医学博文63-综述:间质性肺疾病:结缔组织病······
2014-02-28
张莉芸

主任医师山西大医院

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.

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