特发性肺纤维化急性加重的风险因素

目的：特发性肺纤维化患者中肺纤维化的急性加重是导致患者致残和致死的主要原因。但是，急性加重仍是不可预测的。该研究的目的就是探讨导致肺纤维化加重的危险因素。

方法：回顾性分析年1月～年9月某研究机构的特发性肺纤维化患者。根据年特发性肺纤维化ATS/ERS/JRS/ALAT的官方标准，作者分析了特发性肺纤维化患者急性加重的风险因素。

结果：总共纳入65例患者，中位随访时间为2.6年。随访期间，24例（36.9%）患者出现急性加重。Kaplan-Meier曲线显示1年、2年和3年的急性加重发生率为分别为9.6%、19.2%和31.0%。急性加重对该病的总体生存率有显著影响。对数秩检验显示基线期心血管病、更高的GAP级别(性别、年龄、生理状态)（II）、血清乳酸脱氢酶水平增高(≥U/L)、血清表面活化蛋白D水平增高(≥.7ng/ml)、肺泡灌洗液中性粒细胞(≥1.77%)和嗜酸性粒细胞比例增高(≥3.21%)以及免疫抑制剂治疗急性加重有关。校正免疫抑制剂治疗的因素，Cox分析显示，基线期心血管病、更高的GAP级别（II）以及肺泡灌洗液中嗜酸性粒细胞比例增高(≥3.21%)是特发性肺纤维化急性加重的预测因素。

结论：这项研究提示，基线期心血管病、更高的GAP级别（II）以及肺泡灌洗液中嗜酸性粒细胞比例增高(≥3.21%)是特发性肺纤维化急性加重的预测因素。

Riskfactorsforanacuteexacerbationofidiopathicpulmonaryfibrosis.

KakugawaT,SakamotoN,SatoS,YuraH,HaradaT,NakashimaS,HaraA,OdaK,IshimotoH,YateraK,IshimatsuY,ObaseY,KohnoS,MukaeH.

Abstract

BACKGROUND:Acuteexacerbationsofidiopathicpulmonaryfibrosisaremajorcausesofmorbidityandmortalityamongpatientswithidiopathicpulmonaryfibrosis.However,acuteexacerbationsremainunpredictable.Theaimofthisstudywastoinvestigateriskfactorsforacuteexacerbationsofidiopathicpulmonaryfibrosis.

METHODS:WeperformedaretrospectivecohortstudyofpatientswithidiopathicpulmonaryfibrosiswhovisitedourinstitutionsfromJanuarytoSeptember.WeinvestigatedriskfactorsforacuteexacerbationsinpatientswithidiopathicpulmonaryfibrosisdiagnosedretrospectivelybasedontheofficialidiopathicpulmonaryfibrosisATS/ERS/JRS/ALATUpdateStatement.

RESULTS:Theidiopathicpulmonaryfibrosisstudycohortincluded65subjects.Themedianfollow-upperiodwas2.6years.

Duringfollow-up,24patients(36.9%)experiencedacuteexacerbations.AKaplan-Meiercurvedemonstratedthatthe1-year,2-year,and3-yearincidencesofacuteexacerbationwere9.6,19.2and31.0%,respectively.Acuteexacerbationexertedasignificantimpactonoverallsurvivalamongthosewiththedisease.Alog-ranktestshowedthatbaselinecardiovasculardiseases,higherGAP(gender,age,physiology)stage(≥II),higherserumlactatedehydrogenaselevel(≥U/L),higherserumsurfactantprotein-Dlevel(≥.7ng/mL),higherneutrophil(≥1.77%)andeosinophil(≥3.21%)percentagesinbronchoalveolarlavagefluidsamples,andtreatmentwithanimmunosuppressiveagentafterdiagnosiswereassociatedwithpooracuteexacerbation-freeprobability.IntheCoxanalysisadjustedfortreatmentwithanimmunosuppressiveagent,baselinecardiovasculardiseases,higherGAPstage(≥II),andhighereosinophilpercentage(≥3.21%)inbronchoalveolarlavagefluidsampleswerepredictorsofanacuteexacerbationofidiopathicpulmonaryfibrosis.

CONCLUSIONS:Thisstudydemonstratedthatbaselinecardiovasculardiseases,higherGAPstage(≥II),andhighereosinophilpercentage(≥3.21%)inbronchoalveolarlavagefluidsampleswereassociatedwiththeonsetofanacuteexacerbationofidiopathicpulmonaryfibrosis.

编译者：医院临床免疫科谢荣华