet's disease, the Silk Road and HLA-B51: historical and geographical perspectives. Tissue Antigens. 1999;54(3):213-20.
3.)Onder M, Gurer MA. The multiple faces of Behcet's disease and its aetiological factors. J Eur Acad Dermatol Venereol. 2001;15(2):126-36.
4.)Davatchi F, Shahram F, Chams-Davatchi C, et al. Behcet's disease: from East to West. Clin Rheumatol. 2010;29(8):823-33.
5.)Wang LY, Zhao DB, Gu J, Dai SM. Clinical characteristics of Behcet's disease in China. Rheumatol Int. 2010;30(9):1191-6.
6.)Sakane T, Takeno M, Suzuki N, Inaba G. Behcet's disease. N Engl J Med. 1999;341(17):1284-91.
7.)Bang DS, Oh SH, Lee KH, Lee ES, Lee SN. Influence of sex on patients with Behcet's disease in Korea. J Korean Med Sci. 2003;18(2):231-5.
8.)Hamzaoui A, Houman MH, Massouadia M, et al. Contribution of Hla-B51 in the susceptibility and specific clinical features of Behcet's disease in Tunisian patients. Eur J Intern Med. 2012;23(4):347-9.
9.)Assar S, Sadeghi B, Davatchi F, et al. The association of pathergy reaction and active clinical presentations of Behcet's disease. Reumatologia. 2017;55(2):79-83.
10.)Ryu HJ, Seo MR, Choi HJ, Baek HJ. Clinical phenotypes of Korean patients with Behcet disease according to gender, age at onset, and HLA-B51. Korean J Intern Med. 2018;33(5):1025-31.
11.)Criteria for diagnosis of Behcet's disease. International Study Group for Behcet's Disease. Lancet. 1990;335(8697):1078-80.
12.)Kalra S, Silman A, Akman-Demir G, et al. Diagnosis and management of Neuro-Behcet's disease: international consensus recommendations. J Neurol. 2014;261(9):1662-76.
13.)Yazici H, Tuzun Y, Pazarli H, et al. Influence of age of onset and patient's sex on the prevalence and severity of manifestations of Behcet's syndrome. Ann Rheum Dis. 1984;43(6):783-9.
14.)Balta I, Akbay G, Kalkan G, Eksioglu M. Demographic and clinical features of 521 Turkish patients with Behcet's disease. Int J Dermatol. 2014;53(5):564-9.
15.)Bonitsis NG, Luong Nguyen LB, LaValley MP, et al. Genderspecific differences in Adamantiades-Behcet's disease manifestations: an analysis of the German registry and meta-analysis of data from the literature. Rheumatology (Oxford). 2015;54(1):121-33.
16.)Yavuz S, Ozilhan G, Elbir Y, Tolunay A, Eksioglu-Demiralp E, Direskeneli H. Activation of neutrophils by testosterone in Behcet's disease. Clin Exp Rheumatol. 2007;25(4 Suppl 45):S46-51.
17.)Atasoy M, Karatay S, Yildirim K, Kadi M, Erdem T, Senel K. The relationship between serum prolactin levels and disease activity in patients with Behcet's disease. Cell Biochem Funct. 2006;24(4):353-6.
18.)Guzelant G, Ozguler Y, Esatoglu SN, et al. Exacerbation of Behcet's syndrome and familial Mediterranean fever with menstruation. Clin Exp Rheumatol. 2017;35 Suppl 108(6):95-9.
19.)de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A. HLA-B51/B5 and the risk of Behcet's disease: a systematic review and meta-analysis of case-control genetic association studies. Arthritis Rheum. 2009;61(10):1287-96.
20.)Chajek-Shaul T, Pisanty S, Knobler H, et al. HLA-B51 may serve as an immunogenetic marker for a subgroup of patients with Behcet's syndrome. Am J Med. 1987;83(4):666-72.
21.)Sensi A, Gavioli R, Spisani S, et al. HLA B51 antigen associated with neutrophil hyper-reactivity. Dis Markers. 1991;9(6):327-31.
22.)Mizuki N, Inoko H, Ohno S. Molecular genetics (HLA) of Behcet's disease. Yonsei Med J. 1997;38(6):333-49.
23.)Kaya TI, Dur H, Tursen U, Gurler A. Association of class I HLA antigens with the clinical manifestations of Turkish patients with Behcet's disease. Clin Exp Dermatol. 2002;27(6):498-501.
24.)Gul A, Uyar FA, Inanc M, et al. Lack of association of HLA-B*51 with a severe disease course in Behcet's disease. Rheumatology (Oxford). 2001;40(6):668-72.
25.)Demirseren DD, Ceylan GG, Akoglu G, et al. HLA-B51 subtypes in Turkish patients with Behcet's disease and their correlation with clinical manifestations. Genet Mol Res. 2014;13(3):4788-96.
26.)Yazici H, Tuzun Y, Pazarli H, Yalcin B, Yurdakul S, Muftuoglu A. The combined use of HLA-B5 and the pathergy test as diagnostic markers of Behcet's disease in Turkey. J Rheumatol. 1980;7(2):206-10.
27.)Nishiyama M NK, Takahashi M, Kuriyama T. A study of clinical features and the worsening factors among patients with Behcet’s disease in Japan. Dokkyo J Med Sci. 2003(30):237-42.
28.)Davatchi F, Shahram F, Chams-Davatchi C, et al. Behcet's disease in Iran: analysis of 6500 cases. Int J Rheum Dis. 2010;13(4):367-73.
29.)Krause I, Molad Y, Mitrani M, Weinberger A. Pathergy reaction in Behcet's disease: lack of correlation with mucocutaneous manifestations and systemic disease expression. Clin Exp Rheumatol. 2000;18(1):71-4.
30.)Alpsoy E, Aktekin M, Er H, Durusoy C, Yilmaz E. A randomized, controlled and blinded study of papulopustular lesions in Turkish Behcet's patients. Int J Dermatol. 1998;37(11):839-42.