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INTERSTITIALLUNGDISEASE间质性肺病,ConceptionofILD(DPLD),ILDisaspectrumofnon-infectious,non-neoplasticdiseasesinvolvingpredominatelythealveolarwallandperialveolartissueandsurroundingarchitecture.DPLD(DiffuseParenchymalLungDisease):bronchiolesandparenchyma,MedicalTerminology,Interstitiallungdisease(ILD)间质性肺病Diffuseparenchymallungdisease(DPLD)弥漫性肺实质肺病Idiopathicinterstitialpneumonia(IIP)特发性间质性肺炎Idiopathicpulmonaryfibrosis(IPF)特发性肺纤维化Usualinterstitialpneumonia(UIP)普通性间质性肺炎Non-specificinterstitialpneumonia(NSIP)非特异性间质性肺炎Cryptogenicorganizingpneumonia(COP)隐原性机化性肺炎Acuteinterstitialpneumonia(AIP)急性间质性肺炎Desquamativeinterstitialpneumonia(DIP)脱屑性间质性肺炎,Respiratorybronchiolitis-associatedinterstitiallungdisease(RB-ILD)呼吸细支气管炎间质性肺炎Lymphocyticinterstitialpneumonia(LIP)淋巴细胞性间质性肺炎Granulomatous肉芽肿的Sarcoidosis结节病Lymphangioleiomyomatosis淋巴管平滑肌瘤Histiocytosis(HX)组织细胞增多症Reticular网格状Nodule(nodular)结节Groundglassopacity(GGO)磨(毛)玻璃影Bronchiectasis支气管扩张Honeycombing蜂窝Diffusioncapacity弥散量Bronchoalveolarlavarge(BAL)肺泡灌洗Transbronchoscopiclungbiopsy(TBLB)经气管镜肺活检Crackle爆裂音,ClassificationofILDbyATS/ERS2002,IDIOPATHICINTERSTITIALPNEUMONIA(IIP),GRANULOMATOSISsarcoidosisHypersensitivepneumonitisWegenersgranulomatosis,RAREILDteinosisalv.hemorrhage-nephritissyndromeLangerhanscellhistiocytosisLymphangioleiomyomatosisIdiopathicpulmonaryHemosiderosisChroniceosinophilicpneumonia,ILDofknowncausesOccupationallungdisease(pneumoconiosis)Drug-inducedlungdiseaseConnectivediseaseassociatedILD,ILDofUnknownCauses,AMERICANJOURNALOFRESPIRATORYANDCRITICALCAREMEDICINEVOL1652002,EPIDEMIOLOGY,ILDaccountsfor100,000hospitaladmissionsyearly15%ofpatientsseenbypulmonologistsnationwide.incidenceandprevalenceofILD(5/100,000)intheUSNewMexico:overallprevalenceofILDwas80.9per100,000inmalesand67.2per100,000infemalesincidenceofILDwas31.5per100,000inmenand26.1per100,000inwomen.Idiopathicpulmonaryfibrosisrepresented45%ofthepatientbase.,CLINICALEVALUATION-History,LengthOfIllnessAndClinicalCourse,Acuteonset:daystoweeksAcuteinterstitialpneumoniaAcutepneumonitisfromcollagenvasculardisease(especiallySLE)CryptogenicorganizingpneumoniaDrugsDiffusealveolarhemorrhage(弥漫性肺抛出血)Eosinophiliclungdisease(嗜酸性粒细胞肺病)Hypersensitivitypneumonitis(过敏性肺炎)Subacute:weekstomonthsCollagenvasculardiseaseassociatedILDCryptogenicorganizingpneumoniaDrugsSubacutehypersensitivitypneumonitis,Chronic:monthstoyearsIdiopathicpulmonaryfibrosisChronichypersensitivitypneumonitisCollagenvasculardiseaseassociatedILDNonspecificinterstitialpneumoniaOccupation-relatedlungdisease(e.g.,silicosis,asbestosis),CLINICALEVALUATION-History,OccupationalandEnvironmentalHistory:hypersensitivitypneumonitis,asbestosisDrugHistory:amiodaron,bleomycin,radiotherapyAgeandGender:young-sarcoidosis,IPF-50;women-lymphagioleiomyositosis(LM,淋巴管平滑肌瘤病);men-pneumoconiosis(尘肺)SmokingHistory:high-eosinophilicgranuloma,RB-ILD,IPF,asbestosis,;low-hypersensitivepneumonitis(HP过敏性肺炎),sarcoidosisFamilyHistory:familialIPF,Lymphangioleiomyomatosis,RespiratorySymptomsandSigns,Dyspnea:Progressivedyspnea,exertional/resting:themostcommoncomplaint.10%ILDmaypresentwithdyspneawithanormalchestradiograph.SuspectionafterexclusionofCOPD,PulmonaryEmbolism(肺栓塞).Cough:IPF,sarcoidosis,HP,COPChestPain:CTD-ILD,pneomothoraxwithLMWheezing:sarcoidosis,HP,EP,Bibasilarinspiratorycrackles(爆裂音):characteristicphysicalsigninILD.Mechanism.DryralesDigitalclubbing,(杵状指)amarkerofadvancedfibroticdisease,ofteninIPF;CAsuspectedifafter.,RespiratorySymptomsandSigns,LaboratoryInvestigation,Autoantibodies(rheumatoidfactor,antinuclearfactors)CVD,IPF,WG,MPA,NSIPSerumangiotensinconvertingenzyme(血管紧张素转化酶抑制剂):SarcoidosisEosinophilia:EPAntibasementmembraneantibody:GPSAntineutrophiliccytoplasmicantibody(ANCA,抗中性粒细胞胞浆抗体):WG,MPAIncreasedserumLDH:IPF,PAP,AIP,RadiographicFeatures,Grouns-glassopacipation磨玻璃影Reticularornodular网格/结节影Honey-combing蜂窝影Cyst囊检查方式PlainCXRPlainCTHRCT:crucialforILDdiagnosis,Alveolarfillingpattern肺泡填塞征,Nodularpattern,Honeycomb,Reticularpattern-HP,Nodule-Sarcoidosis,Honey-combing蜂窝肺,IPF,GGOwiththickenedinterlobularsepta-crazypavingfashion(铺路石征),pulmonaryalveolarproteinosis,Ground-glassopacity-idiopathicpulmonaryhemosiderosis,Cyst囊,Lymphangioleiomyomatosis,BronchoalveolarLavage肺泡灌洗,Diagnosis:E35%eosinophilicpneumonia嗜酸性粒细胞肺炎periodicacidSchiff(PAS)+:alveolarproteinosis肺泡蛋白沉积正BALlymphocytes(35%)sarcoidosis,HP,drug-inducedILDResponsetotherapy:lymphocytosis,BAL-Alveolarproteinosis,PhysiologicTesting,Restriction限制性通气功能障碍Diffusiondefect弥散量降低PreservationofairflowIncreaseinP(A-a)O2Exercise-inducedhypoxaemia低氧血症Hyperventilation过度通气,PulmonaryFunctionTest,LungBiopsy-definitivediagnosis,ThefinalstepinthediagnosticevaluationofapatientwithILDistodecidewhetheritisnecessarytoobtainlungtissue.TranbronchialLungBiopsy经气管镜肺活检Percutaneouslungbiopsy经皮肺活检Video-assitedthoracosopiclungbiopsy胸腔镜活检Openlungbiopsy开胸肺活检,Diagnosis,Multi-disciplinaryapproachtothediagnosisofILDwithPulmonologist,radiologistandpathologist.,IDIOPATHICPULMONARYFIBROSIS(ipf),特发性肺纤维化,IDIOPATHICINTERSTITIALPNEUMONIA(IIP)特发性间质性肺炎,TheIIPsareaheterogeneousgroupofnonneoplasticdisordersresultingfromdamagetothelungparenchymabyvaryingpatternsofinflammationandfibrosis.Idiopathicindicatesunknowncauseandinterstitialpneumoniareferstoinvolvementofthelungparenchymabyvaryingcombinationsoffibrosisandinflammation,incontrasttoairspacediseasetypicallyseeninbacterialpneumonia.,IIP的生存曲线,IPF-Definition,IPFisdefinedasaspecificformofchronic,progressivefibrosinginterstitialpneumoniaofunknowncause,occurringprimarilyinolderadults,limitedtothelungs,andassociatedwiththehistopathologicand/orradiologicpatternofUIPdefinedbelow.ThedefinitionofIPFrequirestheexclusionofotherformsofinterstitialpneumoniaincludingotherdiopathicinterstitialpneumoniasandILDassociatedwithenvironmentalexposure,medication,orsystemicdisease.,PathologyofIPF,PotentialRiskFactors,CigarettesmokingEnvironmentalfactorMicrobialagentsGastroesophagealreflux,ClinicalFeatures,Symptoms&SignsExertionaldyspneaCoughBasilarcrackle,Lab&othertest,DEFINITIONOFUIPPATTERN,UIP征象,UIP可能征象,非UIP征象,OTHERTESTS,Pulmonaryfunctiontest:restriction+diffusiondefectwithdecreasedcomplianceArterialBloodGasAnalysis:IncreasedP(A-a),ExertionalhypoxaemiawithhypocapniaBronchoscopy:tranbronchoscopiclungbiopsy(TBLB,经气管镜肺活检):exclusionofotherdiseasesLungbiopsy:percutaneous,thoracoscopy,open-lungbiopsy,DiagnosticCriteria,ThediagnosisofIPFrequiresthefollowing:1.ExclusionofotherknowncausesofILD(e.g.,domesticandoccupationalenvironmentalexposures,connectivetissuedisease,anddrugtoxicity).2.ThepresenceofaUIPpatternonHRCTinpatientsnotsubjectedtosurgicallungbiopsy.3.SpecificcombinationsofHRCTandsurgicallungbiopsypatterninpatientssubjectedtosurgicallungbiopsy.,COMBINATIONOFHRCTANDSURGICALLUNGBIOPSYFORTHEDIAGNOSISOFIPF,IPF诊断标准,未,IPF诊断标准,未,IPF诊断标准,未,FeaturesassociatedwithincreasedmortalityinIPF,BaselinefactorsLevelofdyspneahoneycombingonHRCTPulmonaryhypertensionDLCO,40%predictedDesaturation10%absolutevalueDecreaseinDLCOby15%absolutevalueWorseningoffibrosisonHRCT,AcuteExacerbationofIPF(AEIPF),CriteriaforAEIPFhaveincludedanunexplainedworseningofdyspneawithin1month,evidenceofhypoxemiaasdefinedbyworsenedorseverelyimpairedgasexchange,newradiographicalveolarinfiltrates,andanabsenceofanalternativeexplanationsuchasinfection,pulmonaryembolism,pneumothorax,orheartfailure.,TherapyforIPF,IPFEvidence-BasedTreatment:,Strongrecommendationagainsttheuse:Corticosteroidmonotherapy(verylow)Colchicine(verylow)秋水仙碱CyclosporineA(verylow)环孢霉素Combinedcorticosteroidandimmune-modulatortherapy(low)激素+免疫调节剂Interferongamma1b(high)干扰素Bosentan(moderate)波生坦,IPF药物治疗,强烈不推荐下列治疗方案:单一激素疗法秋水仙碱环保霉素A激素+免疫抑制剂INFgamma波生坦,IPF药物治疗,不主张推荐下列药物:乙酰半胱氨酸+激素+免疫抑制剂乙酰半胱氨酸抗凝治疗吡啡尼酮,乙酰半胱氨酸对IPF的作用IFIGENIA,Demedtsetal.NEJM2005,吡啡尼酮对FVC的影响CAPACITY1,P=0.005*,*RankANCOVA(PFD2403mg/dvs.placeboatWeek72),P.Nobleetal,ATS2009,吡啡尼酮对FVC的影响CAPACITY2剂量反应曲线,Changein%PredictedFVC,P.Noble,ATS2009,吡啡尼酮对无恶化IPF生存的影响-荟萃分析,吡有效吡无效,华法令与IPF,INF-与IPF,InterpretationWecannotrecommendtreatmentwithinterferongamma-1bsincethedrugdidnotimprovesurvivalforpatientswithidiopathicpulmonaryfibrosis,whichrefutespreviousfindingsfromsubgroupanalysesofsurvivalinstudiesofpatientswithmild-to-moderatephysiologicalimpairmentofpulmonaryfunction.,其它治疗,氧疗与对症治疗康复肺移植中医中药,NatureHistoryofIPF,Sarcoidosis,PulmonarySarcoidosis,Sarcoidosisisamultisystemdisorderofunknownorigincharacterizedbynoncaseatinggranulomatousinflammationatsitesofdisease.Lungandintrathoraciclymphnodesmostcommonlyaffected,结节病的发展进程,肉芽肿性炎症,吸收,复发,治疗/观察,宿主因素,纤维化并不可逆改变,CLINICALFEATURES,Thehilarglandsandthelungsaretheorgansmostcommonlyaffectedinsarcoidosisandintrathoracicinvolvementisthemostfrequentaccompanimentofsarcoidosisaffectingotherorgans.Ageandsex:thehighestincidenceinthethirdandfourthdecades,withavariabl
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