內(nèi)科學(xué)英文課件：Bronchial-asthma

Bronchialasthma

支氣管哮喘BronchialasthmaThosetoberememberedThosetoberememberedMorbidityofasthmaMorbidityofasthmaMortalityofasthma在中國(guó)有1500萬(wàn)，全球3億哮喘患者，每100，000位哮喘患者中有36.7位哮喘患者會(huì)因哮喘死亡。Mortalityofasthma在中國(guó)有1500萬(wàn)，全DefinitionAsthmaisaheterogeneous

disease,characterizedbychronicairwayinflammation.Itisdefinedbyhistoryofrespiratorysymptomssuchaswheeze,shortnessofbreath,chesttightnessandcoughthatvaryintimeandintensity,togetherwithvariableexpiratoryflowlimitation.DefinitionAsthmaisaheteroge哮喘的定義Allergicinflammation氣道慢性變態(tài)反應(yīng)性炎癥Inflammatorycells由多種炎癥細(xì)胞：如嗜酸粒細(xì)胞、肥大細(xì)胞、T淋巴細(xì)胞、嗜中性粒細(xì)胞、Structuralcells氣道結(jié)構(gòu)細(xì)胞：平滑肌、氣道上皮細(xì)胞等）細(xì)胞組分(cellularelements)參與的氣道慢性炎癥性疾病慢性炎癥導(dǎo)致氣道高反應(yīng)性（airwayhypersensitivity）引起反復(fù)發(fā)作性的喘息、氣急、胸悶或咳嗽等癥狀，常在夜間和（或）清晨發(fā)作、加劇通常出現(xiàn)廣泛多變的可逆性氣流受限，多數(shù)患者可自行緩解或經(jīng)治療緩解

哮喘的定義Allergicinflammation氣道慢性PathogenesisGeneticfactorsAirwayimmunity-inflammationAllergicresponse-earlyasthmaticandlateasthmaticresponseairwayhyperresponsivenessairwayremodellingNeurologicaccomodationPathogenesisGeneticfactorsPathogenesisofasthma環(huán)境因素遺傳易感個(gè)體炎癥細(xì)胞、細(xì)胞因子及炎癥介質(zhì)相互作用氣道神經(jīng)調(diào)節(jié)失衡及氣道平滑肌結(jié)構(gòu)功能異常氣道炎癥氣道高反應(yīng)性癥狀性哮喘環(huán)境激發(fā)因子氣道高反應(yīng)性氣道炎癥氣道重建Pathogenesisofasthma環(huán)境因素遺傳易感咳嗽喘息呼吸困難

誘發(fā)因素：活動(dòng)后吸入冷空氣后夜間感冒后突然發(fā)作性體征呼氣時(shí)產(chǎn)生哮鳴音規(guī)律：節(jié)律性(夜間發(fā)作)季節(jié)性(春、秋)自然/用藥緩解Cardinalmanifestations-typical咳嗽喘息呼吸困難誘發(fā)因素：體征規(guī)律：CardinalCardinalfeatureofasthmaVariablesymptomsofwheeze,shortnessofbreath,chesttightnessand/orcoughVariableairflowlimitationBothsymptomsandairflowlimitationvaryovertimeandinintensityTriggers:exercise,allergen,irritantexposure,changeinweather,viralrespiratoryinfectionResolutionspontaneouslyorwithmedicationAirwayhyperresponsivenessandinflammationpersists,evenwhensymptomsareabsent/normallungfunctionCardinalfeatureofasthmaVariGenerallymorethan2typesofsymtomsSymptomsoccurvariablyovertimeandvaryinintensitySymptomsareworseatnightoronwakingSymptomsareoftentriggeredbyallergens,exercise,laughter,coldairSymptomsoftenappearorworsenwithviralinfectionCardinalmanifestationsGenerallymorethan2typesofAtypicalasthmaCoughorchesttightnessCough-variantasthma:coughonlyoftenatnightorearlyinthemorningTriggeredbyexercise\coldairRelievedwithbronchodilators\ICSAtypicalasthmaCoughorchest特殊類(lèi)型哮喘Exercise-inducedasthmaAspirinasthma：asthma,nasalpolypandaspirinintoleranceAsthma-COPDoverlapsyndrome特殊類(lèi)型哮喘Exercise-inducedasthmaAsthmaTRIGGERS

ENVIRONMENTALFACTORSAllergens?Indoor:Domesticmites,furredanimals(dogs,cats,mice),cockroachallergen,fungi,molds,yeasts?Outdoor:Pollens,fungi,molds,yeastsInfections(predominantlyviral)ExerciseDrugs:aspirinDiet:fish,milk,egg,prawn,crabPsychiatricOccupationalsensitizersAsthmaTRIGGERS

內(nèi)科學(xué)英文課件：Bronchial-asthma內(nèi)科學(xué)英文課件：Bronchial-asthmaLabTesingLungfunctiontest

Bronchodilationtest:airwayreversibilitypostFEV112%,?FEV1>200ml

Bronchialprovocationtest:bronchialhyperreactivitypost-FEV120%

PEPvariationSpecificallergentestFractionalExhalednitricoxide(FeNO)LabTesingLungfunctiontestLabtestingBloodeosinophilsChestX-rayBloodgasanalysisMild：PaCO正?；蚪档?，PaO2正常，pH正?；蚝粑詨A重度Severe：PaCO正?；蚪档?，PaO2正常，pH偏酸，呼吸性或代謝性酸中毒LabtestingBloodeosinophils1.反復(fù)發(fā)作性喘息、呼吸困難、胸悶或咳嗽，多與接觸變應(yīng)原、冷空氣、物理化學(xué)剌激、病毒感染、運(yùn)動(dòng)等有關(guān)

2.可聞哮鳴音

3.上述癥狀可經(jīng)治療或自行緩解

4.癥狀不典型者(如無(wú)明顯喘息和體征)至少應(yīng)有下列三項(xiàng)中的一項(xiàng)陽(yáng)性∶（1）支氣管激發(fā)試驗(yàn)或運(yùn)動(dòng)試驗(yàn)陽(yáng)性；（2）支氣管舒張?jiān)囼?yàn)陽(yáng)性；（3）呼氣流量峰值（PEF）日內(nèi)變異率或晝夜波動(dòng)率≥20％

5.除外其他疾病所引起的喘息、胸悶和咳嗽符合1～4條或4、5條者，可診斷咳嗽變異性哮喘：Diagnosis1.反復(fù)發(fā)作性喘息、呼吸困難、胸悶或咳嗽，多與接觸變應(yīng)SeverityandstagingSeverity:mild,moderate,severeStaging:Chronicpersistent:weeklyChronicremission:3monAcuteexacerbationSeverityandstagingSeverity:Severityof

asthma輕癥哮喘Mildasthma中度哮喘Moderateasthma重度哮喘severeasthmaGINAupdated2014Severityof

asthma輕癥哮喘中度哮喘重度哮喘表3哮喘急性發(fā)作時(shí)病情嚴(yán)重程度的分級(jí)

臨床特點(diǎn)輕度中度重度危重氣短

體位

講話方式

精神狀態(tài)

出汗

呼吸頻率

輔助呼吸肌活動(dòng)及三凹征

哮鳴音

脈率

奇脈

使用β2激動(dòng)劑后PEF預(yù)計(jì)值PaO2（吸空氣）

PaCO2

SaO2（吸空氣）

PH步行、上樓時(shí)

可平臥

連續(xù)成句

可有焦慮尚安靜

常無(wú)

輕度增加

常無(wú)

散在，呼吸末期

<100次／min

無(wú)，<10mmHg

>80％

正常

<45mmHg

>95％

稍事活動(dòng)

喜坐位

單詞

時(shí)有焦慮或煩燥

有

增加

可有

響亮、彌漫

100～120次可有

60％～80％

≥60mmHg

≤45mmHg

91％一95％

休息時(shí)

端坐呼吸

單字

常有焦慮、煩躁

大汗淋漓

常>30次／min

常有

響亮、彌漫

>120次／min

常有，

<60％或<100％

<60mmHg

>45mmHg

≤90％

不能講話

嗜睡或意識(shí)模糊

胸腹矛盾運(yùn)動(dòng)

減弱、乃到無(wú)

脈率變慢不規(guī)則

無(wú)，提示呼吸肌疲勞

降低

表3哮喘急性發(fā)作時(shí)病情嚴(yán)重程度的分級(jí)臨床特點(diǎn)輕度中度重度AsthmainspecialpopulationExercise-inducedasthmaDrug-inducedasthmaObesityasthmaOccupationalasthma>>>>>>>>>>>>>>>>>>>>AsthmainspecialpopulationExDifferentialdiagnosisLeftheartfailureCOPDUpperairwayobstructionAllergicbronchopulmonaryaspergillosisDifferentialdiagnosisLeftheaComplicationsPneumothoraxPneumomediastinumLungatelectasisBronchiectasisCOPDCorPulmonaleInterstitiallungdiseaseComplicationsPneumothoraxPharmacotherapyofasthmaRelieverInhaledshort-acting2agonistOralshort-acting2agonistAnticholinergicsMethylxanthinesSystemicsteroidControllerInhaledsteroidInhaledlong-acting2agonistOrallong-acting2agonistLeukotrienemodifierMethylxanthinesCromonesSystemicsteroidIgEAbImmunotherapyPharmacotherapyofasthmaRelieClassificationof?2agonists作用時(shí)間Classificationof?2agonists作Inhaledsteroid布地奈德氟替卡松二丙酸倍氯米松環(huán)索奈德Inhaledsteroid布地奈德氟替卡松二丙酸倍氯米松

Managementofasthma

一、治療目標(biāo)

1．達(dá)到并維持哮喘控制

2．保持正常活動(dòng)；包括運(yùn)動(dòng)能力

3．維持肺功能維持在接近正常水平

4．預(yù)防哮喘急性加重5．避免哮喘藥物的不良反應(yīng)

6．預(yù)防哮喘死亡，降低哮喘死亡率

Managementofasthma一CombinationtherapyInhaledcorticosteroid(ICS)+long-actingbetaagonist(LABA)fluticasone+salmeterol(Advair.Seretide舒利迭)Budesonide+formoterol(Symbicort信必可)CombinationtherapyInhaledcorMDIMDI內(nèi)科學(xué)英文課件：Bronchial-asthmaDiskhalerDiskhalerNebulizerNebulizerStep1:Asneededrelieverinhaler,orICS(riskexacerbation.Step2:lowdosereliever+SABAprn(ICS/LTA/theo)Step3:1or2controller+asneededreliever(lowICS+LABA/LTA/theo)Step4:2ormorecontroller+asneededreliever(moderateICS+LABA/LTA/theo)Step5:added-ontherapy:anti-IgE,thermoplasty,OCSStepwisecontrolforasthmaStep1:AsneededrelieverinhStepwisecontrolofasthmaandreducerisksStepwisecontrolofasthmaand哮喘的治療-達(dá)到哮喘控制為基礎(chǔ)哮喘的治療-達(dá)到哮喘控制為基礎(chǔ)LevelsofasthmacontrolDaytimesymptoms>2/wkNightawakingRelieverneeded>2/wkActivitylimitationNone1~2ofthose3~4ofthoseWellcontrolledPartlycontrolledUncontrolledGINAupdated2014Symptomspast4wksLevelofcontrolLevelsofasthmacontrolDaytim哮喘治療與評(píng)價(jià)多數(shù)哮喘從2級(jí)開(kāi)始，癥狀頻繁者3級(jí)2-4wk復(fù)診，后1-3月復(fù)診哮喘評(píng)價(jià)：良好控制/部分控制/未控制達(dá)到控制后維持3月后減量激素減量50%低劑量改為qd聯(lián)合用藥者，減激素50%哮喘治療與評(píng)價(jià)多數(shù)哮喘從2級(jí)開(kāi)始，癥狀頻繁者3級(jí)哮喘升級(jí)治療持續(xù)升級(jí)（2-3月）：初始治療反應(yīng)差短期升級(jí)（1-2周）：病毒感染、季節(jié)過(guò)敏原日間調(diào)整：含福莫特羅制劑，維持、緩解方案哮喘升級(jí)治療持續(xù)升級(jí)（2-3月）：初始治療反應(yīng)差哮喘長(zhǎng)期治療方案哮喘教育環(huán)境控制按需使用速效2-激動(dòng)劑按需使用速效2-激動(dòng)劑可選擇的控制藥物選用一種選用一種在第三級(jí)基礎(chǔ)上，選用一種或多種在第四級(jí)基礎(chǔ)上，加用一種低劑量ICS低劑量ICS加長(zhǎng)效2-激動(dòng)劑中/高劑量ICS加長(zhǎng)效2-激動(dòng)劑口服糖皮質(zhì)激素(最小劑量)白三烯調(diào)節(jié)劑/茶堿中/高劑量ICS白三烯調(diào)節(jié)劑抗IgE治療低劑量ICS加白三烯調(diào)節(jié)劑緩釋茶堿低劑量ICS加緩釋茶堿第一級(jí)第二級(jí)第三級(jí)第四級(jí)第五級(jí)升級(jí)降級(jí)5.FromtheGlobalStrategyforAsthmaManagementandPrevention,GlobalInitiativeforAsthma(GINA)2011.Availablefrom:/.哮喘長(zhǎng)期治療方案哮喘教育環(huán)境控制按需使用速效2-激動(dòng)控制部分控制未控制急性加重控制水平維持治療并明確最低治療級(jí)別考慮升級(jí)治療，已達(dá)到控制升級(jí)治療直至達(dá)到控制按急性加重治療治療措施治療級(jí)別降級(jí)升級(jí)第1級(jí)第2級(jí)第3級(jí)第4級(jí)第5級(jí)降級(jí)升級(jí)控制部分控制未控制急性加重控制水平維持治療并明確最低治療級(jí)別OthertherapiesAvoidanceoftriggersImmunotherapyEducationBiotherapy:monoclonalantibody:anti-IL5;anti-IL4α(blockIL13/IL14)BronchialthermoplastyOthertherapiesAvoidanceoftrOxygentherapySaO2>92%Bronchodilatortherapy:SABA+iprotropium+theophyllineSteroidtherapy:methylprednisone40-160mg/dManagementofacid-baseandelectrolytedisturbanceFluidtherapy:2500ml/dAntibiotictherapyManagementofcomplicationManagementofsevereexacerbationOxygentherapySaO2>92%ManagManagementofAsthmaExacerbationsinAcuteCareSettingManagementofAsthmaExacerbatOverallcontrolSymptomscontrolNormalactivityReducefuturerisks：AsthmaattackFixedairflowlimitationSideeffectsOverallcontrolofasthmaGINAupdated2014BatemanED,etal.JAllergyClinImmunol.2010;125(3):600-8.OverallcontrolSymptomscontroSummaryInflammatoryairwaydiseasewithBHRTriggersorenvironmentalexposureAsthmaticsymptomsAntiasthmaticdrugsManagementofasthma(includingexacerbation)SummaryInflammatoryairwaydisINTERSTITIALLUNGDISEASE彌漫性間質(zhì)性肺病INTERSTITIALLUNGDISEASE肺間質(zhì)肺間質(zhì)包含肺泡上皮與肺血管內(nèi)皮的空間，包含多種細(xì)胞如成纖維細(xì)胞、成纖維細(xì)胞母細(xì)胞、巨噬細(xì)胞，還有基質(zhì)包括膠原、彈力蛋白和糖蛋白).肺間質(zhì)肺間質(zhì)包含肺泡上皮與肺血管內(nèi)皮的空間，包含多種細(xì)胞如成Interstitium肺間質(zhì)的概念I(lǐng)nterstitium肺間質(zhì)的概念

肺臟基本功能單位與肺間質(zhì)肺臟基本功能單位與肺間質(zhì)肺容積縮小彌散量降低低氧血癥呼吸困難肺容積縮小彌散量降低低氧血癥呼吸困難間質(zhì)性肺病的概念間質(zhì)性肺?。↖nterstitialLungDisease，ILD）為累及肺泡壁以及肺泡周?chē)M織和其周?chē)Y(jié)構(gòu)的非感染、非腫瘤性一組疾病。

ILD可累及細(xì)支氣管與肺實(shí)質(zhì)，又被稱(chēng)為彌漫性實(shí)質(zhì)性肺病(DiffuseParenchymalLungDisease，DPLD)間質(zhì)性肺病的概念間質(zhì)性肺?。↖nterstitialLun間質(zhì)性肺病的共性肺容積縮小肺彌散功能減低呼吸困難，特別運(yùn)動(dòng)后運(yùn)動(dòng)后或疾病低氧血癥好影像學(xué)彌漫性改變病理學(xué)表現(xiàn)為肺實(shí)質(zhì)/間質(zhì)不同程度纖維化/炎癥間質(zhì)性肺病的共性肺容積縮小已知原因的DPLD職業(yè)性肺病藥物性肺病結(jié)締組織疾病相關(guān)性ILD特發(fā)性間質(zhì)性肺炎（IIP）肉芽腫所致DPLD結(jié)節(jié)病過(guò)敏性肺炎肉芽腫并血管炎少見(jiàn)DPLD肺泡蛋白沉積癥肺出血-腎炎綜合征淋巴管平滑肌肌瘤病朗格漢斯組織細(xì)胞增生癥特發(fā)性含鐵血黃素沉著癥慢性嗜酸細(xì)胞性肺炎彌漫性肺間質(zhì)疾?。―PLD）間質(zhì)性肺病的分類(lèi)已知原因的DPLD職業(yè)性肺病癥狀與體征

RespiratorySymptomsandSignsDyspnea:Progressivedyspnea,exertional/resting--themostcommoncomplaint.10%ILDmaypresentwithdyspneawithanormalchestradiograph.SuspectionafterexclusionofCOPD,Pulmonarythromboembolism.Cough:IPF,sarcoidosis,HP,COPChestPain:CTD-ILD,pneomothoraxwithLMWheezing:sarcoidosis,Hypersensitivitypneumonia癥狀與體征

RespiratorySymptomsandBibasilarinspiratorycrackles(爆裂音):characteristicphysicalsigninILD.Mechanism.DryralesDigitalclubbing,(杵狀指)amarkerofadvancedfibroticdisease,ofteninIPF;癥狀與體征RespiratorySymptomsandSignsBibasilarinspiratorycrackles實(shí)驗(yàn)室檢查

LaboratoryInvestigationAutoantibodies(rheumatoidfactor,antinuclearfactors)CVD,IPF,WG,MPA,NSIPSerumangiotensinconvertingenzyme:SarcoidosisEosinophilia:EosinophilicpneumoniaAntibasementmembraneantibody:Antineutrophiliccytoplasmicantibody:WG,MPAIncreasedserumLDH:IPF實(shí)驗(yàn)室檢查

LaboratoryInvestigation影像學(xué)檢查

RadiographicFeaturesGround-glassopacification毛玻璃影Reticularornodular網(wǎng)格/結(jié)節(jié)影Honey-combing蜂窩影影像學(xué)檢查

RadiographicFeaturesGroReticularpattern網(wǎng)格影Reticularpattern網(wǎng)格影Nodule-結(jié)節(jié)影Nodule-結(jié)節(jié)影Honey-combing蜂窩肺IPFHoney-combing蜂窩肺IPFGroundglassopacity(GGO)pulmonaryalveolarproteinosisGroundglassopacity(GGO)pulmoBronchoalveolarLavage肺泡灌洗Normal：細(xì)胞總數(shù)5~10x106，巨噬細(xì)胞85~90%，L10~15%，N+E1%Diagnosis:E>25%eosinophilicpneumoniaperiodicacid–Schiff(PAS)+:alveolarproteinosisBALlymphocytes(>35%)sarcoidosis,HP,LIP,drug-inducedILDResponsetotherapy:lymphocytosisBronchoalveolarLavage肺泡灌洗Norm內(nèi)科學(xué)英文課件：Bronchial-asthmaBAL-AlveolarproteinosisBAL-AlveolarproteinosisPhysiologicTestingRestrictionDiffusiondefectPreservationofairflowIncreaseinP(A-a)O2Exercise-inducedhypoxaemiaHyperventilationPhysiologicTestingRestrictionPFTPFTLungBiopsy-definitivediagnosisThefinalstepinthediagnosticevaluationofapatientwithILDistodecidewhetheritisnecessarytoobtainlungtissue.TranbronchialLungBiopsy經(jīng)氣管鏡肺活檢Percutaneouslungbiopsy經(jīng)皮肺活檢Video-assitedthoracosopiclungbiopsy胸腔鏡活檢Openlungbiopsy開(kāi)胸肺活檢LungBiopsy-definitivediagnosDiagnosisDiagnosisThyerapyAvoidanceofexposureSteroidtherapyAntifibrotictherapySymptomatictherapyOthersThyerapyAvoidanceofexposureIDIOPATHICPULMONARYFIBROSIS(IPF)特發(fā)性肺纖維化IDIOPATHICPULMONARYFIBROSIS(IDIOPATHICINTERSTITIALPNEUMONIA(IIP)

特發(fā)性間質(zhì)性肺炎IIPsareagroupofdiffuseparenchymallungdiseases(DPLDs),agroupalsodescribedasinterstitiallungdiseasesTheIIPsareaheterogeneousgroupofnonneoplasticdisordersresultingfromdamagetothelungparenchymabyvaryingpatternsofinflammationandfibrosis.Idiopathicindicatesunknowncauseandinterstitialpneumoniareferstoinvolvementofthelungparenchymabyvaryingcombinationsoffibrosisandinflammation,incontrasttoairspacediseasetypicallyseeninbacterialpneumonia.IDIOPATHICINTERSTITIALPNEUMO特發(fā)性間質(zhì)性肺炎（IIP）特發(fā)性肺纖維化（IPF/UIP）呼吸性細(xì)支氣管炎伴間質(zhì)性肺?。≧BILD）隱原性機(jī)化性肺炎（COP）脫屑型間質(zhì)性肺炎（DIP）急性間質(zhì)性肺炎（AIP）非特異性間質(zhì)性肺炎(NSIP)淋巴細(xì)胞間質(zhì)性肺炎(LIP)間質(zhì)性肺病的分類(lèi)主要IIP少見(jiàn)

IIP未能分類(lèi)的IIP特發(fā)性胸膜肺實(shí)質(zhì)的彈力纖維增生癥AmJRespirCritCareMedVol188,Iss.6,pp733–748,Sep15,2013特發(fā)性間質(zhì)性肺炎（IIP）特發(fā)性肺纖維化（IPF/UIP）呼ClassificationsofIIPsClassificationsofIIPs特發(fā)性間質(zhì)性肺炎的病理學(xué)特征特發(fā)性間質(zhì)性肺炎的病理學(xué)特征IIP的臨床病理相關(guān)分類(lèi)臨床-影像-病理診斷臨床和/或病理形態(tài)學(xué)類(lèi)型慢性至纖維化性IP特發(fā)性肺纖維化IPF特發(fā)性非特異性間質(zhì)性肺炎INSIP普通型間質(zhì)性肺炎UIP非特異性間質(zhì)性肺炎NSIP吸煙相關(guān)性IP呼吸性細(xì)支氣管炎-間質(zhì)性肺炎R(shí)B-ILD脫屑型間質(zhì)性肺炎DIP呼吸性細(xì)支氣管炎R(shí)B脫屑型間質(zhì)性肺炎DIP急性/亞急性IP隱源性機(jī)化性肺炎COP急性間質(zhì)性肺炎AIP機(jī)化性肺炎OP彌漫性肺泡損傷DADIIP的臨床病理相關(guān)分類(lèi)臨床-影像-病理診斷臨床和/或病理形根據(jù)病情IIP分類(lèi)根據(jù)病情IIP分類(lèi)Idiopathicpulmonaryfibrosis(IPF)isdefinedasaspecificformofchronic,progressivefibrosinginterstitialpneumoniaofunknowncause,occurringprimarilyinolderadults,andlimitedtothelungs.Itischaracterizedbyprogressiveworseningofdyspneaandlungfunctionandisassociatedwithapoorprognosis.IPFasadistinctclinicalentityassociatedwiththehistologicappearanceofusualinterstitialpneumonia(UIP).IPF-DefinitionIdiopathicpulmonaryfibrosisPotentialRiskFactorsGeneticfactorsCigarettesmokingEnvironmentalfactorMicrobialagentsGastroesophagealrefluxPotentialRiskFactorsGeneticClinicalFeaturesSymptoms&SignsExertionaldyspneaCoughClubbingfingerBasilarcrackle（Velcro啰音）ClinicalFeaturesSymptoms&SignHRCT診斷UIP征象UIP征象(所有4項(xiàng))可能UIP征象(所有3項(xiàng))不符合UIP征象(任何1項(xiàng))胸膜下、基地分布網(wǎng)格樣改變蜂窩肺+伴支氣管擴(kuò)張無(wú)不符合UIP征象胸膜下、基地分布網(wǎng)格樣改變無(wú)不符合UIP征象上或中肺野分布支氣管周?chē)植紡V泛磨玻璃陰影為主廣泛微結(jié)節(jié)分散囊樣病變彌漫馬賽克征肺支氣管葉、段實(shí)變SubjectedtoexternalreviewHRCT診斷UIP征象UIP征象可能UIP征象不符合UIDEFINITIONOFUIPPATTERNDEFINITIONOFUIPPATTERNInconsistentwithUIPpatternInconsistentwithUIPpatternUIP的病理診斷典型UIP不符合UIP很可能UIP可能UIPUIP的病理診斷典型UIP不符合UIP很可能UIP可能UIPDiagnosisofIPFDiagnosisofIPFIPF診斷標(biāo)準(zhǔn)

DiagnosticCriteria1.除外其它已知原因ILD(如室內(nèi)或職業(yè)性環(huán)境暴露，結(jié)締組織病、藥物毒性等).2.HRCT具備典型UIP特征無(wú)需性外科肺活檢。3.不典型需要結(jié)合HRCT及外科活檢病理特征。肺功能、癥狀、體征---輔助作用IPF診斷標(biāo)準(zhǔn)

DiagnosticCriteria1.影像-病理-臨床診斷影像-病理-臨床診斷AcuteExacerbationofIPF（AEIPF）CriteriaforAEIPF:unexplainedworseningofdyspneawithin1month,Hypoxemianewradiographicalveolarinfiltratesabsenceofanalternativeexplanationinfectionpulmonaryembolismpneumothoraxheartfailure.AcuteExacerbationofIPF（AEIPTherapyforIPFTherapyforIPFIPF–Evidence-BasedTreatment:Strongrecommendationagainsttheuse:Corticosteroidmonotherapy(verylow)Colchicine(verylow)秋水仙堿CyclosporineA(verylow)環(huán)孢霉素Combinedcorticosteroidandimmune-modulatortherapy(low)激素+免疫調(diào)節(jié)劑Interferongamma1b(high)干擾素Bosentan(moderate)波生坦IPF–Evidence-BasedTreatment激素+免疫調(diào)節(jié)劑無(wú)效

NEnglJMed2012;366:1968-77.激素+免疫調(diào)節(jié)劑無(wú)效

NEnglJMed2012;3華法令與IPF華法令與IPF吡非尼酮