白塞病64例临床分析

Analysis of Clinical Data of 64 Cases of Behcet's Disease

目的了解昆明地区白塞病患者的临床特征，并与其他地区以及其他国家进行横向比较，从而全面的了解白塞病，为临床医生提供参考依据。方法参考2005年国际白塞病诊断标准，对本院2000年1月--2014年6月收入院治疗的64例白塞病患者的临床资料进行回顾性分析，并与国内及国际其他地区同类研究资料进行比较。结果64例白塞病患者中，男：女为1：2．2，好发年龄30—40岁，与其他地区及国家一致，平均发病年龄38．57±14．58岁，平均病程6．18±7．25年。完全型6例（9．38％），不完全型58例（90．63％）。临床表现：口腔溃疡62例（96．88％），低于新加坡、土耳其（100％）；皮肤损害44例（68．75％），低于韩国（84．28％）；生殖器溃疡28例（43．75％），低于土耳其（88．22％）；关节病损14例（21．88％）、胃肠道损害11例（17．18％）、发热9例（14．06％）、眼部病损7例（10．94％）、血管病变6例（9．37％）、肾损害5例（7．81％）等上述器官损害与其他国家、地区大体一致。64例患者中有26例接受皮肤针刺反应检查，阳性率60．94％（男50．00％，女65．91％），略高于其他国家及地区。46例（71．87％）予中小剂量糖皮质激素治疗，其中有18例联合免疫抑制剂治疗。64例患者均好转出院。结论白塞病临床表现存在地域差异，其临床特点、发病规律大体保持一致，本组资料中白塞病患者血管病变发生率高，眼部病变及关节病变发生率低。早期针刺反应检查可提高本病早期诊断率。早期予以糖皮质激素和免疫抑制剂联合治疗可缓解本病的症状，改善预后。

Objective To determine the clinical features of 64 cases of Behcet＇s disease （BD） in our hospital over the last 14 years, and to compare them with other regions of the world. Methods According the International Study Group criteria for the diagnosis of Behcet＇s syndrome, clinical data of 64 cases of BD were retrospectively analyzed and compared with others reported previously. Results The prevalence of BD in our region was similar to other regions of the world. The ratio of males to females was 1： 2.2. Majority of them were 30 -40 years old with a mean onset age of 38.57 ±14.58 years. The average disease course was 6.18 ±7.25 years. Com- plete type of BD was 6 cases （9.38%） while 58 cases （90.63%） were incomplete type. Oral ulcers occurred in 62 cases （96.88%）, which was slightly lower than that in Singapore and Turkey （ 100% in both countries）. Skin involvement was less common in our region （44 cases,68.75% ） than in Korea （84.28%）. The incidence of genital ulcers was lower in our region than in Turkey （28 case, 43.75% vs. 88.22% in Tur- key）. The incidence of other body site involvement was similar to other regions and countries （joint 14 cases, 21.88%; gastrointestinell cases, 17.18%; fever 9 cases, 14.06%; ocular lesion 7 cases, 10.94%; vascu- lar involvement 6 cases, 9.37% ; renal damage 5 cases, 7.81% ）. 26/64 cases （60.94 % ）, including 50% males and 65.91% females, showed positive for skin pathergy test, which was slightly higher than other regions and countries. 46 cases （71.87%） were treated with low dose of glucocorticoids, and 18 cases were given both glucocorticoids and immunosuppressant drugs. All patients were improved and discharged. Conclusions Generally, the clinical features of BD do not vary dramatically with regions or nations. Our patients have a higher incidence of vascular involvement, but low incidence of ocular and joint damages. Early pathergy test is helpful for early diagnosis. Promptly given glucocorticoids and immunosuppressant drugs can improve clinical symptoms and prognosis.