白塞病大血管病变的临床特点分析

Clinical features of large vessel involvements in Behcet＇s disease

目的探讨白塞病大血管病变的临床特点。方法回顾性分析1991年1月至2011年12月在首都医科大学附属北京安贞医院和北京大学人民医院风湿免疫科、血管外科、眼科、皮肤科、消化科及心内科住院的141例白塞病患者的临床资料。根据有无大血管病变，将2组患者分为大血管病变组（36例）和无大血管病变组（105例）。记录和比较2组患者基本资料和实验室检查结果，并对患者血管受累特点进行分析。结果大血管病变组男性、接受外科手术、应用环磷酰胺、神经系统受累、C反应蛋白升高和抗核抗体阳性比例均明显高于无大血管病变组[44．4％（16／36）比33．3％（35／105）；33．3％（12／36）比0．0％（0／105）；36．1％（13／36）比11．4％（12／105）；30．6％（11／36）比8．6％（9／105）；55．2％（16／29）比32．6％（28／86）；24．0％（6／25）比6．0％（5／84）]，白细胞计数和中性粒细胞计数均明显高于无大血管病变组[8（7，11）×10^9／L比7（5，10）×10^9／L、6．5（4．8，9．6）×10^9／L比4．5（3．0，6．3）×10^9／L]，而应用非甾体消炎药比例明显低于无大血管病变组[13．9％（5／36）比42．9％（45／105）]，差异均有统计学意义（均P〈0．05）。动脉病变患者比例为61．1％（22／36），其中最常受累部位为颅内动脉22．2％（8／36），女性比例明显高于男性[57．1％（4／7）比13．8％（4／29）]，差异有统计学意义（P〈0．05）；动脉病变受累性质最常表现为真性／假性动脉瘤36．1％（13／36），且均发于男性44．8％（13／29）。女性动脉血栓／栓塞发生率明显高于男性[85．7％（6／7）比20．7％（6／29）]，差异有统计学意义（P〈0．05）。静脉病变患者比例为52．8％（19／36），其中最常受累部位为下肢静脉33．3％（12／36）；静脉病变受累性质主要表现为静脉血栓形成41．7％（15／36）。结论白塞病大血管病变男女有别，白细胞计数和中性粒细胞计数明显升高，颅内动脉和下肢静脉常受累，常表现为真性／假性动脉瘤和静脉血栓形成的临床特点，对疾病的早期诊断、早期治疗具有非常重要的意义。

Objective To explore clinical characteristics of large vessel involvements in patients with Behcet＇s disease（BD）. Methods Totally 141 patients with BD from January 1991 to December 2011 in Beijing Anzhen Hospital, Capital Medical University and Peking University People＇s Hospital were retrospectively enrolled; 36 patients had large vascular lesions. General information and laboratory examination results were recorded. Features of vascular lesions were analyzed. Results Constituent ratios of male, surgical operation, cyclophosphamide application, nervous system impairment, C-reactive protein increasing and antinuclear antibody positive in large vascular lesion group（36 cases） were significantly higher than those in non-large vascular lesion group（ 105 cases） [44. 4% （16/36） vs 33. 3% （35/105）, 33. 3% （12/36） vs 0. 0% （0/105）, 36. 1% （13/36） vs 11.4% （ 12/105）, 30. 6% （11/36） vs 8. 6% （9/105）, 55. 2% （16/29） vs 32. 6% （28/86）, 24. 0% （6/25） vs 6. 0% （5/84） 1 （ P 〈 0. 05 ） ; leukocyte count and neutrophil count in large vascular lesion group were significantly higher than those in non-large vascular lesion group[8（7,11 ）×10^9/L vs 7（5,10） ×10^9/L, 6. 5（4. 8,9. 6） ×10^9/L vs 4. 5 （3.0,6. 3 ） ×10^9/L] （ P 〈 O. 05 ） ; constituent ratio of non-steroid anti-inflammatory drug application in large vascular lesion group was significantly lower than that in non-large vascular lesion group[ 13.9% （5/36） vs 42.9% （45/105） ] （P 〈 0. 05）. Twenty-two patients （61.1%） had arterial lesions; intracranial artery was the main involvement [ 22. 2% （8/36） ] ; the proportion of arterial lesions in female was significantly higher than that in male [57. 1% （4/36） vs 13.8% （ 4/36 ） ] （ P 〈 0. 05 ） ; true/false aneurysm accounted for the largest proportion [ 36. 1% （ 13/36 ） ] and all occurred in male [ 44. 8 % （13/29） ]. The incidence of arterial thrombosis/embolism in female was significantly higher than that in male[85.7% （6/7） vs 20. 7% （6/29） ] （P 〈0. 05）. Nineteen patients （52. 8% ） had venous lesions; lower limb vein was the main involvement [ 33.3% （12/36） ] ; venous thrombus accounted for the largest proportion[41.7% （15/36） ]. Conclusion Clinical of large vascular lesions of Behcet＇s disease including different characteristics of men and women, increasing white blood cell count and neutrophil count, frequently involved the intracranial artery and venous, often expressed as a true / false aneurysm and venous thrombosis has significance for diagnosis and treatment of the disease.