间质性肺炎ILD英文课件-Interstitial-Lung-Disease

Non-infectiousInterstitialLungDiseaseBahmanSaatian,M.D.PulmonaryCriticalCareMedicineUCIrvineMedicalCenterOBJECTIVES•ReviewthespectrumofILD•Identifythecluesonpresentationtomakethediagnosis•ReviewthecommonradiographicfindingsinILD•RoleofBAL,TBBXandOLBindiagnosisofILD•AnalgorithmtomakethediagnosisILDsareheterogeneousgroupofdisordersthatareclassifiedtogetherbecauseofsimilarclinical,physiologicradiographic,orpathologicmanifestation.PulmonaryInterstitium•Alveolarliningcells(types1and2)•Thinelastin-richconnectivecomponentcontainingcapillarybloodvessels.WhatisPulmonaryInterstitium?•Betweentheepithelialandendothelialbasementmembrane•Expansionoftheinterstitiumcompartmentbyinflammationwithorwithoutfibrosis-Necrosis-Hyperplasia-Collapseofbasementmembrane-Inflammatorycells•Interstitialreflectsthepathologicalabnormalitybeginsintheinterstitium→extensivealterationofalveolarandairwayarchitecture.Pathogenesis•ThepathogenesisofILDisunknown.•StudieshaveshownthatimmunecellsandtheircytokinesplayanimportantroleinthecourseofILD.Pathogenesis•ThepathogenesisofILDisunknown.•StudieshaveshownthatimmunecellsandtheircytokinesplayanimportantroleinthecourseofILD.•Intra-alveolarinflammation.•Immunecellsandtheircytokinesinjureepithelialandendothelialcells.•Intra-alveolarfibrosis/collapseClassificationClassification•Associatedwithknowncauses•IdiopathicTreatmentchoicesandprognosisvaryamongdifferenttypesofILDs.Coultas,etal.AJRCCM,1994,150,679-72.ClassificationofidiopathicInterstitialPneumoniaAnOfficialAmericanThoracicSociety/EuropeanRespiratorySocietyStatement:UpdateoftheInternationalMultidisciplinaryClassificationoftheIdiopathicInterstitialPneumonias,2013ClinicalAssessment•History•PhysicalExam•ChestRadiograph•PulmonaryFunctionTesting•LaboratoryStudies•TissueExaminationClinicalManifestation•Progressivedyspnea•Drycough•Othersymptomssuchasfatigue,weightloss,drymouth,rash,jointpain,etc.HISTORY•Ageandgender•Onsetofsymptoms•Pastmedicalhistory•Smokinghistory•Familyhistory•Priormedicationuseandirradiation•Occupationalandenvironmentalexposures•Age:SomeoftheILDsaremorecommonincertainagegroups:Age20-40years-Sarcoidosis-CTD-LAM-EG50years-Idiopathicpulmonaryfibrosis•Gender-Premenopausalfemale:LAM(lymphangioleiomatosis)-Femalepredominant:ILDassociatedwithCTD.-Malepredominant:ILDassociatedwithRAPneumoconiosis•Acute(daystoweeks)-Acuteidiopathicinterstitialpneumonia(AIP)-Eosinophilicpneumonia-Hypersensitivitypneumonitis-BOOP,Drug,CVD•Subacute(weekstomonths)-Sarcoidosis-Somedrug-inducedILDs-Alveolarhemorrhagesyndromes-COP,HSP,CellularNSIP-Connectivetissuedisease(systemiclupuserythematosusorpolymyositis)•Chronic(monthstoyears)-IPF/UIP,FibroticNSIP,Pneumoconiosis,chronicHSP,CVD,-Sarcoidosis-smokingrelated-ILD(PLCH,RB-ILD)DurationofIllnessSMOKING-HistiocytosisX-DIP-RB-ILDDiseaseslesslikelytobeseeninsmoker:-Hypersensditivitypneumonitis-Sarcoidosis•InGoodpasture'ssyndrome:100%ofsmokersvs.20%ofnonsmokersexperiencepulmonaryhemorrhage.•Smokingincreasestheriskofasbestosisinindividualswithhistoryofasbestosexposure.AntibioticsNitrofurantoin,acuteandchronicSulfasalazineAnti-inflammatoryagentsAspirinGoldPencillamineChemotherapeuticagentsAntibioticsBleomycinsulfateMitomycinCAlkylatingagentsBusulfanCyclophosphamideChlorambucilMelphalanAntimetabolitesAzathioprineCytosinearabinosideMethotrexateDrug-InducedInterstitialLungDiseaseMiscellaneousO2DrugsinducingpulmonaryinfiltratesandeosinophiliaRadiationL-tryptophanDrug-inducedsystemiclupuserythematosusProcainamidehydrochlorideIsoniazidHydralazinehydrochlorideThehydantoinsPencillamineIllicitdrugsHeroinMethadonehydrochloridePropoxyphenehydrochloride(Darvon)TalcPHYSICALEXAMINATION•Lungexamination•Cardiacexamination•Clubbing•Extrapulmonaryfindingsofsystemicdisease•Bilateralbasilarinspiratorycrackles•Wheezing,rhonchi,coarsecrackles•Withadvanceddiseasetachypnea,tachycardia•Atlast,pulmonaryhypertensionandcorpulmonalePHYSICALFINDINGLABORATORYTESTSLABORATORYTESTSAnti-JO-1Abevenintheabsenceofclinicalmyositis,asILDprecedestheonsetofmyositisinabout70%ofpatientswiththeanti-synthetasesyndrome.SERUMMARKERS•SurfactantproteinAandB(SP-A,SP-B)•Monocytechemoattractantprotein-1(MCP1)•KerbsVonLungren(KL)-6acirculating,HMWglycoproteinexpressedbytypeIIpneumocytesSERUMMARKERSEVALUATION•Radiographic-CXR-HRCT•Physiologictest-PFT-Exercisetest•Lungsampling-BAL-LungbiopsyCHESTRADIOGRAPHY•ImportantfordiagnosisILDs.•Correlationbetweenimagingandthestageofdisease(clinicalorpathologic)isgenerallypoor.•Honeycombing(smallcysticspaces)portendsapoorprognosis.•Reviewallpreviousimagingstudies.PatternsofInterstitialLungDisease•Inearlystage:adiffusegroundglasspattern•Progress:nodular,reticularoracombination•Latestage:fibrosis,honeycombchangesCHESTRADIOGRAPHYHRCT•Bothsupineandproneimagestorecognizedependentatelectasisandwithinterstitialopacities.•Providesgreaterdiagnosticaccuracy•Narrowsthedifferentialdiagnosis•B/lsymmetrichilaradenopathy/upperlungzonereticularopacities-Sarcoidosis-Granulomatousdisease•Pleuralplaqueswithlinearcalcification-Asbestosis•Centrilobularnodulesspa