间质性肺炎英文课件演示教学

间质性肺炎英文课件MedicalTerminologyInterstitiallungdisease（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/ERS2002IDIOPATHICINTERSTITIALPNEUMONIA(IIP)GRANULOMATOSISsarcoidosisHypersensitivepneumonitisWegener'sgranulomatosis,RAREILDteinosisalv.hemorrhage-nephritissyndromeLangerhanscellhistiocytosisLymphangioleiomyomatosisIdiopathicpulmonaryHemosiderosisChroniceosinophilicpneumoniaILDofknowncausesOccupationallungdisease(pneumoconiosis)Drug-inducedlungdiseaseConnectivediseaseassociatedILDILDofUnknownCausesAMERICANJOURNALOFRESPIRATORYANDCRITICALCAREMEDICINEVOL1652002EPIDEMIOLOGYILDaccountsfor100,000hospitaladmissionsyearly15%ofpatientsseenbypulmonologistsnationwide.incidenceandprevalenceofILD(5/100,000)intheUSNewMexico:overallprevalenceofILDwas80.9per100,000inmalesand67.2per100,000infemalesincidenceofILDwas31.5per100,000inmenand26.1per100,000inwomen.Idiopathicpulmonaryfibrosisrepresented45%ofthepatientbase.CLINICALEVALUATION--HistoryLengthOfIllnessAndClinicalCourseAcuteonset: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:weekstomonthsCollagenvasculardisease–associatedILDCryptogenicorganizingpneumoniaDrugsSubacutehypersensitivitypneumonitisChronic:monthstoyearsIdiopathicpulmonaryfibrosisChronichypersensitivitypneumonitisCollagenvasculardisease–associatedILDNonspecificinterstitialpneumoniaOccupation-relatedlungdisease(e.g.,silicosis,asbestosis)CLINICALEVALUATION--HistoryOccupationalandEnvironmentalHistory: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,LymphangioleiomyomatosisRespiratorySymptomsandSignsDyspnea:Progressivedyspnea,exertional/resting：themostcommoncomplaint.10%ILDmaypresentwithdyspneawithanormalchestradiograph.SuspectionafterexclusionofCOPD,PulmonaryEmbolism(肺栓塞).Cough:IPF,sarcoidosis,HP,COPChestPain:CTD-ILD,pneomothoraxwithLMWheezing:sarcoidosis,HP,EPBibasilarinspiratorycrackles(爆裂音):characteristicphysicalsigninILD.Mechanism.DryralesDigitalclubbing,(杵状指)amarkerofadvancedfibroticdisease,ofteninIPF;CAsuspectedifafter.RespiratorySymptomsandSignsLaboratoryInvestigationAutoantibodies(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,AIPRadiographicFeaturesGrouns-glassopacipation磨玻璃影Reticularornodular网格/结节影Honey-combing蜂窝影Cyst囊

检查方式PlainCXRPlainCTHRCT：crucialforILDdiagnosisAlveolarfillingpattern肺泡填塞征NodularpatternHoneycombReticularpattern-HPNodule-SarcoidosisHoney-combing蜂窝肺IPFGGOwiththickenedinterlobularsepta-crazypavingfashion(铺路石征）pulmonaryalveolarproteinosisGround-glassopacity-idiopathicpulmonaryhemosiderosisCyst囊LymphangioleiomyomatosisBronchoalveolarLavage肺泡灌洗Diagnosis:E>35%eosinophilicpneumonia嗜酸性粒细胞肺炎periodicacid–Schiff(PAS)+:alveolarproteinosis肺泡蛋白沉积正BALlymphocytes(>35%)sarcoidosis,HP,drug-inducedILDResponsetotherapy:lymphocytosisBAL-AlveolarproteinosisPhysiologicTestingRestriction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PulmonaryFunctionTestLungBiopsy-definitivediagnosisThefinalstepinthediagnosticevaluationofapatientwithILDistodecidewhetheritisnecessarytoobtainlungtissue.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DiagnosisMulti-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-DefinitionIPFisdefinedasaspecificformofchronic,progressivefibrosinginterstitialpneumoniaofunknowncause,occurringprimarilyinolderadults,limitedtothelungs,andassociatedwiththehistopathologicand/orradiologicpatternofUIPdefinedbelow.ThedefinitionofIPFrequirestheexclusionofotherformsofinterstitialpneumoniaincludingotherdiopathicinterstitialpneumoniasandILDassociatedwithenvironmentalexposure,medication,orsystemicdisease.Pathology

ofIPFPotentialRiskFactorsCigarettesmokingEnvironmentalfactorMicrobialagentsGastroesophagealrefluxClinicalFeaturesSymptoms&SignsExertionaldyspneaCoughBasilarcrackleLab&othertestDEFINITIONOFUIPPATTERNUIP征象UIP可能征象非UIP征象OTHERTESTSPulmonaryfunctiontest：restriction+diffusiondefectwithdecreasedcomplianceArterialBloodGasAnalysis：IncreasedP(A-a)，ExertionalhypoxaemiawithhypocapniaBronchoscopy：tranbronchoscopiclungbiopsy（TBLB，经气管镜肺活检）：exclusionofotherdiseasesLungbiopsy：percutaneous，thoracoscopy，open-lungbiopsyDiagnosticCriteriaThediagnosisofIPFrequiresthefollowing:1.ExclusionofotherknowncausesofILD(e.g.,domesticandoccupationalenvironmentalexposures,connectivetissuedisease,anddrugtoxicity).2.ThepresenceofaUIPpatternonHRCTinpatientsnotsubjectedtosurgicallungbiopsy.3.SpecificcombinationsofHRCTandsurgicallungbiopsypatterninpatientssubjectedtosurgicallungbiopsy.COMBINATIONOFHRCTANDSURGICALLUNGBIOPSYFORTHEDIAGNOSISOFIPFIPF诊断标准未IPF诊断标准未IPF诊断标准未FeaturesassociatedwithincreasedmortalityinIPFBaselinefactorsLevelofdyspneahoneycombingonHRCTPulmonaryhypertensionDLCO,40%predictedDesaturation<88%during6MWTExtentofLongitudinalfactorsIncreaseinlevelofdyspneaDecreaseinForcedVitalCapacityby>10%absolutevalueDecreaseinDLCOby>15%absolutevalueWorseningoffibrosisonHRCTAcuteExacerbationofIPF（AEIPF）CriteriaforAEIPFhaveincludedanunexplainedworseningofdyspneawithin1month,evidenceofhypoxemiaasdefinedbyworsenedorseverelyimpairedgasexchange,newradiographicalveolarinfiltrates,andanabsenceofanalternativeexplanationsuchasinfection,pulmonaryembolism,pneumothorax,orheartfailure.TherapyforIPFIPF–Evidence-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

–IFIGENIADemedtsetal.NEJM2005吡啡尼酮对FVC的影响CAPACITY1P=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%PredictedFVCP.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IPFINF-ᵞ与IPFInterpretationWecannotrecommendtreatmentwithinterferongamma-1bsincethedrugdidnotimprovesurvivalforpatientswithidiopathicpulmonaryfibrosis,whichrefutespreviousfindingsfromsubgroupanalysesofsurvivalinstudiesofpatientswithmild-to-moderatephysiologicalimpairmentofpulmonaryfunction.其它治疗氧疗与对症治疗康复肺移植中医中药NatureHistoryofIPFSarcoidosisPulmonarySarcoidosisSarcoidosisisamultisystemdisorderofunknownorigincharacterizedbynoncaseatinggranulomatousinflammationatsitesofdisease.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CLINICALFEATURESThehilarglandsandthelungsaretheorgansmostcommonlyaffectedinsarcoidosisandintrathoracicinvolvementisthemostfrequentaccompanimentofsarcoidosisaffectingotherorgans.Ageandsex:thehighestincidenceinthethirdandfourthdecades,withavariablefemalepredominance.NotdiagnosticPulmonary:cough,expectorationofsputun,breathlessness,hemoptysisExtrathoracic:skin(erythemanodosum),neurological,cardiac,etcDiagnosisbyaccidentCLINICALFEATURESLabtestHaemopoieticsystem:increasedESR,anemia,hypercalcimia,increasedLDH,increasedactivityofserumangiotensin-converti