间质性肺炎英文.ppt

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 GRANULOMATOSISsarcoidosisHypersensitivepneumonitisWegener sgranulomatosis RAREILDalv proteinosisalv 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 weekstomonthsCollagenvasculardisease associatedILDCryptogenicorganizingpneumoniaDrugsSubacutehypersensitivitypneumonitis Chronic monthstoyearsIdiopathicpulmonaryfibrosisChronichypersensitivitypneumonitisCollagenvasculardisease associatedILDNonspecificinterstitialpneumoniaOccupation 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 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 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 absolutevalueDecreaseinDLCOby 15 absolutevalueWorseningoffibrosisonHRCT AcuteExacerbationofIPF AEIPF CriteriaforAEIPFhaveincludedanunexplainedworseningofdyspneawithin1month evidenceofhypoxemiaasdefinedbyworsenedorseverelyimpairedgasexchange newradiographicalveolarinfiltrates andanabsenceofanalternativeexplanationsuchasinfection pulmonaryembolism pneumothorax orheartfailure TherapyforIPF IPF 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 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 withavariablefemalepredominance NotdiagnosticPulmonary cough expectorationofsputun breathlessness hemoptysisExtrathoracic skin erythemanodosum neurological cardiac etcDiagnosisbyaccident CLINICALFEATURES Labtest Haemopoieticsystem increasedESR anemia hypercalcimia increasedLDH increasedactivityofserumangiot