急性间质性肺炎.ppt

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1、急性间质性肺炎AIP概述Rare,fulminantformoflunginjurySimilartoARDSARDS=AIPwithanetiologyDescribedbyHammanandRichEtiologyunknown流病日本1992-1993年间共报道30多例我国仅10多例M=FMostpatientsoverage40,mean50-55病生neutrophilmediatedlunginjuryviatoxicoxygenspeciesandproteasesleadingtoepithelialcellinjuryandairspa

2、ceexudation.Increasedpermeabilityofalveolarcapillaries,interstitialedemaandintraalveolarhyalinemembranes.Thenastageoforganizationensuescharacterizedbyfibroblastproliferationandconnectivetissuesynthesis病理Acuteexudativephase(uptoday6)早期表现以透明膜形成，水肿和肺泡腔内纤维化为特点Subacuteproliferativepha

3、se(days4-10)明显的泡隔增厚，胶原的沉积较轻Chronicfibroticphase(day8-)IdenticaltoalveolardamageseeninARDSAIPAIP前驱期ClinicalFeatures7-14daysFever,cough,shortnessofbreath表现罕见的暴发性肺损伤，呈急性发作（数日至数周内）常发生于原先体健者。表现为发热、咳嗽和气短。常规实验室检查无特异性。大部分中到严重程度的低氧血症，易发生ARF表现2ARF，快中老年人初咳嗽痰乏力发热（有/无）1-2周内出现氧CO2符合ARDSVELCRO音

4、持续存在，不会消失WBCＥＳＲ高ＢＡＬＦ意义不大诊断AIP的诊断需要：特发性ARDS的临床表现病理证实为机化性弥漫性肺泡损伤（DAD）BALFN（46%）E（17%）升高鉴别病因明确的DAD:sepsis,drug-induced,shock,原因不明的ＡＲＤＳ肺泡出血急性嗜酸细胞性肺炎RapidlyprogressiveIIDwithCTDBOOP治疗SupportivecareItisnotclearifcorticosteroidtherapyiseffectiveMortalityis>60%Majoritydiewithin6monthsofp

5、resentationThosewhorecoverusuallydonothaverecurrenceofdiseasebutmaydevelopchronicinterstitiallungdisease治疗现有认为AIP可归于激素敏感型IIP激素有效者大于50%预后AIP的病死率相当高（＞60%又5-88%）多数死于6个月（又1-2个月）内acuterespiratorydistresssyndromehasabetterprognosisthanpreviouslywith<50%mortalityEurRespirJ2000;15:412-18

6、影像学弥漫性、双侧肺泡腔内实变阴影。CT可发现双侧（中下肺野）对称性斑片状、小结节和磨玻璃样改变，短期内（几天）融合现象，肺实变，牵拉性支扩无肺容积缩小和蜂窝肺主要病变主要分布于胸膜下。这与急性呼吸窘迫综合征（ARDS）相似。HRCT弥散性肺实变67%GGO分散67%弥漫33%两侧对称肺底部为主HRCTGGOBilateral,patchy&diffuseW/WOairspaceconsolidationPresentinallpatientsItsextentcorrelateswiththedurationofdiseaseNotnecessaril

7、ythepredictorofreversibleortreatablediseaseSeeninallthreephasesofAIPandreflectsdifferingpathologyineachphaseExudativephase-alveolarseptaledema&hyalinemembranesalongalveolarwallsProliferativephase-intraalveolarandinterstitialorganizationFibroticphase-alveolarseptalfibrosisAirspace

8、consolidationNodularopacitiesIntralobula