內科學英文課件：Bronchial asthma

Bronchialasthma

支氣管哮喘BronchialasthmaThosetoberememberedThosetoberememberedMorbidityofasthmaMorbidityofasthmaMortalityofasthma在中國有1500萬，全球3億哮喘患者，每100，000位哮喘患者中有36.7位哮喘患者會因哮喘死亡。Mortalityofasthma在中國有1500萬，全DefinitionAsthmaisaheterogeneous

disease,characterizedbychronicairwayinflammation.Itisdefinedbyhistoryofrespiratorysymptomssuchaswheeze,shortnessofbreath,chesttightnessandcoughthatvaryintimeandintensity,togetherwithvariableexpiratoryflowlimitation.DefinitionAsthmaisaheteroge哮喘的定義Allergicinflammation氣道慢性變態(tài)反應性炎癥Inflammatorycells由多種炎癥細胞：如嗜酸粒細胞、肥大細胞、T淋巴細胞、嗜中性粒細胞、Structuralcells氣道結構細胞：平滑肌、氣道上皮細胞等）細胞組分(cellularelements)參與的氣道慢性炎癥性疾病慢性炎癥導致氣道高反應性（airwayhypersensitivity）引起反復發(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，多數患者可自行緩解或經治療緩解

哮喘的定義Allergicinflammation氣道慢性PathogenesisGeneticfactorsAirwayimmunity-inflammationAllergicresponse-earlyasthmaticandlateasthmaticresponseairwayhyperresponsivenessairwayremodellingNeurologicaccomodationPathogenesisGeneticfactorsPathogenesisofasthma環(huán)境因素遺傳易感個體炎癥細胞、細胞因子及炎癥介質相互作用氣道神經調節(jié)失衡及氣道平滑肌結構功能異常氣道炎癥氣道高反應性癥狀性哮喘環(huán)境激發(fā)因子氣道高反應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：活動后吸入冷空氣后夜間感冒后突然發(fā)作性體征呼氣時產生哮鳴音規(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特殊類型哮喘Exercise-inducedasthmaAspirinasthma：asthma,nasalpolypandaspirinintoleranceAsthma-COPDoverlapsyndrome特殊類型哮喘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

內科學英文課件：Bronchialasthma內科學英文課件：BronchialasthmaLabTesingLungfunctiontest

Bronchodilationtest:airwayreversibilitypostFEV112%,?FEV1>200ml

Bronchialprovocationtest:bronchialhyperreactivitypost-FEV120%

PEPvariationSpecificallergentestFractionalExhalednitricoxide(FeNO)LabTesingLungfunctiontestLabtestingBloodeosinophilsChestX-rayBloodgasanalysisMild：PaCO正?；蚪档?，PaO2正常，pH正常或呼吸性堿重度Severe：PaCO正常或降低，PaO2正常，pH偏酸，呼吸性或代謝性酸中毒LabtestingBloodeosinophils1.反復發(fā)作性喘息、呼吸困難、胸悶或咳嗽，多與接觸變應原、冷空氣、物理化學剌激、病毒感染、運動等有關

2.可聞哮鳴音

3.上述癥狀可經治療或自行緩解

4.癥狀不典型者(如無明顯喘息和體征)至少應有下列三項中的一項陽性∶（1）支氣管激發(fā)試驗或運動試驗陽性；（2）支氣管舒張試驗陽性；（3）呼氣流量峰值（PEF）日內變異率或晝夜波動率≥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.反復發(fā)作性喘息、呼吸困難、胸悶或咳嗽，多與接觸變應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)作時病情嚴重程度的分級

臨床特點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

輔助呼吸肌活動及三凹征

哮鳴音

脈率

奇脈

使用β2激動劑后PEF預計值PaO2（吸空氣）

PaCO2

SaO2（吸空氣）

PH步行、上樓時

可平臥

連續(xù)成句

可有焦慮尚安靜

常無

輕度增加

常無

散在，呼吸末期

<100次／min

無，<10mmHg

>80％

正常

<45mmHg

>95％

稍事活動

喜坐位

單詞

時有焦慮或煩燥

有

增加

可有

響亮、彌漫

100～120次可有

60％～80％

≥60mmHg

≤45mmHg

91％一95％

休息時

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

嗜睡或意識模糊

胸腹矛盾運動

減弱、乃到無

脈率變慢不規(guī)則

無，提示呼吸肌疲勞

降低

表3哮喘急性發(fā)作時病情嚴重程度的分級臨床特點輕度中度重度AsthmainspecialpopulationExercise-inducedasthmaDrug-inducedasthmaObesityasthmaOccupationalasthma>>>>>>>>>>>>>>>>>>>>AsthmainspecialpopulationExDifferentialdiagnosisLeftheartfailureCOPDUpperairwayobstructionAllergicbronchopulmonaryaspergillosisDifferentialdiagnosisLeftheaComplicationsPneumothoraxPneumomediastinumLungatelectasisBronchiectasisCOPDCorPulmonaleInterstitiallungdiseaseComplicationsPneumothoraxPharmacotherapyofasthmaRelieverInhaledshort-acting2agonistOralshort-acting2agonistAnticholinergicsMethylxanthinesSystemicsteroidControllerInhaledsteroidInhaledlong-acting2agonistOrallong-acting2agonistLeukotrienemodifierMethylxanthinesCromonesSystemicsteroidIgEAbImmunotherapyPharmacotherapyofasthmaRelieClassificationof?2agonists作用時間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

1．達到并維持哮喘控制

2．保持正常活動；包括運動能力

3．維持肺功能維持在接近正常水平

4．預防哮喘急性加重5．避免哮喘藥物的不良反應

6．預防哮喘死亡，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內科學英文課件：BronchialasthmaDiskhalerDiskhalerNebulizerNebulizerStep1: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-達到哮喘控制為基礎哮喘的治療-達到哮喘控制為基礎LevelsofasthmacontrolDaytimesymptoms>2/wkNightawakingRelieverneeded>2/wkActivitylimitationNone1~2ofthose3~4ofthoseWellcontrolledPartlycontrolledUncontrolledGINAupdated2014Symptomspast4wksLevelofcontrolLevelsofasthmacontrolDaytim哮喘治療與評價多數哮喘從2級開始，癥狀頻繁者3級2-4wk復診，后1-3月復診哮喘評價：良好控制/部分控制/未控制達到控制后維持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%哮喘治療與評價多數哮喘從2級開始，癥狀頻繁者3級哮喘升級治療持續(xù)升級（2-3月）：初始治療反應差短期升級（1-2周）：病毒感染、季節(jié)過敏原日間調整：含福莫特羅制劑，維持、緩解方案哮喘升級治療持續(xù)升級（2-3月）：初始治療反應差哮喘長期治療方案哮喘教育環(huán)境控制按需使用速效2-激動劑按需使用速效2-激動劑可選擇的控制藥物選用一種選用一種在第三級基礎上，選用一種或多種在第四級基礎上，加用一種低劑量ICS低劑量ICS加長效2-激動劑中/高劑量ICS加長效2-激動劑口服糖皮質激素(最小劑量)白三烯調節(jié)劑/茶堿中/高劑量ICS白三烯調節(jié)劑抗IgE治療低劑量ICS加白三烯調節(jié)劑緩釋茶堿低劑量ICS加緩釋茶堿第一級第二級第三級第四級第五級升級降級5.FromtheGlobalStrategyforAsthmaManagementandPrevention,GlobalInitiativeforAsthma(GINA)2011.Availablefrom:/.哮喘長期治療方案哮喘教育環(huán)境控制按需使用速效2-激動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別考慮升級治療，已達到控制升級治療直至達到控制按急性加重治療治療措施治療級別降級升級第1級第2級第3級第4級第5級降級升級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彌漫性間質性肺病INTERSTITIALLUNGDISEASE肺間質肺間質包含肺泡上皮與肺血管內皮的空間，包含多種細胞如成纖維細胞、成纖維細胞母細胞、巨噬細胞，還有基質包括膠原、彈力蛋白和糖蛋白).肺間質肺間質包含肺泡上皮與肺血管內皮的空間，包含多種細胞如成Interstitium肺間質的概念Interstitium肺間質的概念

肺臟基本功能單位與肺間質肺臟基本功能單位與肺間質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性肺病的概念間質性肺?。↖nterstitialLungDisease，ILD）為累及肺泡壁以及肺泡周圍組織和其周圍結構的非感染、非腫瘤性一組疾病。

ILD可累及細支氣管與肺實質，又被稱為彌漫性實質性肺病(DiffuseParenchymalLungDisease，DPLD)間質性肺病的概念間質性肺?。↖nterstitialLun間質性肺病的共性肺容積縮小肺彌散功能減低呼吸困難，特別運動后運動后或疾病低氧血癥好影像學彌漫性改變病理學表現(xiàn)為肺實質/間質不同程度纖維化/炎癥間質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締組織疾病相關性ILD特發(fā)性間質性肺炎（IIP）肉芽腫所致DPLD結節(jié)病過敏性肺炎肉芽腫并血管炎少見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胞增生癥特發(fā)性含鐵血黃素沉著癥慢性嗜酸細胞性肺炎彌漫性肺間質疾?。―PLD）間質性肺病的分類已知原因的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實驗室檢查

LaboratoryInvestigationAutoantibodies(rheumatoidfactor,antinuclearfactors)CVD,IPF,WG,MPA,NSIPSerumangiotensinconvertingenzyme:SarcoidosisEosinophilia:EosinophilicpneumoniaAntibasementmembraneantibody:Antineutrophiliccytoplasmicantibody:WG,MPAIncreasedserumLDH:IPF實驗室檢查

LaboratoryInvestigation影像學檢查

RadiographicFeaturesGround-glassopacification毛玻璃影Reticularornodular網格/結節(jié)影Honey-combing蜂窩影影像學檢查

RadiographicFeaturesGroReticularpattern網格影Reticularpattern網格影Nodule-結節(jié)影Nodule-結節(jié)影Honey-combing蜂窩肺IPFHoney-combing蜂窩肺IPFGroundglassopacity(GGO)pulmonaryalveolarproteinosisGroundglassopacity(GGO)pulmoBronchoalveolarLavage肺泡灌洗Normal：細胞總數5~10x106，巨噬細胞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內科學英文課件：BronchialasthmaBAL-AlveolarproteinosisBAL-AlveolarproteinosisPhysiologicTestingRestrictionDiffusiondefectPreservationofairflowIncreaseinP(A-a)O2Exercise-inducedhypoxaemiaHyperventilationPhysiologicTestingRestrictionPFTPFTLungBiopsy-definitivediagnosisThefinalstepinthediagnosticevaluationofapatientwithILDistodecidewhetheritisnecessarytoobtainlungtissue.TranbronchialLungBiopsy經氣管鏡肺活檢Percutaneouslungbiopsy經皮肺活檢Video-assitedthoracosopiclungbiopsy胸腔鏡活檢Openlungbiopsy開胸肺活檢LungBiopsy-definitivediagnosDiagnosisDiagnosisThyerapyAvoidanceofexposureSteroidtherapyAntifibrotictherapySymptomatictherapyOthersThyerapyAvoidanceofexposureIDIOPATHICPULMONARYFIBROSIS(IPF)特發(fā)性肺纖維化IDIOPATHICPULMONARYFIBROSIS(IDIOPATHICINTERSTITIALPNEUMONIA(IIP)

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

IIP未能分類的IIP特發(fā)性胸膜肺實質的彈力纖維增生癥AmJRespirCritCareMedVol188,Iss.6,pp733–748,Sep15,2013特發(fā)性間質性肺炎（IIP）特發(fā)性肺纖維化（IPF/UIP）呼ClassificationsofIIPsClassificationsofIIPs特發(fā)性間質性肺炎的病理學特征特發(fā)性間質性肺炎的病理學特征IIP的臨床病理相關分類臨床-影像-病理診斷臨床和/或病理形態(tài)學類型慢性至纖維化性IP特發(fā)性肺纖維化IPF特發(fā)性非特異性間質性肺炎INSIP普通型間質性肺炎UIP非特異性間質性肺炎NSIP吸煙相關性IP呼吸性細支氣管炎-間質性肺炎RB-ILD脫屑型間質性肺炎DIP呼吸性細支氣管炎RB脫屑型間質性肺炎DIP急性/亞急性IP隱源性機化性肺炎COP急性間質性肺炎AIP機化性肺炎OP彌漫性肺泡損傷DADIIP的臨床病理相關分類臨床-影像-病理診斷臨床和/或病理形根據病情IIP分類根據病情IIP分類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)可能UIP征象(所有3項)不符合UIP征象(任何1項)胸膜下、基地分布網格樣改變蜂窩肺+伴支氣管擴張無不符合UIP征象胸膜下、基地分布網格樣改變無不符合UIP征象上或中肺野分布支氣管周圍分布廣泛磨玻璃陰影為主廣泛微結節(jié)分散囊樣病變彌漫馬賽克征肺支氣管葉、段實變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診斷標準

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

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+免疫調節(jié)劑Interferongamma1b(high)干擾素Bosentan(moderate)波生坦IPF–Evidence-BasedTreatment激素+免疫調節(jié)劑無效

NEnglJMed2012;366:1968-77.激素+免疫調節(jié)劑無效

NEnglJMed2012;3華法令與IPF華法令與IPF吡非尼酮Pirfenidone吡非尼酮Pirfenidone可以抑制TGF-beta-1誘導的人纖維母細胞膠原的生成。抑制由血小板源性生長因子、成纖維細胞生長因子FGF和轉化生長因子TGF-beta-1誘導的人纖維母細胞的增殖。吡非尼酮Pirfenidone吡非尼酮Pirfenidone吡非尼酮治療IPF有效NEnglJMed2014;370:2083-92.吡非尼酮治療IPF有效NEnglJMed2014;3尼達尼布(nintedanib)尼達尼布(nintedanib)為三重酪氨酸激酶抑制劑,靶點包括血小板源生長因子(PDGF)、血管內皮生長因子(VEGF)和堿性纖維母細胞生長因子(bFGF),也可抑制MAPK和Akt激活尼達尼布(nintedanib)尼達尼布(nintedaniNintedanib（尼達尼布）治療IPF有效NEnglJMed2014;370:2071-82.Nintedanib（尼達尼布）治療IPF有效NEngl抗胃食道反流治療IPF抗胃食道反流治療IPFN-乙酰半胱氨酸（NAC）對IPF的作用

Demedtsetal.NEJM2005N-乙酰半胱氨酸（NAC）對IPF的作用DemedtseNEnglJMed2014;370:2093-101NAC治療IPF；對肺功能無影響NEnglJMed2014;370:2093-101其它治療肺移植氧療與對癥治療康復中醫(yī)中藥其它治療肺移植TherapyforacuteexacerbationofIPFSteroidtherapymethylpredisone0.5-1.0givgttx3d，followedby1-2mg/dCyclosporinAIgAntimicrobialtherapyOxygentherapySupporativetherapyTherapyforacuteexacerbationNatureHistoryofIPFNatureHistoryofIPFIPF小結IPF是一種預后較差的疾病，病程各異疾病惡化表現(xiàn)為呼吸道癥狀加重、肺功能下降、肺CT進行性肺纖維化、急性呼吸衰竭甚至死亡臨床表現(xiàn)為呼吸困難和肺部Velcro啰音高分辨率肺CT（HRCT）是診斷重要手段目前內科治療有效的藥物為：吡非尼酮pirfenidone，尼達尼布NintedanibIPF小結IPF是一種預后較差的疾病，病程各異疾病惡化表現(xiàn)為SuggestingreadingGINAREPORT,GLOBALSTRATEGYFORASTHMAMANAGEMENTANDPREVENTION./AnOfficialATS/ERS/JRS/ALATClinicalPracticeDuideline:TreatmentofIdiopathicPulmonaryFibrosis.AmJRespirCritCareMedVol192(2).ppe3-e19,2015AnOfficialAmericanThoracicSociety/EuropeanRespiratorySocietyStatement:UpdateoftheInternationalMultidisciplinaryClassificationoftheIdiopathicInterstitialPneumonias.AmJRespirCritCareMedVol188,Iss.6,pp733–748,Sep15,2013SuggestingreadingGINAREPORT,

THANKYOUTHANKYOUBronchialasthma

支氣管哮喘BronchialasthmaThosetoberememberedThosetoberememberedMorbidityofasthmaMorbidityofasthmaMortalityofasthma在中國有1500萬，全球3億哮喘患者，每100，000位哮喘患者中有36.7位哮喘患者會因哮喘死亡。Mortalityofasthma在中國有1500萬，全DefinitionAsthmaisaheterogeneous

disease,characterizedbychronicairwayinflammation.Itisdefinedbyhistoryofrespiratorysymptomssuchaswheeze,shortnessofbreath,chesttightnessandcoughthatvaryintimeandintensity,togetherwithvariableexpiratoryflowlimitation.DefinitionAsthmaisaheteroge哮喘的定義Allergicinflammation氣道慢性變態(tài)反應性炎癥Inflammatorycells由多種炎癥細胞：如嗜酸粒細胞、肥大細胞、T淋巴細胞、嗜中性粒細胞、Structuralcells氣道結構細胞：平滑肌、氣道上皮細胞等）細胞組分(cellularelements)參與的氣道慢性炎癥性疾病慢性炎癥導致氣道高反應性（airwayhypersensitivity）引起反復發(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，多數患者可自行緩解或經治療緩解

哮喘的定義Allergicinflammation氣道慢性PathogenesisGeneticfactorsAirwayimmunity-inflammationAllergicresponse-earlyasthmaticandlateasthmaticresponseairwayhyperresponsivenessairwayremodellingNeurologicaccomodationPathogenesisGeneticfactorsPathogenesisofasthma環(huán)境因素遺傳易感個體炎癥細胞、細胞因子及炎癥介質相互作用氣道神經調節(jié)失衡及氣道平滑肌結構功能異常氣道炎癥氣道高反應性癥狀性哮喘環(huán)境激發(fā)因子氣道高反應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：活動后吸入冷空氣后夜間感冒后突然發(fā)作性體征呼氣時產生哮鳴音規(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特殊類型哮喘Exercise-inducedasthmaAspirinasthma：asthma,nasalpolypandaspirinintoleranceAsthma-COPDoverlapsyndrome特殊類型哮喘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

內科學英文課件：Bronchialasthma內科學英文課件：BronchialasthmaLabTesingLungfunctiontest

Bronchodilationtest:airwayreversibilitypostFEV112%,?FEV1>200ml

Bronchialprovocationtest:bronchialhyperreactivitypost-FEV120%

PEPvariationSpecificallergentestFractionalExhalednitricoxide(FeNO)LabTesingLungfunctiontestLabtestingBloodeosinophilsChestX-rayBloodgasanalysisMild：PaCO正常或降低，PaO2正常，pH正?；蚝粑詨A重度Severe：PaCO正?；蚪档停琍aO2正常，pH偏酸，呼吸性或代謝性酸中毒LabtestingBloodeosinophils1.反復發(fā)作性喘息、呼吸困難、胸悶或咳嗽，多與接觸變應原、冷空氣、物理化學剌激、病毒感染、運動等有關

2.可聞哮鳴音

3.上述癥狀可經治療或自行緩解

4.癥狀不典型者(如無明顯喘息和體征)至少應有下列三項中的一項陽性∶（1）支氣管激發(fā)試驗或運動試驗陽性；（2）支氣管舒張試驗陽性；（3）呼氣流量峰值（PEF）日內變異率或晝夜波動率≥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.反復發(fā)作性喘息、呼吸困難、胸悶或咳嗽，多與接觸變應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)作時病情嚴重程度的分級

臨床特點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

輔助呼吸肌活動及三凹征

哮鳴音

脈率

奇脈

使用β2激動劑后PEF預計值PaO2（吸空氣）

PaCO2

SaO2（吸空氣）

PH步行、上樓時

可平臥

連續(xù)成句

可有焦慮尚安靜

常無

輕度增加

常無

散在，呼吸末期

<100次／min

無，<10mmHg

>80％

正常

<45mmHg

>95％

稍事活動

喜坐位

單詞

時有焦慮或煩燥

有

增加

可有

響亮、彌漫

100～120次可有

60％～80％

≥60mmHg

≤45mmHg

91％一95％

休息時

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

嗜睡或意識模糊

胸腹矛盾運動

減弱、乃到無

脈率變慢不規(guī)則

無，提示呼吸肌疲勞

降低

表3哮喘急性發(fā)作時病情嚴重程度的分級臨床特點輕度中度重度AsthmainspecialpopulationExercise-inducedasthmaDrug-inducedasthmaObesityasthmaOccupationalasthma>>>>>>>>>>>>>>>>>>>>AsthmainspecialpopulationExDifferentialdiagnosisLeftheartfailureCOPDUpperairwayobstructionAllergicbronchopulmonaryaspergillosisDifferentialdiagnosisLeftheaComplicationsPneumothoraxPneumomediastinumLungatelectasisBronchiectasisCOPDCorPulmonaleInterstitiallungdiseaseComplicationsPneumothoraxPharmacotherapyofasthmaRelieverInhaledshort-acting2agonistOralshort-acting2agonistAnticholinergicsMethylxanthinesSystemicsteroidControllerInhaledsteroidInhaledlong-acting2agonistOrallong-acting2agonistLeukotrienemodifierMethylxanthinesCromonesSystemicsteroidIgEAbImmunotherapyPharmacotherapyofasthmaRelieClassificationof?2agonists作用時間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

1．達到并維持哮喘控制

2．保持正?；顒?；包括運動能力

3．維持肺功能維持在接近正常水平

4．預防哮喘急性加重5．避免哮喘藥物的不良反應

6．預防哮喘死亡，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內科學英文課件：BronchialasthmaDiskhalerDiskhalerNebulizerNebulizerStep1: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-達到哮喘控制為基礎哮喘的治療-達到哮喘控制為基礎LevelsofasthmacontrolDaytimesymptoms>2/wkNightawakingRelieverneeded>2/wkActivitylimitationNone1~2ofthose3~4ofthoseWellcontrolledPartlycontrolledUncontrolledGINAupdated2014Symptomspast4wksLevelofcontrolLevelsofasthmacontrolDaytim哮喘治療與評價多數哮喘從2級開始，癥狀頻繁者3級2-4wk復診，后1-3月復診哮喘評價：良好控制/部分控制/未控制達到控制后維持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%哮喘治療與評價多數哮喘從2級開始，癥狀頻繁者3級哮喘升級治療持續(xù)升級（2-3月）：初始治療反應差短期升級（1-2周）：病毒感染、季節(jié)過敏原日間調整：含福莫特羅制劑，維持、緩解方案哮喘升級治療持續(xù)升級（2-3月）：初始治療反應差哮喘長期治療方案哮喘教育環(huán)境控制按需使用速效2-激動劑按需使用速效2-激動劑可選擇的控制藥物選用一種選用一種在第三級基礎上，選用一種或多種在第四級基礎上，加用一種低劑量ICS低劑量ICS加長效2-激動劑中/高劑量ICS加長效2-激動劑口服糖皮質激素(最小劑量)白三烯調節(jié)劑/茶堿中/高劑量ICS白三烯調節(jié)劑抗IgE治療低劑量ICS加白三烯調節(jié)劑緩釋茶堿低劑量ICS加緩釋茶堿第一級第二級第三級第四級第五級升級降級5.FromtheGlobalStrategyforAsthmaManagementandPrevention,GlobalInitiativeforAsthma(GINA)2011.Availablefrom:/.哮喘長期治療方案哮喘教育環(huán)境控制按需使用速效2-激動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別考慮升級治療，已達到控制升級治療直至達到控制按急性加重治療治療措施治療級別降級升級第1級第2級第3級第4級第5級降級升級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彌漫性間質性肺病INTERSTITIALLUNGDISEASE肺間質肺間質包含肺泡上皮與肺血管內皮的空間，包含多種細胞如成纖維細胞、成纖維細胞母細胞、巨噬細胞，還有基質包括膠原、彈力蛋白和糖蛋白).肺間質肺間質包含肺泡上皮與肺血管內皮的空間，包含多種細胞如成Interstitium肺間質的概念Interstitium肺間質的概念

肺臟基本功能單位與肺間質肺臟基本功能單位與肺間質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性肺病的概念間質性肺?。↖nterstitialLungDisease，ILD）為累及肺泡壁以及肺泡周圍組織和其周圍結構的非感染、非腫瘤性一組疾病。

ILD可累及細支氣管與肺實質，又被稱為彌漫性實質性肺病(DiffuseParenchymalLungDisease，DPLD)間質性肺病的概念間質性肺?。↖nterstitialLun間質性肺病的共性肺容積縮小肺彌散功能減低呼吸困難，特別運動后運動后或疾病低氧血癥好影像學彌漫性改變病理學表現(xiàn)為肺實質/間質不同程度纖維化/炎癥間質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締組織疾病相關性ILD特發(fā)性間質性肺炎（IIP）肉芽腫所致DPLD結節(jié)病過敏性肺炎肉芽腫并血管炎少見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胞增生癥特發(fā)性含鐵血黃素沉著癥慢性嗜酸細胞性肺炎彌漫性肺間質疾?。―PLD）間質性肺病的分類已知原因的DPLD職業(yè)性肺病癥狀與體征

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