以肺部受累为主要表现的抗中性粒细胞胞质抗体相关性血管炎患者的临床特征分析  

Clinical Features of Anti-Neutrophil Cytoplasmic Antibodies AssociatedVasculitis with Pulmonary Involvement as Primary Manifestation

作　　者：韩李周[1] 郑红[1] 薛东鹰[1] 李玉[1] HAN Lizhou;ZHENG Hong;XUE Dongying;LI Yu(Department of Pulmonary and Critical Care Medicine,Jiaozuo People’s Hospital,Jiaozuo 454002,China)

机构地区：[1]焦作市人民医院呼吸与危重症医学科,河南焦作454002

出　　处：《河南医学研究》2021年第2期220-224,共5页Henan Medical Research

摘　　要：目的总结以肺部受累为主要表现的抗中性粒细胞胞质抗体相关性血管炎(AAV)的临床特征,以提高诊治水平。方法回顾分析2014年9月至2020年4月焦作市人民医院呼吸与危重症医学科收治的5例AAV患者的临床资料。结果5例患者中,男2例,女3例,年龄为68~85岁,中位年龄70岁。临床表现为气促、呼吸困难或胸闷、喘息、咳嗽、咳痰、咯血、发热等。5例均有肺部受累,单纯肺部受累2例;3例肾脏受累,均有镜下血尿和蛋白尿,肾功能不全1例;1例心脏受累,表现为肺动脉高压和心包积液;1例肌电图证实周围神经受累。初诊易误诊为肺炎、特发性肺纤维化(IPF)等。核周型抗中性粒细胞胞质抗体(pANCA)或抗髓过氧化物酶抗体(MPO-ANCA)阳性5例,胞浆型抗中性粒细胞胞质抗体(c-ANCA)阳性1例。肺穿刺证实肉芽肿性炎1例。胸部CT表现为多发结节或实变伴或不伴小空洞形成、片状渗出浸润影、肺动脉高压、间质性肺炎、支气管扩张、纤维条索影等。糖皮质激素联合环磷酰胺等免疫抑制剂治疗2例,单纯激素治疗1例均好转,1例放弃治疗后死亡,1例拒绝进一步治疗。结论AAV起病年龄较大,临床症状无特异性,累及肺部时易误诊为肺炎、IPF等,从查体和检查化验中发现多系统受累的证据,结合抗中性粒细胞胞质抗体(ANCA)、肺穿刺病理证据等有助于诊断。Objective The clinical features of anti-neutrophil cytoplasmic antibodies associated vasculitis(AAV)with pulmonary involvement as primary manifestation were summarized to improve the diagnosis and treatment.Methods The clinical data of 5 AAV patients admitted to the Department of Pulmonary and Critical Care Medicine of Jiaozuo People’s Hospital from September 2014 to April 2020 were retrospectively analyzed.Results Among the 5 patients,there were 2 males and 3 females,aged from 68 to 85 years old,and the median age was 70 years.Clinical manifestations were shortness of breath,dyspnea or chest tightness,wheezing,cough,sputum,hemoptysis,fever and so on.All 5 patients had pulmonary involvement,and 2 patients had pulmonary involvement alone.There were 3 cases of renal involvement,including microscopic hematuria and proteinuria,and 1 case of renal insufficiency.One case of cardiac involvement presented with pulmonary hypertension and pericardial effusion.Electromyography confirmed peripheral nerve involvement in 1 case.At initial diagnosis,these cases were misdiagnosed as pneumonia or idiopathic pulmonary fibrosis(IPF).Perinuclear pattern anti-neutrophil cytoplasmic antibodies(pANCA)or myeloperoxidase anti-neutrophil cytoplasmic antibody(MPO-ANCA)positive in 5 cases,and cytoplasmic pattern anti-neutrophil cytoplasmic antibodies(c-ANCA)positive in 1 case.Lung biopsy confirmed granulomatous inflammation in 1 case.Chest CT showed multiple nodules or consolidation with or without small cavities,patchy exudation infiltrating shadows,pulmonary hypertension,interstitial pneumonia,bronchiectasis,and fiber cord shadows.Both the 2 patients treated with glucocorticoid combined with cyclophosphamide and 1 patient treated with glucocorticoid alone improved.One patient died after giving up treatment and the other refused further treatment.Conclusion The onset age of AAV is relatively old,and its clinical symptoms are not specific.When the lungs are involved,it is easy to be misdiagnosed as pneumonia or IPF,etc.Evidence of multi-s

关 键 词：抗中性粒细胞胞质抗体相关性血管炎 临床表现 诊断 

分 类 号：R593.2[医药卫生—内科学]