白塞病173例的临床特点分析

Clinical analysis for 173 cases of Behcet's disease

目的总结白塞病患者的临床特点,提高对白塞病的认识。方法收集北京协和医院1983年1月至2004年6月确诊白塞病的173例患者的临床资料,回顾性分析其临床表现、系统受累及在不同性别的发生率和发生时间差异。结果平均发病年龄为(28±12)岁,病程为5(2～10)年。173例患者中,口腔溃疡是最常见的临床表现(173例,100%),其后依次为外阴溃疡(128例,74.0%)和眼部受累(91例,52.6%)。HLAB51阳性率为41%(16/39),针刺反应阳性率为60.0%(102/173)。口腔溃疡为最常见的首发表现(119例,68.2%),其后依次为生殖器溃疡(47例,27.2%)、结节红斑(28例,16.2%)。白塞病可以导致重要脏器的病变,眼部症状发生在首发症状后平均1(0～7)年,中枢神经系统症状在4(2～6)年,消化系统症状在4(1～7)年,大血管症状在3(0.6～4)年。临床表现存在显著性别差异的有男性大血管受累较多(OR=10.6,P<0.01)、女性血液系统受累较多(OR=0.094,P<0.05)。结论白塞病的临床表现多种多样,可累及多个系统,存在性别差异。

Objective To sum up clinical characteristics of in-patients with Behcet＇ s disease,so to improve our knowledge about it. Methods Clinical data of 173 cases with Behcet＇ s disease admitted to Peking Union Medical College Hospital during January 1983 to June 2004 were collected. Their clinical manifestations were analyzed retrospectively, including its prevalence by gender and time, and involvement in the varied organs and systems. Results Mean age at onset of the 173 patients was （28 ± 12）years, with an average length of clinical course of 5 （2 - 10） years. Oral ulcer （aphtha） was found in all the 173 （ 100% ） patients,and then followed by genital ulcer in 128 （74. 0% ） cases, and ophthalmologic lesions in 91 （52. 6% ） cases,in a descending order. Sixteen of 39 cases were positive in HLA-B51 （41%） and 102 of 173 （60. 0% ） cases were positive for pathergy test. Oral ulcer （aphtha） was the initial sign found in 119 of 173 （68. 2% ） cases, and then followed by genital ulcer in 47 case （27. 2% ） ,and erythema nodosum in 28 （ 16. 2% ） cases. Pathological changes in several important organs could be caused by Behcet＇ s disease. Ophthalmologie lesions occurred in one year, symptoms of the central nervous system occurred in 4 （ 2 - 6） years,symptoms of gastroenterological impairment in 4 （1 - 7 ） years, and symptoms of large vessels in 3 （0. 6 - 4） years after onset of the initial sign in average. Significant difference in clinical manifestations between men and women patients could be seen, with more involvements in large vessels in men than in women （ OR = 10.6, P 〈 0. 01 ）, and more involvements in hematological in women than in men （ OR = 0. 094,P 〈 0. 05）. Conclusion Multiple organs and systems could be involved in Beheet＇ s disease with diversity of clinical manifestations and significant difference between men and women patients.