抗中性粒细胞胞浆抗体相关性间质性肺炎的临床特征及预后  

Clinical features and prognosis of anti-neutrophil cytoplasmic antibodies associated interstitial pneumonia

作　　者：王国庆[1] 钱龙[1] 徐舟舟 王春淮[1] 李东旭[1] WANG Guo-qing;QIAN Long;XU Zhou-zhou;WANG Chun-huai;LI Dong-xu(Department of Rheumatology,the Second Affiliated Hospital of Anhui Medical University,Hefei 230601,China)

机构地区：[1]安徽医科大学第二附属医院风湿免疫科,合肥230601

出　　处：《中华临床免疫和变态反应杂志》2019年第1期17-23,共7页Chinese Journal of Allergy & Clinical Immunology

摘　　要：目的提出抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibody,ANCA)相关性间质性肺炎概念并分析其临床特征与预后。方法回顾性分析比较2011年1月至2016年8月本院36例诊断符合间质性肺炎(interstitial pneumonia,IP)但不符合ANCA相关性血管炎(ANCA-associated vasculitis,AAV)诊断的ANCA阳性的间质性肺炎(ANCA-IP),19例同时符合IP及AAV诊断(AAV-IP)及40例特发性间质性肺炎(idiopathic interstitial pneumonia,IIP)患者的临床资料、治疗及预后。采用Kruskal-Wallis H检验、卡方分割法、多元Logistic回归、Kaplan-meier法进行统计分析。结果(1)ANCA-IP、AAV-IP、IIP三组间比较年龄(χ2=0.177,P=0.915)、性别(χ2=3.555,P=0.169)比较差异无统计学意义;呼吸困难更常见于IIP组(χ2=17.042,P=0.001);AAV-IP组患者WBC(χ2=11.333,P=0.003)、Cr(χ2=17.371,P=0.001)、CRP(χ2=8.549,P=0.014)、ESR(χ2=13.062,P=0.001)均显著高于ANCA-IP及IIP组;ANCA-IP患者胸部HRCT更多表现为蜂窝肺(χ2=6.671,P=0.036)。(2)ANCA-IP组内治疗方式对比,不同治疗方式对预后有显著差异(χ2=6.440,P=0.040),与大剂量激素+CTX组相比,小剂量激素/对症治疗组患者预后更好(χ2=6.438,P=0.011);IIP患者不同治疗方式间比较无显著差异(χ2=0.029,P=0.865)。(3)随访过程中,1例(2.8%)ANCA-IP患者在25月后进展为AAV。(4)多元Logistic回归分析结果显示,大剂量激素+CTX的治疗方式(OR=0.029,P=0.018)是存活的相关危险因素。结论ANCA-IP患者病情可能长期保持稳定而不进展为AAV,它可能是AAV疾病的早期阶段,也可能是一个独立的疾病,应避免过度免疫治疗。Objective To clarify the concept of anti-neutrophil cytoplasmic antibodies(ANCA)associated interstitial pneumonia(IP)and analyze its clinical characteristics and prognosis.Methods Thirty-six patients diagnosed as IP but not conformed to ANCA associated vasculitis(ANCA-IP group),19 both IP and ANCA associated vasculitis(AAV-IP group),and 40 idiopathic interstitial pneumonia(IIP group)were studied by retrospectively.The clinical features,laboratory indexes,pulmonary high resolution CT(HRCT)images,treatment and prognosis were compared among these three groups.Kruskal-Wallis H test,chi square method,multivariate Logistic regression and Kaplan-meier method were used for statistical analysis.ResultsThere were no significant differences in age and sex among these three groups.Dyspnea was the most common clinical manifestation in the IIP group.White blood cells(WBC),serum creatinine(Cr),C-reactive protein(CRP),erythrocyte sedimentation rate(ESR)were all significantly higher in the AAV-IP group.Honeycomb sign on HRCT was found more frequently in the ANCA-IP group.The patients treated with low dosagesof glucocorticoidhad a better prognosis than those with large dosages combined with cyclophosphamide.No differences were exhibited among different treatments in the IIP group.Only one patient(2.8%)in the ANCA-IP group progressed to AAV after a follow-up of 25 months.Multivariate Logistic regression analysis showed that large doses of glucocorticoid combined with cyclophosphamide treatment was a related risk factor for survival of ANCA-IP patients.Conclusions ANCA-IP patients may be in a stable condition for a relatively long time,and hardly progress to AAV.ANCA-IP may be an early stage of AAV or an independent state of the disease,which suggest that aggressive immunosuppressive therapy might be appropriate.

关 键 词：抗中性粒细胞胞浆抗体 间质性肺炎 

分 类 号：R56[医药卫生—呼吸系统]