重型ANCA相关小血管炎临床特征分析  

The Clincal Characteristic Analyze of Sever ANCA Realted Vasculitis

作　　者：费锦萍[1] 邬秀娣[1] 

机构地区：[1]宁波市第二医院,浙江宁波315010

出　　处：《现代实用医学》2008年第6期422-424,共3页Modern Practical Medicine

摘　　要：目的探讨重型抗中性粒细胞胞浆抗体(ANCA)相关小血管炎的临床表现,以提高诊断水平。方法对16例ANCA相关小血管炎患者的临床及病理资料进行分析总结,系统描述全身特别是肾外临床表现。结果16例中男11例,女5例,年龄26～66岁,95%患者在50岁以后发病。胞浆型抗中性粒细胞胞浆抗体(C-ANCA)13例,均识别蛋白酶3(PR3),环核型抗中性粒细胞胞浆抗体(pANCA)阳性3例,8例识别髓过氧化物酶(MPO)。临床上表现为多器官损害,肾脏受累占96.4%,约半数表现为急性肾功能衰竭;肺脏受累占75%;47.6%有关节痛;35.7%有肌肉痛;38.1%有皮疹;26.2%眼受累;16.7%耳受累;28.6%有消化道症状。全身表现中发热占70.8%、体重下降占31.0%。实验室检查81.6%的患者有贫血,多为中重度贫血;半数患者WBC计数增高;100%患者有血沉快。本研究中仅不足1/5患者在发病1个月内得到确诊。结论ANCA相关小血管炎临床呈多系统损害,肾脏最易受累,肾外以肺脏为著,多表现为肺出血或肺部阴影。其它脏器如关节肌肉、皮肤、眼耳等均可受累。ANCA相关小血管炎患者多有贫血、血沉明显增快。Objective To analyze the clinical manifestations of patients with sever ANCA related vasculitis. Methods Clinical and pathological data were collected form 16 patients with ANCA related vasculitis,their sys-temic,especially extra-renal manifestations were analyzed. Results Of the 16 patients,11 were male and 5 were female with age at 26～66 years old.Thirteen of the 16 were cANCA positive,all recognized PR3;the other 3 were pANCA positive,and 8 of the 16 recognized MPO.Clinically,patients with ANCA related vasculitis had multi-systemic involvment.96.4% patients had kidney involvement;75% had lung involvment,47.6% patients had arthralgia;35.7% had muscle pain;38.1% had skin rash;26.2% had eye involvment;16.7% had ear involv-ment and 28.6 had gastrointestinal symptoms.Also,70.8% patients had fever and 31.0% of them had body weight loss.In laboratory examinations,81.6% of the patients had anemia,over half had an increased WBC count and all had an increased ESR.In this study,only less than 20% of the patients was correctly diagnosed as ANCA related vasculitis within l month. Conclusion ANCA related vasculitis was a multisystemic autoimmune disease.Kid-ney was the most vulnerable organ to be involved and lung was the most important extrarenal organ to be affected. Other organs,such as joint,muscle,skin,eye and ear,could also be involved.Patents with ANCA related vascul-itis tended to have anemia and increased ESR.

关 键 词：抗中性粒细胞胞浆抗体 血管炎 体征与症状 

分 类 号：R692.16[医药卫生—泌尿科学]