46例白塞病的临床资料分析
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摘要
目的探讨白塞病的发病特点、临床表现,提高对白塞病的认识。方法收集中日友好医院从1998年3月10日至2011年3月9日期间入院的诊断为白塞病的46例患者的临床资料,统计分析其发病年龄、临床表现、系统受累、辅助检查的不同性别发生率。结果共有男性患者27例(59%),女性患者19例(41%),男女比率约为1.4：1。平均发病年龄为(31.24±14.34)岁,平均病程为(9.28±9.34)年。各年龄段均可发病,中青年为主35例(76%)。发病年龄不存在性别差异(P>0.05)。首发症状按发生频率由高到低排列为：口腔溃疡37例(80%),生殖器溃疡10例(22%),皮肤损害5例(11%),发热3例(7%),关节炎/关节痛2例(4%)。以两个或两个以上表现起病的患者有10例(22%)。临床表现按发生的频率从高到低依次为：口腔溃疡46例(100%)；生殖器溃疡37例(80%)；皮肤损害35例(76%),其中结节红斑22例(48%),假性毛囊炎/丘疱疹12例(26%),下肢丹毒1例(2%)；针刺反应阳性25例(54%)；关节炎/关节痛23例(50%)；眼部受累20例(43%),其中眼炎9例(20%)；发热17例(37%)；消化系统受累7例(15%)；大血管损害4例(9%)；神经系统损害2例(4%)；附睾炎1例(2%)。皮肤受累患者35例,其中男性26例,女性9例,存在显著统计学差异(P=0.010)。皮损为结节性红斑者22例(48%),假毛囊炎/丘疱疹型患者12例(26%),两者发病均无性别差异。行针刺试验或同等价值损伤的患者29例,阳性25例(54%),其中男性患者20例(43%),女性患者5例(11%),存在显著统计学差异(P=0.002)。其他临床表现发生率在男性女性之间无统计学差异(P>0.05)。5例患者家族中存在类似疾病患者,男性3例,女性2例。其中4例患者的家属有反复口腔溃疡病史,但未诊断白塞病。另有1例患者其姐诊断为干燥综合征。行PPD试验的患者共29例(63%),反应阳性者12例(26%),与性别无明显相关。常规实验室检查中常见的异常指标有：白细胞计数增加(WBC t),红细胞沉降率升高(ESR t)、C反应蛋白增高(CRP t)、抗“0”增高(ASO t)、A/G比例倒置(A/G↓)。按检查结果异常发生频率依次为：ESR增快32例(70%)、CRP升高25例(54%)、A/G下降15例(33%)、WBC增多9例(20%)、ASO升高5例(11%)。各指标正常异常与性别无关。行HLA-AB分型检测的患者15例,2例阳性,男女各1例。行血清免疫学ANA检测患者41例,其中34例正常,7例患者呈弱阳性(5例滴度为1：40,2例滴度为1：80),其中6例为女性。经辨证论治应用中药汤剂的患者为18例(39%)。结论白塞病各年龄均可发病,临床表现多样,可累及多系统,皮肤损害发生和针刺反应阳性存在显著性别差异。
Objective To improve understanding of Behcet's disease, by exploring the clinical characteristic and manifestations.Methods To statistically analyze onset ages, clinical manifestations, system involvement, auxiliary examination, all clinical data of 46 patients who had been hospitalized in China-Japan Friendship Hospital with the diagnosis of Behcet's disease in 13 years was cllcected. Results 46 patients were available for the analysis, in which 27 were males,19 were females with about 1.4:1 ratio. Average onset ages was (31.24±14.34) years. The course duration was (9.28±9.34) years. Behcet's disease occurred in all age groups, the young and middle-aged 35 (76%) patients were primarily,with no difference between men and women patients. The most frequent manifestations at disease onset were oral aphthae (80%) and genital aphthae (22%), followed by skin lesions (11%), fever (7%) and arthralgia/arthritis (4%).22% patients had two or more manifestations at disease onset. The most frequent manifestations were oral aphthae (100%)and genital aphthae (80%), followed by skin lesions (76%), which were described as erythema nodosum(48%), pseudo-folliculitis (26%), and lower limb erysipelas (2%), followed by positive pathergy test (54%), arthralgia/arthritis(50%), inflammatory ocular disorders (43%), fever (37%), digestive system involvement(15%), big vascular impairment(9%), neurological damage(4%) and epididymitis(2%).76% patients presented skin lesions, and most of them were males (57%vs20%, P=0.010).54% patients presented positive pathergy test, and most of them were males (43%vs 11%, P=0.002). No significant sexual difference had been found of other clinical manifestations. Conclusion Behcet's disease occurred in all age groups, multiple organs and systems could be involved, with diversity of clinical manifestations and significant difference of skin lesions and pathergy test between men and women patients.
Objective To improve understanding of Behcet's disease, by exploring the clinical characteristic and manifestations.Methods To statistically analyze onset ages, clinical manifestations, system involvement, auxiliary examination, all clinical data of 46 patients who had been hospitalized in China-Japan Friendship Hospital with the diagnosis of Behcet's disease in 13 years was cllcected. Results 46 patients were available for the analysis, in which 27 were males,19 were females with about 1.4:1 ratio. Average onset ages was (31.24±14.34) years. The course duration was (9.28±9.34) years. Behcet's disease occurred in all age groups, the young and middle-aged 35 (76%) patients were primarily,with no difference between men and women patients. The most frequent manifestations at disease onset were oral aphthae (80%) and genital aphthae (22%), followed by skin lesions (11%), fever (7%) and arthralgia/arthritis (4%).22% patients had two or more manifestations at disease onset. The most frequent manifestations were oral aphthae (100%)and genital aphthae (80%), followed by skin lesions (76%), which were described as erythema nodosum(48%), pseudo-folliculitis (26%), and lower limb erysipelas (2%), followed by positive pathergy test (54%), arthralgia/arthritis(50%), inflammatory ocular disorders (43%), fever (37%), digestive system involvement(15%), big vascular impairment(9%), neurological damage(4%) and epididymitis(2%).76% patients presented skin lesions, and most of them were males (57%vs20%, P=0.010).54% patients presented positive pathergy test, and most of them were males (43%vs 11%, P=0.002). No significant sexual difference had been found of other clinical manifestations. Conclusion Behcet's disease occurred in all age groups, multiple organs and systems could be involved, with diversity of clinical manifestations and significant difference of skin lesions and pathergy test between men and women patients.
引文
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20. 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 Behget's syndrome. nn Rheum Dis,2002,43:783.
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2. Yi D, Shi GY. The synopsis of the 8th International meeting on BD. Natl Med J China,1999,38:135-136.
3.蒋明.风湿病学[M].北京：科学技术出版社,1995：1220.
4. 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,1984,43:783-789.
5. Komiyama Y S, Nakae T, Matsuki A, et al. IL-17 plays an important role in the development of experimental autoimmune encephalomyelit is. [J]JImmunol, 2006,177(1):566-573.
6. Khader S A, Pearl J E, Sakamoto K, et al. IL-23 compensates for the absence of IL-12p70 and is essential for the IL-17 response.during tuberculosis but is dispensable for protection and antigen-specific.IFN-gamma responses if IL-12p70 is available. [J] JImmunol,2005,175 (2):788-795.
7. Yen D J, Cheung H, Scheerens F, et al. IL-23 is essential for T cell-mediated colitis and promotes inflammation via IL-17 and IL-6. [J] J Clin Invest,2006, 116(5):1310-1316.
8. Walther M, Tongren J E, Andrews L, et al. Upregulation of TGF-beta, FOXP3, and CD4+CD25+ regulatory T cells correlates with more rapid parasite growth in human malaria infection. [J] Immunity,2005,23 (3):287-296.
9. Iwakura Y. Ishigame H. The IL-23/IL-17 axis in inflammation. [J]J Clin Invest.2006.116(5):1218-1222.
10. Hofstetter H H, Ibrahim S M, Koczan D, et al.The rapeutic efficacy of IL-17 neutralization in murine experimental autoimmune encephalomyelitis. [J]Cell Immunol,2005,237 (2):123-130.
11. Aggarwal S, Ghilardi N, Xie M H, et al. Interleukin-23 promotes a distinct CD4 T cell activation state characterized by the production of interleukin-17. [J]Biol Chem,2003,278 (3):1910-1914.
12. Veldhoen M, Stockinger B. The link with pro-inflammatory IL-17-Producing T cells. [J] Trends Immunol,2006,27 (8):358-361.
13. Veldhoen M, Hocking R J, Atkins C J, et al. TGFbeta in the context of an inflammatory cytokine milieu supports de novo differentiation of IL-17-producing T cells. [J]Immunity,2006,24 (2):179-189.
14. Mangan P R, Harrington L E, Quinn D B, et al. Transforming growth factor-beta induces development of the Th171ineage. [J]Nature,2006,441(7090):231-234.
15. Starnes T, Robertson M G, Sledge G, et al. Cutting edge:IL-17F, a novel cytokine selectively expressed in activated T cells and monocytes, regulates angiogenesis and endothelial cell cytokine production. [J]J Immunol,2001,167 (8):4137-4140.
16. Zheng S G, Wang J H, Stohl W, et al. GF-beta requires CTLA-4 early after T cell activation to induce FoxP3 and generate adaptive CD4+CD25+ regulatory cells. [J]J Immunol,2006,176 (6):3321-3329.
17.Davatchi F, Shahram F, Gharibdoost F, et al. Behcet's disease. Analysis of 2806 cases. Arthritis Rheum,995,38 (Suppl):391.
18. Bang D, Lee JH, Lee ES, et al. Epidemiologic and clinical surveyof Behcet's disease in Korea:the first multicenter study. [J]Korean Med Sci.2001, 16:615-618.
19. Tursen U, Gurler A, Boyvat A. Evaluation of clinical findings ac-cording to sex in 2313 Turkish patients with Behcet's disease. Int. [J]Dermatol.2003, 42:346-351.
20. 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 Behget's syndrome. nn Rheum Dis,2002,43:783.
21. Park KD, Lee ES, Lee SH, et al. Clinical study on death in Behcet's syndrome. J Korean Med Sci,2004,8:241.
22.李晓明,张智,王雪丽.白塞病53例临床分析[J].陕西医学杂志中,2009,38(7)：905.
23. Schnurr M, Toy Y, Shin A, et al. Extracellular nucleotide signaling by P2 receptors inhibits IL-12 and enhances IL-23 expression in human dendritic cells:a novel role for the cAMP pathway. [J]Blood,2005,105(4):1582-1589.
24.董怡,邱小明,张乃峥等.白塞综合征诊断标准的探讨及针刺反应的临床意义[J].中华内科杂志,1990,29(9)：547-549.
25. Ghate JV. Jorizzojz. Behcet's disease and complexaphtthosis [J]. J A m Alad Dermatol,1999,40(1):1-18.
26. Pirim I, AtasoyM, IkbalM, et al. HLA class Ⅰ and class Ⅱgenoty-ping in patients with Beh et s disease:a regional study of eastern part ofTurkey[J]. TissueAntigens,2004,64(3):293-297.
27. ItohY, InokoH, Kulski JK, et al. Four-digit allele genotyping of theHLA-A and HLA-B genes in Japanese patients with Beh et sdisease by a PCR-SSOP-Luminex method [J]. Tissue Antigens,2006,67(5):390-394.
28. BennaniN, AtoufO, Benseffa jN, et al. HLA polymorphism and Behet's disease inMoroccan population[J]. Pathol Biol (Paris),2009,57(5):403-409.
29.贾汝琳,李茹,李永哲等.间接免疫荧光法检测抗内皮细胞抗体在白塞病诊断中的意义[J].中华风湿病学杂志,2008,12(10)：688-690.
30.0. Shaker, M. A. Ay El-Deen, H. El Hadidi, et al. The role of heat shock protein 60, vascular endothelial growth factor and an-tiphosphplipid antibodies in Behcet disease [J]. British Joumal of Dermatology,2007,15(6):32-37.
31.魏丽,赵岩,杨平等.对白塞病的再认识[J].中国医刊.2003,38(6)：36.
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