摘要
目的探讨肺脏受累的原发性抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎患者的临床特征,为肺部受累的原发性ANCA相关性小血管炎的诊断提供帮助。方法回顾性分析2009年3月至2013年9月在湘雅二医院住院的符合2012年美国Chapel Hill会议关于系统性小血管炎诊断标准,并血清ANCA阳性的43例肺脏受累的的原发性ANCA相关性小血管炎患者的临床资料。结果 43例患者中,肺部症状首发就诊者22例,常见肺部症状依次为咳嗽咯痰、活动后气促、咯血、哮喘等,肺外受累器官依次为肾脏、神经系统、眼、鼻;肺外症状首发就诊者21例,肾脏为最常见受累器官;显微镜下多血管炎(MPA)34例,其中抗髓过氧化物酶(MPO)抗体(P-ANCA)阳性率97.1%(33/34),抗蛋白酶3(PR3)抗体(C-ANCA)阳性率2.9%(1/34);肉芽肿性多血管炎(GPA)(韦格纳肉芽肿)8例,抗蛋白酶3(PR3)抗体(C-ANCA)阳性率62.5%(5/8),抗髓过氧化物酶(MPO)抗体(P-ANCA)阳性率为37.5%(3/8);嗜酸细胞性肉芽肿性多血管炎(EGPA)1例,为抗髓过氧化物酶(MPO)抗体阳性;胸部影像学表现多为双肺间质病变,如双肺网格样改变、磨玻璃影、蜂窝肺、多发条索状及结节性病变等,或为条索或斑片状病变、支气管扩张、胸腔积液及肿块病变等。经激素和免疫抑制剂治疗多数患者病情可缓解,19例患者(44.2%)于住院及随访期间因血管炎活动并肺部感染、大咯血及肾功能衰竭等原因死亡。结论肺脏受累的原发性ANCA相关性小血管炎临床表现无特异性,多数患者合并有肾脏受累,影像学多为肺间质病变,具有提示诊断价值,血清ANCA检查有特殊诊断价值,此类患者死亡率高,多为疾病活动并感染致死,应积极控制感染并合理采用抑制免疫治疗。
Objective To analyze the clinical features of anti-neutrophil cytoplasmic antibody （ANCA） associated vasculitis with pulmonary damage.Methods We retrospectively investigated the clinical data of 43 patients hospitalized with ANCA associated vasculitis with pulmonary damage from March 2009 to September 2013.Results Among 43 patients with lung damage in primary ANCA associated vascutitis cases,pulmonary symptoms starting treatment in 22 cases,Extrapulmonary symptoms starting treatment in 21 cases,The common pulmonary symptoms of vasculitis were cough and expectoration,Exertional dyspnea,cough hemoptysis,kidney is the most common organ involved.All patients were positive for ANCA,microscopic polyangiitis （MPA） 34 cases,including myeloperoxidase （MPO） positive rate of 97.1％ （33/34） ;Granulomatosis with polyangiitis （Wegener＇s） （GPA） 8 cases of anti-PR3 antibody positive rate of 62.5％ （5/8）; Eosinophilic granulomatosis with polyangilitis （EGPA） （Churg-Strauss） 1 cases of MPO positive.Radiographic manifestations most included interstitial lung disease,such as lung grid-like changes,ground-glass opacities,honeycomb lung,multiple funicular and nodular lesions,ect,but also for Streak or patchy changes,bronchiectasis,pleural effusion,nodular lesions,and mass shadow.With hormones and immunosuppressive therapy,most patients can relieve,19 （44.2 ％） patients died of vasculitis activities and lung infections,hemoptysis and renal failure and other causes within the induction therapy period or during follow-up.Conclusion The clinical manifestations of pulmonary involvement in patients with ANCA associated vasculitis were nonspecific,renal involvement was common,radiographic manifestations mostly included interstitial lung disease,which have prompt diagnosis value,examination of ANCA have special diagnostic value.This high mortality mostly due to infection of disease activity,We Should act more actively to cure infection and give immunosuppressive therapy.
出处
《中华肺部疾病杂志（电子版）》
CAS
2014年第6期24-27,共4页
Chinese Journal of Lung Diseases(Electronic Edition)
关键词
抗中性粒细胞胞浆抗体
小血管炎
肺脏受累
Anti-neutrophil cytoplasmic antibody
Small vasculitis
Lung damage
