原发性抗中性粒细胞胞浆抗体相关性血管炎的临床表现及肺部CT特征  

Clinical manifestations and pulmonary radiological findings of primary anti-neutrophil cytoplasmic antibody-associated vasculitis

作　　者：虞有超 俞海瑾[2] 侯小霞[1] 倪颖梦[1] 陈虹[1] 时国朝[1] 

机构地区：[1]上海交通大学医学院附属瑞金医院呼吸科,200025 [2]上海交通大学医学院附属瑞金医院肾脏科,200025

出　　处：《中华全科医师杂志》2016年第7期534-538,共5页Chinese Journal of General Practitioners

摘　　要：目的 探讨不同抗原类型原发性抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）患者的临床表现及肺部CT特征.方法 回顾性分析2003年1月1日至2015年11月30日在上海交通大学医学院附属瑞金医院住院诊断为原发性AAV且血清ANCA阳性的271例患者的临床资料,按照靶抗原类型分为髓过氧化物酶（MPO）-ANCA阳性（MPO组）、蛋白酶3（PR3）-ANCA阳性（PR3组）和MPO/PR3双阳性组,比较3组患者的一般情况、临床表现、肺部CT特征、ANCA水平及肾功能.结果 271例原发性ANCA阳性AAV患者中,MPO组211例,PR3组52例,双阳性组8例.MPO组发生肾脏受累[96.2％ （203/211）比88.5％ （46/52）,x2=4.968,P=0.026]、全身症状[39.3％ （83/211）比17.3％ （9/52）,x2=8.901,P=O.003]的比率高于PR3组,而PR3组有耳鼻喉症状[48.1％（25/52）比18.5％（39/211）,x2=19.843,P＜0.001]、咳嗽[67.3％（35/52）比47.4％（100/211）,x2=6.623,P =0.010]、咯血[28.8％ （15/52）比12.3％ （26/211）,x2 =8.656,P =0.003]、呼吸困难[34.6％（18/52）比19.9％ （42/211）,x2=5.127,P=0.024]、黏膜及眼部症状[7.7％（4/52）比2.8％ （6/211）,x2=4.818,P=0.028]的比率高于MPO组;MPO组肺部CT发现肺间质改变的比率高于PR3组[25.2％ （39/155）比10.9％ （5/46）,x2=4.237,P=0.040],而PR3组发现肺部结节影的比率高于MPO组[41.3％ （19/46）比25.2％（39/155）,x2=4.503,P=0.034];双阳性组发生神经、呼吸、肾脏受累的比率高;MPO组肌酐（Z=-5.529,P＜0.001）、尿素氮（Z=-4.646,P＜0.001）、尿酸（Z=-2.331,P=0.020）高于PR3组.双阳性组肌酐（Z=-3.251,P=O.001）、尿素氮（Z=-2.882,P=0.004）高于PR3组,但与MPO组比较差异无统计学意义（P＞0.05）.结论 MPO-ANCA阳性与PR3-ANCA阳性的原发性AAV患者存在明显的临床表现和肺部CT特征不同,MPO组、双阳性组的肾功能损害重于PR3组.通过比较临床及肺部CT特征,有助于临床医师提高对AAV的全面认识.Objective To analyze the clinical features and pulmonary radiological findings of primary anti-neutrophil cytoplasmic antibody（ANCA）-associated vasculitis （AAV）.Methods Clinical data of 271 ANCA positive primary AAV patients admitted in Shanghai Ruijin Hospital from January 2003 to November 2015 were retrospectively analyzed.Among 271 patients,there were 211 myeloperoxidase （MPO）-ANCA positive cases （MPO subgroup）,52 proteinase 3 （PR3）-ANCA positive cases （PR3 subgroup）,and 8 dual positive cases （dual subgroup） according to ANCA classification.The demography,clinical characteristics,pulmonary radiological manifestation and renal function were compared among three subgroups.Results MPO subgroup had more renal （x2 =4.968,P =0.026） and constitutional symptoms （x2.=8.901,P =0.003） than PR3 group,while PR3 group had more ENT symptoms （x2 =19.843,P ＜ 0.001）,cough （x2 =6.623,P =0.010）,hemoptysis （x2 =8.656,P =0.003）,dyspnea （x2 =5.127,P =0.024）,buccal and ocular mucosal symptoms （x2 =4.818,P =0.028） than MPO group.In lung radiology,interstitial manifestations were displayed more frequently in MPO than PR3 group （x2 =4.237,P =0.040）,while pulmonary nodules was more frequent in PR3 than MPO group （x2 =4.503,P =0.034）.Dual subgroup tended to have more nervous,respiratory and renal impairment.Renal function showed that MPO subgroup had higher creatinine （Z =-5.529,P ＜ 0.001）,urea （Z =-4.646,P ＜ 0.001） and uric acid levels （Z =-2.331,P =0.020） than PR3 subgroup.Dual subgroup had higher creatinine （Z =-3.251,P =0.001） and urea （Z =-2.882,P =0.004） levels than PR3 subgroup,but there was no difference with MPO subgroup.Conclusion There are significant differences in both clinical and pulmonary radiological manifestations between the MPO and PR3-ANCA subgroup of primary AAV.

关 键 词：抗中性粒细胞胞浆抗体相关性系统性血管炎 髓过氧化物酶 蛋白酶3 临床表现 肺部CT 

分 类 号：R593.2[医药卫生—内科学]