Strategies for treating idiopathic pulmonary fibrosis

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1、REVIEWSStrategiesfortreatingidiopathicpulmonaryfibrosisR.M.duBoisAbstract

2、Idiopathicpulmonaryfibrosis(IPF)isthemostcommonandmostlethaldiffusefibrosinglungdisease,withamortalityratethatexceedsthatofmanycancers.Recently,therehavebeenmanyclinicaltrialsofnoveltherapiesforIPF.Theresultshavemostlybeen

3、disappointing,althoughtwotreatmentapproacheshaveshownsomeefficacy.ThisReviewdescribesthedifficultiesoftreatingIPFandtheapproachesthathavebeentriedorareindevelopment,andconcludeswithsuggestionsoffuturetherapeutictargetsandstrategies.Thelungservesthevitalfunctionofprovidingaccessforandorrepeatedin

4、juries,resultinginabnormalfunctionoxygentothepulmonarycirculation,allowingitsdelivery(forreviewsofthevariousdiseasesthatcanscarthelung,toallotherorgans.Toachievethis,thelunghasevolvedtoseeRef.5).Ifasufficientproportionofthelungbecomescompriseaseriesoftubes—thebronchialtree—thatleadscarred,respir

5、atoryfailurecanoccur.Progressivelunginabranchingnetworktonumerousairsacs—alveoliscarringmayresultfromarecurrentseriesofinsults—thattogetherformastructurethatissimilartoabee’stodifferentregionsofthelungorafailuretohaltthehoneycomb.Thebronchialtreeprovidestheconduitrepairprocessaftertheinjuryhashe

6、aled.Insuchcasesthroughwhichairtravelstothealveoli.Theextremelythinthescarringprocessbecomesuncontrolledanddereguwallsofthealveoliallowthediffusionofoxygenintothelated.Insomeformsoffibrosinglungdiseasescarringadjacentbloodcapillariesandtheoppositemovementofremainslocalizedtoalimitedregion,butino

7、thersitcarbondioxide(forcomprehensivereviews,seeRef.1).canaffectamorediffuseandextensivearea.ThisgroupThesedelicatealveolarstructuresareexquisitelysensiofdisordersisthereforeoftenreferredtoasthediffusetivetoinjuryandsubsequentlungscarring.Thebodylungdiseases6.respondstoinjurybyformingascar(fibro

8、sis)—forexample,aspartoftheresponsetotuberculosisorfungalDiseaseclassification.Diffuselungdiseaseisalsoknowninfections.However,inothercircumstances—suchasasinterstitiallungdiseasebecausethemajorsiteofhisinsarcoidosisorhypers