综述：间质性肺疾病：结缔组织病相关的间质性肺疾病与IPF有何不同？临床诊疗有何不同？

译者：侯建文 校对：李玉翠 审核：张莉芸

Abstract

Thelungisfrequentlyinvolvedinconnectivetissuediseases(CTDs),althoughthefrequencyoflungmanifestationsvariesaccordingtothetypeofCTD.Interstitiallungdiseases(ILD)arefrequentlyseeninCTDs,particularlysystemicsclerosis(SSc),polymyositis/ dermatomyositis(PM/DM)andrheumatoidarthritis(RA),accountingforasignificantproportionofdeaths.AlargepercentageofpatientswithCTD- associatedILDhaslimitedandstabledisease,notrequiringtreatment.However,asignificantminorityhassevereand/orprogressivedisease,necessitatingpromptinitiationoftreatment.CTD-ILDhistologicalpatternsincludenon-specificinterstitialpneumonia(NSIP),usualinterstitialpneumonia(UIP),organizingpneumonia(OP),diffusealveolardamage(DAD)andlymphocyticinterstitialpneumonia(LIP).NSIPisthemostcommonpatterninallCTDs,exceptforRA,characterizedbyahigherfrequencyofUIP.ILDcanpresentacutelyorchronically,withacutepresentations beingmorecommoninsystemiclupuserythematosusandPM/DM.Idiopathicpulmonaryfibrosis(IPF)isaprogressivelyworseningILDcharacterizedbyinflammationandfibrosis.Thecharacteristic histologicalpatternofIPFisUIP.Interestingly,aUIPpatternisassociated withasignificantlybettersurvivalinCTD-relateddiseasecomparedtotheidiopathicvariety.PrognosisinIPFisdismal,withamediansurvivalsincediagnosisof2-3years.NotreatmentregimenhasbeenshowntoimprovesurvivalinIPF.Bycontrast,althoughtherehavebeenonlytworandomizedplacebo-controlledtrialsinvestigatingtheeffectofimmunosuppressivetreatmentinSSc-associatedILD,clinicalexperiencesuggeststhatimmunosuppressivedrugsinCTD-relatedILDsarecapableofbenefitingasignificantproportionofpatients,particularlythosewithcertainhistologicalpatternsofdisease.ThisreviewwillessentiallyfocusonCTD-associatedILDandwillcompareaspectsofclinicalpresentationandmanagementtothoseofIPF.

摘要：结缔组织病（CTDs）多有肺部受累，不同类型的CTD肺部表现不同。间质性肺疾病(ILD) 可见于多种结缔组织病，特别是硬皮病(SSc)、多肌炎/皮肌炎(PM/DM)及类风湿关节炎(RA)，且是其主要死因。大部分CTD相关间质肺病患者病情平稳，不需要治疗。然而，极少数人病情严重和/或呈进展性，必须积极早期治疗。CTD-ILD的组织学形态包括非特异性间质性肺炎（NSIP）、普通型间质性肺炎（UIP）、机化性肺炎（OP）、弥漫性肺泡损伤（DAD）和淋巴细胞性间质性肺炎（LIP）。除RA外,NSIP是所有CTDs最常见类型，而RA则多表现为UIP。ILD可急性或慢性起病，在系统性红斑狼疮和PM/DM多为急性起病。特发性肺纤维化（IPF）是一种以炎症和纤维化为特征的进行性恶化的ILD。IPF典型的组织学形态是普通型间质性肺炎（UIP）。有趣的是，与IPF相比,UIP在CTD相关特发性疾病中预后较好。IPF预后不佳，平均生存期为诊断后2-3年。 IPF尚无有效的治疗方法。相比之下，仅有两个随机安慰剂对照研究试验研究硬皮病相关ILD的免疫抑制治疗效果，临床经验表明，大部分CTD相关ILD免疫抑制剂治疗有效，特别是有确定组织学形态的疾病。此综述主要把CTD相关ILD和特发性肺纤维化在其临床表现和疾病管理方面作一对比。

引自：LauretisA, VeeraraghavanS, RenzoniE. Reviewseries Aspectsofinterstitiallungdisease:connectivetissuedisease-associatedinterstitiallungdisease:howdoesitdifferfromIPF?Howshouldtheclinicalapproachdiffer?ChronRespirDis. 2011;8(1):53-82.