Yonsei Med J.  2009 Oct;50(5):630-636. 10.3349/ymj.2009.50.5.630.

The Clinical Feature of Behcet's Disease in Northeastern China

• Shang Y
• Han S
• Li J
• Ren Q
• Song F
• Chen H

• KMID: 1103815
• DOI: http://doi.org/10.3349/ymj.2009.50.5.630

Abstract

PURPOSE
Behcet's disease (BD) is a chronic, relapsing, multi-system vasculitis of unknown aetiology with complicated and diversified clinical features predominantly involving oral and genital ulcers, and ocular and cutaneous lesions. The clinical features of this disease have been described to be different according to geographical areas and gender. We investigated the specific clinical features of BD patients in Northeastern China. MATERIALS AND METHODS: 116 patients involved in this study fulfilled the classification criteria of the International Study Group for BD. The clinical manifestations and results of laboratory tests of BD were recorded in each patient. RESULTS: The onset was typically between 20-39 years with a slight female predominance. Oral ulcers were the most common manifestation, followed by skin lesions, positive pathergy reaction/genital ulcers, and ocular lesions. Vascular lesion and epididymitis were rare in patients with BD. The frequency of erythema nodosum-like lesion and articular involvement were significantly higher in females, while gastrointestinal involvement was significantly higher in males. The results of laboratory tests showed that the human leukocyte antigen (HLA)-B*51 alleles were positive in 30.9% of patients and the immunological abnormities were present in some patients. CONCLUSION: The clinical features of BD showed geographical and gender difference. Genetic and immune factors might participate in aetiopathogenesis of BD.

Keyword

Behcet's disease; clinical feature; autoimmune disease

MeSH Terms

Behcet Syndrome/*diagnosis/epidemiology/pathology
China
Female
Geography
Humans
Male

Reference

1. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med. 1999. 341:1284–1291.

2. Kaklamani VG, Vaiopoulos G, Kaklamanis PG. Behçet's disease. Semin Arthritis Rheum. 1998. 27:197–217.

3. Yurdakul S, Hamuryudan V, Yazici H. Behcet syndrome. Curr Opin Rheumatol. 2004. 16:38–42.

4. Krause I, Leibovici L, Guedj D, Molad Y, Uziel Y, Weinberger A. Disease patterns of patients with Behçet's disease demonstrated by factor analysis. Clin Exp Rheumatol. 1999. 17:347–350.

5. Yazici H, Yurdakul S, Hamuryudan V. Behçet's disease. Curr Opin Rheumatol. 2001. 13:18–22.

6. Ghate JV, Jorizzo JL. Behçet's disease and complex aphthosis. J Am Acad Dermatol. 1999. 40:1–18.

7. Chamberlain MA. Behçet's syndrome in 32 patients in Yorkshire. Ann Rheum Dis. 1977. 36:491–499.

8. Chang HK, Kim JW. The clinical features of Behçet's disease in Yongdong districts: analysis of a cohort followed from 1997 to 2001. J Korean Med Sci. 2002. 17:784–789.

9. Yamamoto S, Toyokawa H, Matsubara J. A nationwide survey of Behcet's disease in Japan. Jap J Opthalmol. 1974. 18:282–290.

10. Evereklioglu C. Current concepts in the etiology and treatment of Behçet disease. Surv Ophthalmol. 2005. 50:297–350.

11. Yazici H, Tüzün Y, Pazarli H, Yurdakul S, Ozyazgan Y, Ozdoğan H, et al. Influence of age of onset and patient's sex on the prevalence and severity of manifestations of Behçet's syndrome. Ann Rheum Dis. 1984. 43:783–789.

12. Bang DS, Oh SH, Lee KH, Lee ES, Lee SN. Influence of sex on patients with Behçet's disease in Korea. J Korean Med Sci. 2003. 18:231–235.

13. International Study Group for Behçet's disease. Criteria for diagnosis of Behçet's Disease. Lancet. 1990. 335:1078–1080.

14. Bang D, Lee JH, Lee ES, Lee S, Choi JS, Kim YK, et al. Epidemiologic and clinical survey of Behcet's disease in Korea: the first multicenter study. J Korean Med Sci. 2001. 16:615–618.

15. Nakae K, Masaki F, Hashimoto T, Inaba G, Mochizuki M, Sakane T. Wechsler B, Godeau P, editors. Recent epidemiological features of Behçet's disease in Japan. Behçet's disease. 1993. Amsterdam: Excerpta Medica;145–151.

16. Dai Lie, Tang Meian, Yin Peida, Liu Yaguang. Clinical analysis on 60 case of Behcet's disease. Chin J Rheumatol. 1997. 2:13–16.

17. Liu Xiangyuan, Li Shengguang, Shi Guiying, Huang Cibo, Yuan Guohua, Huang Feng. Comparative study on male and female patients with Behcet's disease. Chin J Rheumatol. 2000. 4:82–84.

18. Chen Lan, Ye Lan, Chen Xinping, Xu Zhusen. A retrospective study on 58 patients with Behcet's disease. Chin J Lepr Skin Dis. 2003. 19:25–27.

19. Gürler A, Boyvat A, Türsen U. Clinical manifestations of Behçet's disease: an analysis of 2147 patients. Yonsei Med J. 1997. 38:423–427.

20. Ozen S. Vasculopathy, Behçet's syndrome, and familial Mediterranean fever. Curr Opin Rheumatol. 1999. 11:393–398.

21. Zouboulis CC, Kötter I, Djawari D, Kirch W, Kohl PK, Ochsendorf FR, et al. Epidemiological features of Adamantiades-Behçet's disease in Germany and in Europe. Yonsei Med J. 1997. 38:411–422.

22. Grenier P, Bletry O, Cornud F, Godeau P, Nahum H. Pulmonary involvement in Behcet disease. AJR Am J Rosentgenol. 1981. 137:565–569.

23. Mizuki N, Inoko H, Mizuki N, Tanaka H, Kera J, Tsuiji K, et al. Human leukocyte antigen serologic and DNA typing of Behçet's disease and its primary association with B51. Invest Ophthalmol Vis Sci. 1992. 33:3332–3340.

24. Verity DH, Wallace GR, Vaughan RW, Kondeatis E, Madanat W, Zureikat H, et al. HLA and tumour necrosis factor (TNF) polymorphisms in ocular Behçet's disease. Tissue Antigens. 1999. 54:264–272.

25. Mizuki N, Ohno S, Ando H, Chen L, Palimeris GD, Stavropoulos-Ghiokas E, et al. A strong association between HLA-B*5101 and Behçet's disease in Greek patients. Tissue Antigens. 1997. 50:57–60.

26. Kötter I, Günaydin I, Stübiger N, Yazici H, Fresko I, Zouboulis CC, et al. Comparative analysis of the association of HLA-B*51 suballeles with Behçet's disease in patients of German and Turkish origin. Tissue Antigens. 2001. 58:166–170.

27. Choukri F, Chakib A, Himmich H, Hüe S, Caillat-Zucman S. HLA-B*51 and B*15 alleles confer predisposition to Behçet's disease in Moroccan patients. Hum Immunol. 2001. 62:180–185.

28. Kilmartin DJ, Finch A, Acheson RW. Primary association of HLA-B51 with Behçet's disease in Ireland. Br J Ophthalmol. 1997. 81:649–653.

29. Krause I, Uziel Y, Guedj D, Mukamel M, Molad Y, Amit M, et al. Mode of presentation and multisystem involvement in Behçet's disease: the influence of sex and age of disease onset. J Rheumatol. 1998. 25:1566–1569.