显微镜下多血管炎相关间质性肺疾病28例临床特征及生存分析  

Clinical features and survival analysis of microscopic polyangiitis-associated interstitial lung disease:a retrospective study of 28 patients

作　　者：吴挺挺 岑泽凯 周海军[2] 孙超 唐攀 张筠[1] 丁群力[1] Wu Tingting;Cen Zekai;Zhou Haijun;Sun Chao;Tang Pan;Zhang Yun;Ding Qunli(Department of Respiratory and Critical Care Medicine,the Affiliated Hospital of Medical School of Ningbo University,Ningbo 315020,China;Department of Rheumatology,the Affiliated Hospital of Medical School of Ningbo University,Ningbo 315020,China)

机构地区：[1]宁波大学医学院附属医院呼吸与危重症医学科,宁波315020 [2]宁波大学医学院附属医院风湿免疫科,宁波315020

出　　处：《中华结核和呼吸杂志》2022年第10期1022-1030,共9页Chinese Journal of Tuberculosis and Respiratory Diseases

基　　金：浙江省自然科学基金(LBY22H180004)。

摘　　要：目的探讨显微镜下多血管炎相关间质性肺疾病(MPA-ILD)临床、实验室检查及影像学特征并进行生存分析。方法回顾性分析2014年8月至2021年11月在宁波大学医学院附属医院确诊的28例MPA-ILD患者的临床资料, 28例中男18例, 女10例, 年龄为(70.1±9.3)岁, 总结本组患者的临床表现、实验室检查、肺功能、心脏彩超、胸部CT及治疗方案等, 并对相关数据进行统计学分析。结果本组患者中13例存在吸烟史, 临床主要表现为咳嗽(14/28), 发热(12/28), 胸闷气促(12/28)及咯血(3/28), 16例存在肾脏累及, 多数患者存在C反应蛋白(22/28)和红细胞沉降率(25/28)升高, 16例存在类风湿因子(RF)增高, 23例髓过氧化物酶(MPO)-ANCA阳性。27例患者在MPA诊断之前发现ILD或与MPA同时诊断ILD, 胸部CT模式主要为UIP型或UIP样(15/28), 其次为NSIP型(8/28)。UIP型或UIP样患者较非UIP样患者年龄更大(P=0.018), 血LDH更高(P=0.041), 但血肌酐却明显较低(P=0.041)。通过单因素和多因素生存分析显示未正规治疗(HR=9.81, 95%CI:1.68~57.29, P=0.011)及血LDH升高(HR=4.11, 95%CI:0.99~17.00, P=0.051)是MPA-ILD生存期缩短的独立危险因素, 而RF升高(HR=0.22, 95%CI:0.06~0.91, P=0.037)是生存期延长的保护因素。结论 MPA-ILD全身血管炎症状较少, 绝大部分ILD在MPA诊断之前出现或与MPA同时诊断, 影像表现主要为UIP型或UIP样, 其次为NSIP型, 尽早使用激素联合免疫抑制剂或激素联合利妥昔单抗能够提高MPA-ILD生存率, 血LDH升高是MPA-ILD生存期缩短的独立危险因素, 而RF升高则是生存期延长的保护因素。Objective To explore the clinical features,laboratory examination and imaging features of microscopic polyangiitis(MPA)-associated interstitial lung disease(ILD),and to perform survival analysis.Methods The records of 28 patients with MPA-ILD who were treated at the Affiliated Hospital of Medical School of Ningbo University were reviewed retrospectively from August 2014 to November 2021.The patients′clinical features,laboratory parameters,pulmonary function test,echocardiography,chest CT scan findings and therapeutic regimen were analyzed,and the relevant data were statistically analyzed.Results There were 18 males and 10 females,with an average age of(70.1±9.3)years.Among them,13 patients had a history of smoking.The main clinical manifestations were cough(14/28),fever(12/28),chest tightness,shortness of breath(12/28)and hemoptysis(3/28).Sixteen patients had renal involvement,and 78.57%(22/28)and 89.28%(25/28)of the patients had elevated C-reactive protein(CRP)and ESR respectively.Sixteen(16/28)patients had increased rheumatoid factor(RF),and the positive rate of myeloperoxidase antineutrophil cytoplasmic antibodies(MPO-ANCA)was 82.12%(23/28).96.43%(27/28)of ILDs were diagnosed before or at the same time as MPA.The chest radiological pattern was mainly usual interstitial pneumonia(UIP)or UIP-like(15/28),followed by nonspecific interstitial pneumonia(NSIP)(8/28).Compared with non-UIP-like patients,UIP or UIP-like patients were older(P=0.018),and had higher serum LDH level(P=0.041),but serum creatinine level was significantly lower(P=0.041).Univariate and multivariate survival analysis showed that inappropriate treatment(HR=9.81,95%CI:1.68-57.29,P=0.011)and elevated serum LDH(HR=4.11,95%CI:0.99-17.00,P=0.051)were independent risk factors for shortened survival of MPA-ILD,while elevated RF(HR=0.22,95%CI:0.06-0.91,P=0.037)was a protective factor for prolonged survival.Conclusions MPA-ILD patients had fewer systemic vasculitis symptoms.Most of the ILD patients were diagnosed before or at the same time as MPA.The c

关 键 词：显微镜下多血管炎 肺疾病 间质性 普通型间质性肺炎 

分 类 号：R563[医药卫生—呼吸系统]