轻链自身抗体在髓过氧化物酶-抗中性粒细胞胞质抗体相关性血管炎肺损害中的意义  

A preliminary study of the significance of autoantibodies against light chain of myeloperoxidase on pulmonary damages in myeloperoxidase-antineutrophil cytoplasmic antibody associated vasculitis

作　　者：张蕾[1] 帅宗文[1] 胡子盈 张铭明[1] 陈珊宇[1] 

机构地区：[1]安徽医科大学第一附属医院风湿免疫科,合肥230022

出　　处：《中华内科杂志》2015年第6期511-516,共6页Chinese Journal of Internal Medicine

基　　金：中华医学会临床医学科研专项资金项目（08010290107）

摘　　要：目的 初步探讨髓过氧化物酶（MPO）-抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）合并肺损害的临床特点及其与抗MPO轻链ANCA的关系.方法 收集195例核周型-ANCA（p-ANCA）及MPO-ANCA均阳性的初诊原发性MPO-AAV患者的临床资料,第3版修订的伯明翰血管炎活动度评分（BVAS-V3）评估病情活动性,使用重组MPO轻链检测MPO轻链ANCA,分析肺损害的特点及其与外周血p-ANCA、MPO-ANCA及MPO轻链ANCA的关系.结果 195例患者中显微镜下多血管炎191例（98.0％）,肉芽肿性多血管炎3例（1.5％）,嗜酸性肉芽肿性多血管炎1例（0.5％）,男64例,女131例,年龄（63.2±13.5）岁.全部患者MPO-ANCA与总BVAS-V3呈正相关（r =0.193,P=0.007）,MPO轻链ANCA与肺BVAS-V3呈正相关（r=0.228;P=0.001）.多系统损害中,肺损害118例（60.51％）,仅次于肾损害（140例,71.80％）.常见肺损害主要为肺浸润80.51％（95/118）、胸腔积液/胸膜炎41.53％（49/118）、肺结节或空洞22.03％（26/118）.有肺损害者较无肺损害者年龄高[（66.39±10.70）岁比（58.30±15.72）岁;t=4.277,P＜0.001]、病程短[2.00（1.00,10.50）个月比3.00（1.00,3.50）个月;Z=-1.604,P=0.109]、总BVAS-V3更高[（18.21±6.08）分比（15.18±5.64）分;t=3.501,P=0.001]、MPO轻链ANCA阳性率明显升高（35.59％比6.49％;x2 =21.569,P＜0.001）、ELISA检测MPO轻链ANCA的吸光度值显著升高（0.377 ±0.229比0.285±0.079;t=3.399,P=0.001）.MPO轻链ANCA阳性者较阴性者肺BVAS-V3明显升高[（4.34±2.10）分比（2.59±2.52）分;=4.301,P＜0.001].结论 MPO-AAV中肺损害常见且多隐匿,ANCA检查及随访对AAV患者肺损害诊断具有重要价值,其中MPO轻链ANCA在AAV肺损害的发病中可能发挥重要作用.Objective To investigate the clinical characteristics of myeloperoxidase （MPO）-antineutrophil cytoplasmic antibody （ANCA） associated vasculitis （MPO-AAV） with pulmonary injury and the relationship between pulmonary injury and ANCA against light chain of MPO （LCMPO-ANCA）.Methods A total of 195 patients with newly diagnosed primary active MPO-AAV were recruited in this prospective study.Indirect immunofiuorescence assay was used to detect peri-nuclear ANCA （p-ANCA）.Immunoblotting and ELISA were used to detect myeloperoxidase-antineutrophil cytoplasmic antibody （MPO-ANCA）.Clinical features of patients with both positive p-ANCA and MPO-ANCA were collected.Disease activity was evaluated by Birmingham Vasculitis Activity Score-version 3 （BVAS-V3） Recombinant light chain of MPO was used to coat substrate of LCMPO-ANCA by ELISA.The clinical characteristics of pulmonary injury and its correlation with serum levels of p-ANCA,MPO-ANCA and LCMPO-ANCA were explored.Results All 195 patients （64 male and 131 female）,consisted of 191 patients （98.0%） with microscopic polyangiitis,3 patients （1.5%） with granulomatosis with polyangiitis,and 1 （0.5%） with eosinophilic granulomatosis with polyangiitis including 64 men and 131 women.Their mean age was （63.2 ±13.5） years old.The level of MPO-ANCA had a positive correlation with general BVAS-V3 （r =0.193,P =0.007） in all patients,and the level of LCMPO-ANCA was positively related with the pulmonary BVAS-V3 （r =0.228,P =0.001）.As for multiple systemic damages,the incidence of lung involvement was 60.51%（118/195）,which ranked second to renal involvement （71.80%,140/195）.The most common pulmonary injuries represented as pulmonary infiltration of 80.51% （95/118）,pleural effusion / pleurisy of 41.53%（49/118）,pulmonary nodule or cavity of 22.03% （26/118）.Compared with those without lung involvement,the patients with pulmonary injuries were older [（66.39 ± 10.70） years old vs （58.30 ±15.72） years old;t =4.2

关 键 词：抗体 抗中性粒细胞胞质 抗髓过氧化物酶抗体 抗髓过氧化物酶轻链抗体 血管炎 肺损害 

分 类 号：R593.2[医药卫生—内科学]