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Sarcoidosisisamultisystemdisorderthatischaracterizedbynoncaseousepithelioidcellgranulomas,whichmayaffectalmostanyorgan.結(jié)節(jié)病是一種以非干酪樣壞死的上皮細(xì)胞肉芽腫為特點的多系統(tǒng)疾病,幾乎可以累及所有器官。Sarcoidosismaybeasymptomaticorchronic.Itcommonlyimprovesorclearsupspontaneously(自愈或好轉(zhuǎn)).Morethan2/3ofpeoplewithlungsarcoidosishavenosymptomsafter9years.About50%haverelapses.About10%developseriousdisability.
Sarcoidosisofthelungisprimarilyan
interstitiallungdiseaseinwhichtheinflammatoryprocessinvolvesthealveoli,smallbronchi,andsmallbloodvessels.TypicalandAtypicalFeaturesofPulmonarySarcoidosisatHigh-ResolutionCT
Typicalfeatures1Lymphadenopathy:hilar,mediastinal(rightparatracheal),bilateral,symmetric,andwelldefined
2Nodules:micronodules(2–4mmindiameter;welldefined,bilateral);macronodules(≥5mmindiameter,coalescing)
3Lymphangiticspread:peribronchovascular,subpleural,interlobularseptal
4Fibroticchanges:reticularopacities,architecturaldistortion,tractionbronchiectasis,bronchiolectasis,volumelossBilateralperihilaropacities
5Predominantupper-andmiddle-zonelocationsofparenchymalabnormalities結(jié)節(jié)病原本是肺間質(zhì)性病變,累及肺泡,支氣管和小血管淋巴結(jié)增大,兩側(cè)對稱,境界清楚大小結(jié)節(jié)淋巴管播散,支氣管血管鞘,胸膜下,小葉間隔纖維化改變,網(wǎng)狀陰影,肺結(jié)構(gòu)扭曲,牽拉性支擴(kuò),肺容積縮小,兩肺門旁致密影中上肺為主典型特征
Atypicalfeatures1Lymphadenopathy:unilateral,isolated,anteriorandposteriormediastinal
2Airspaceconsolidation:masslikeopacities,conglomeratemasses,solitarypulmonarynodules,confluentalveolaropacities(alveolarsarcoidpattern)
3Ground-glassopacities
4Linearopacities:interlobularseptalthickening,intralobularlinearopacities
5Fibrocysticchanges:cysts,bullae,blebs,emphysema,honeycomb-likeopacitieswithupper-andmiddle-zonepredominance
單側(cè)孤立前后縱隔淋巴結(jié)增大肺泡實變:腫塊,實質(zhì)性結(jié)節(jié),實變?nèi)诤厦Aш幱熬€狀陰影小葉間隔增厚小葉年線狀陰影不典型表現(xiàn)囊性纖維化改變:囊,大泡,小泡,肺氣腫,蜂窩樣改變6Miliaryopacities
粟粒樣改變
7Airwayinvolvement:mosaicattenuationpattern,tracheobronchialabnormalities,atelectasis
氣道累及:馬賽克改變,氣管支氣管異常,肺不張8Pleuraldisease:effusion,chylothorax,hemothorax,pneumothorax,pleuralthickening,calcificationPleuralplaquelikeopacities
9Mycetoma,aspergilloma
霉菌球Duringthepastdecade,advanceshavebeenmadeinthestudyofsarcoidosis
TheMycobacteriumtuberculosiscatalase-peroxidase(mKatG)protein,apotentialantigen,hasbeenidentified,結(jié)核分枝桿菌過氧化氫酶過氧化物酶(mkatg)蛋白,作為一個潛在的抗原已被確定。PETscanninghasprovenvaluableinlocatingoccultsitesfordiagnosticbiopsy.PET掃描已被證明在定位活檢診斷隱匿性病灶的價值
Endobronchialultrasound-guidedtransbronchialneedleaspiration(EBUS-TBNA)ofmediastinallymphnodeshasfacilitateddiagnosis,ofteneliminatingtheneedformore-invasiveprocedures,suchasmediastinoscopy.支氣管內(nèi)超聲引導(dǎo)下經(jīng)支氣管針吸活檢(EBUS-TBNA)的縱隔淋巴結(jié)有利于診斷,往往避免更具侵襲性的操作,如縱隔鏡。Geneexpressionanalyseshavemoreclearlydefinedcytokinedysregulationinsarcoidosis
Currently,noreliableprognosticbiomarkershavebeenidentified.目前,還沒有可靠的預(yù)后標(biāo)志物已被確定。Thetumornecrosisfactor(TNF)inhibitors,arelativelynewclassofsteroid-sparingagents,havebeenusedinpatientswithrefractorydisease.Itisunclearwhethermedicationsusedtotreatpulmonaryarterialhypertensionaresafeandeffectiveforthetreatmentofsarcoidosis-associatedpulmonaryhypertension.腫瘤壞死因子(TNF)抑制劑,一個相對較新的類固醇保代理類藥品,已被用于治療難治性疾病。目前還不清楚是否用于治療肺動脈高壓的藥物治療結(jié)節(jié)病相關(guān)性肺動脈高壓是安全和有效的。PathologicCorrelation相關(guān)病理
Granulomasinthelungparenchymahaveacharacteristicdistributioninrelationtolymphaticsintheperibronchovascularinterstitialspace,subpleuralinterstitialspace,and,toalesserextent,theinterlobularsepta(ie,alymphangiticdistribution)
肺實質(zhì)肉芽腫分布與支氣管血管鞘,胸膜下結(jié)締組織,小葉間隔中淋巴管相關(guān).
ThickenedbronchovascularbundlesandsmallperivascularnodulesseenatCTcorrespondedtogranulomaswithintheconnectivetissuesheathsurroundingpulmonaryairwaysandvessels.PleuralorsubpleuralnoduleswerecorrelatedwithgranulomasadjacenttothevisceralpleuraCT上支氣管血管鞘增厚和小結(jié)節(jié)是與包繞氣道血管結(jié)締組織鞘中肉芽腫,胸膜和胸膜下結(jié)節(jié)與臟層胸膜旁肉芽腫相關(guān)。
Ground-glassopacitiesrepresentedanaccumulationofmanygranulomatouslesions,withorwithoutfibrosis,inthealveolarseptaandaroundthesmallvessels.Noalveolitiswasseen
肺泡間隔小血管周圍大量肉芽腫是毛玻璃陰影主要原因,可伴纖維化,但沒有肺泡炎。
Largeparenchymalnodules(>1cmindiameter)representedcoalescentgranulomas
大結(jié)節(jié)是肉芽腫病變的融合
AirbronchiologramswithinregionsofdenseconsolidationonCTimagescorrespondedtobronchiolardilatationwithsurroundingfibrosis
支氣管充氣癥是纖維化旁的支擴(kuò)honeycomb-likepatternofmicroscopiccystsseenatpathologicanalysis.
蜂窩樣改變在顯微鏡下就是很多的小囊pulmonarysarcoidosisshowsthetypicalperilymphaticdistributionofmicronodules(arrow).
(外周淋巴分布的微結(jié)節(jié))
Photomicrographofalungbiopsyspecimendemonstratesnumerousepithelioidgranulomas(arrow)surroundingthebronchialwallsandimmediatelybeneaththenormalbronchialepithelium(arrowheads).CTscanshowsmultiplemicronoduleswithaperibronchovasculardistributioninbothlungs,predominantlyintheupperandmiddlelobes.Oneclusterofnodulesintheperipheryoftheleftupperlobe(arrow)hascoalescedtoformaconglomeratelesion(macronodule).
Coronalreformattedimagefromhigh-resolutionCTclearlyshowsupper-lobepredominanceofthemicronodules.
Low-magnificationphotomicrographslicefromthelowerpartoftherightupperlobeshowsmultipleconfluentgranulomasinfiltratingtheperibronchovascular(arrows)andsubpleural(arrowheads)interstitium.CTscanshowsmediastinallymphnodeenlargementandareticularpatternproducedbynodularityandthickeningofinterlobularsepta,pleuralsurfaces,andfissures,CT掃描顯示縱隔淋巴結(jié)腫大和形成網(wǎng)狀圖案的小葉間隔增厚,及胸膜表面,及產(chǎn)生的裂縫。
(b)Photomicrographofaspecimenfromfine-needleaspirationbiopsyofanenlargedrightparatracheallymphnodeshowsagroupofhistiocytesagainstalymphocyticbackground,acytologicstructurecharacteristicofsarcoidgranuloma.(c)Photomicrographofalungbiopsyspecimenfromanotherpatientshowsprogressivethickeningoftheinterlobularseptum(*)becauseoftheaccumulationofnumeroussarcoidgranulomas(arrowheads),anappearancethatcorrelateswellwiththeCTfeaturesseenina.從擴(kuò)大的右氣管旁淋巴結(jié)細(xì)針穿刺活檢標(biāo)本顯微鏡下顯示一組以淋巴細(xì)胞的背景的組織細(xì)胞,具有結(jié)節(jié)病肉芽腫細(xì)胞的結(jié)構(gòu)特征。(C)從另一個病人的肺活檢標(biāo)本的顯微照片顯示小葉間隔增厚(*),大量積累的肉芽腫結(jié)節(jié)(箭頭)。expiratoryCTscanobtainedinapatientwithpulmonarysarcoidosisshowsamosaicpatternconsistingofmultipleareasoflowattenuation(arrows)interspersedwithlargerareasofnormallungparenchyma.Thisappearanceisproducedbyairtrapping.Photomicrographsofatransbronchiallungbiopsyspecimenshowaccumulationsofsarcoidgranulomas(*)inthemucosalandsubmucosallayersofbronchiolarepithelium(arrowsinb).呼氣相CT掃描在肺結(jié)節(jié)病患者顯示馬賽克征,由低密度的多個區(qū)域(箭頭)穿插著大面積的正常肺實質(zhì)。這種現(xiàn)象是由空氣滯留產(chǎn)生。一經(jīng)支氣管鏡肺活檢標(biāo)本顯示的積累結(jié)節(jié)病肉芽腫的顯微照片(×)在粘膜和粘膜下的細(xì)支氣管上皮層(箭頭B)。5Fibrocysticchanges:cysts,bullae,blebs,emphysema,honeycomb-likeopacitieswithupper-andmiddle-zonepredominance這種現(xiàn)象是由空氣滯留產(chǎn)生。Low-magnificationphotomicrograph肺實質(zhì)肉芽腫分布與支氣管血管鞘,胸膜下結(jié)締組織,小葉間隔中淋巴管相關(guān)Avideo-assistedthoracoscopicsurgicalbiopsywasperformed.PathologicCorrelation相關(guān)病理(b)Low-powerphotomicrograph(originalmagnification,×10;H-Estain)obtainedathistopathologicanalysisshowsasubpleuralnodulethatisdarkerincolorbecauseofanthracosis(塵肺).5Predominantupper-andmiddle-zonelocationsofparenchymalabnormalitiesAlveolarsarcoid多房型肉瘤patternofairspaceconsolidationinpulmonarysarcoidosis.Typicalfeatures呼氣相CT掃描在肺結(jié)節(jié)病患者顯示馬賽克征,由低密度的多個區(qū)域(箭頭)穿插著大面積的正常肺實質(zhì)。Mildbilateralhilarlymphadenopathyandmarkedsub-carinallymphadenopathyalsoareseen.一經(jīng)支氣管鏡肺活檢標(biāo)本顯示的積累結(jié)節(jié)病肉芽腫的顯微照片(×)在粘膜和粘膜下的細(xì)支氣管上皮層(箭頭B)。Photomicrographsofatransbronchiallungbiopsyspecimenshowaccumulationsofsarcoidgranulomas(*)inthemucosalandsubmucosallayersofbronchiolarepithelium(arrowsinb).A,High-resolutionCTscanoflungsrevealsill-definedsmallnodularopacitiesinperibronchovascularandissuraldistribution.Characteristicfeaturesofchronicdiseasearedepicted,includingtractionbronchiectasis牽拉性支氣管擴(kuò)張,severearchitecturaldistortion,volumeloss,andinterlobularseptalthickening.CT掃描顯示彌漫性磨玻璃影,由多個融合的結(jié)節(jié)產(chǎn)生,伴支氣管擴(kuò)張(11b)Magnifiedaxialhigh-resolutionCTscanoftherightlungclearlydepictsseparatenodulesinasubpleural(blackarrow)andfissural(whitearrow)distributionandalongthebronchovascularbundles(arrowheads)放大的軸位高分辨率CT掃描清楚顯示右肺胸膜下區(qū)分胸膜下的結(jié)節(jié)(黑色箭頭)和沿支氣管血管束分布的“裂”(白色箭頭).蜂窩樣改變在顯微鏡下就是很多的小囊纖維化改變,網(wǎng)狀陰影,肺結(jié)構(gòu)扭曲,牽拉性支擴(kuò),肺容積縮小,兩肺門旁致密影纖維化改變,網(wǎng)狀陰影,肺結(jié)構(gòu)扭曲,牽拉性支擴(kuò),肺容積縮小,兩肺門旁致密影
.(11a)CTscanshowsadiffuseground-glasspatternproducedbymultipleconfluentmicronodules,withassociatedbronchiectasis.CT掃描顯示彌漫性磨玻璃影,由多個融合的結(jié)節(jié)產(chǎn)生,伴支氣管擴(kuò)張(11b)Magnifiedaxialhigh-resolutionCTscanoftherightlungclearlydepictsseparatenodulesinasubpleural(blackarrow)andfissural(whitearrow)distributionandalongthebronchovascularbundles(arrowheads)放大的軸位高分辨率CT掃描清楚顯示右肺胸膜下區(qū)分胸膜下的結(jié)節(jié)(黑色箭頭)和沿支氣管血管束分布的“裂”(白色箭頭).(11c)High-powerphotomicrographshowsanaccumulationofinterstitialgranulomas(white*),whichcausesathickenedappearanceoftheinteralveolarsepta,andacinargranulomas(black*),whichformintheinterstitiumofthealveolarwallandprotrudeintothealveoli(arrowheads).高倍鏡下涂片顯示間質(zhì)肉芽腫集聚(白*),使肺小泡壁的增厚,和腺泡肉芽腫(黑色),形成在肺泡壁的間質(zhì)和伸入肺泡(箭頭)。SarcoidosisinapatientwithahistoryofstageIIIprimarycutaneousmalignantmelanoma.結(jié)節(jié)病III期原發(fā)性皮膚惡性黑色素瘤病史。(a)Contrast-enhancedCTscansshowpulmonarynodules(arrow)insubpleural(right)andfissural(left)regions.Avideo-assistedthoracoscopicsurgicalbiopsywasperformed.(b)Low-powerphotomicrograph(originalmagnification,×10;H-Estain)obtainedathistopathologicanalysisshowsasubpleuralnodulethatisdarkerincolorbecauseofanthracosis(塵肺).(c)Photo-micrographobtainedathigherpower(originalmagnification,×100;H-Estain)showsmultiplenonnecroticgranulomas(壞死性肉芽腫)(arrows)expandingtheinterstitiumthatsurroundsthesubpleuralnoduleinb.Typical(a,b)andatypical(c,d)radiologicfindingsoflymphadenopathyinfourpatientswithsarcoidosis四例結(jié)節(jié)病淋巴結(jié)病變的影像學(xué)表現(xiàn).(a)Axialcontrastmaterial–enhancedCTscan(mediastinalwindow)showstypicalbilateralandsymmetrichilar(ar-rows)andsubcarinal(*)lymphadenopathy.CT增強(qiáng)掃描(縱隔窗)顯示了典型的雙側(cè)對稱性肺門(箭頭)及隆突下淋巴結(jié)腫大(*)(b)AxialunenhancedCTscan(mediastinalwindow)obtainedattheleveloftheleftpulmonaryarteryshowsenlargementofrightparatrachealandlefthilarlymphnodes(arrows)右氣管旁和左肺門淋巴結(jié)腫大.Althoughtherighthilumisnotshown,ittoowasaffected.(c)AxialunenhancedCTscan(mediastinalwindow)showspunctatecal-cificationsofhilarlymphnodes(arrows),apatternthatalsooccursinotherchronicgranulomatousdiseases.(d)Axialcontrast-enhancedCTscanshowsbilateraleggshell-likecalcificationsofhilarandmediastinallymphnodes雙側(cè)縱隔、肺門淋巴結(jié)蛋殼樣鈣化(arrows),findingsthatwarranttheinclusionofsilicosis矽肺inthedifferentialdiagnosisinthiscase.Axialhigh-resolutionCTscansobtainedattheleveloftheupperlobes(a)andcarina(b)inapatientwithpulmonarysarcoidosisshowafibrotic-cicatricialpatternofdisease,withmultiplelesionsinaperibronchovasculardistribution.Characteristicfeaturesofchronicdiseasearedepicted,includingtractionbronchiectasis牽拉性支氣管擴(kuò)張,severearchitecturaldistortion,volumeloss,andinterlobularseptalthickening.Coales-centirregularmasslikeopacities(whitearrows)andacalcifiedrightlowerparatra-chealnode(blackarrowinb)alsoareseen.Mosaicattenuation,whichismostvisibleina,presumablyresultsfromairwaydistor-tionduetofibrosis.Axialhigh-resolutionCTscanshowsseverallarge,ill-definednodulesandareasofcon-solidationresultingfromtheconfluenceofmultipleparenchymalmicronodulescomposedofnumeroustinygranulomasinbothlungs.Finenodularopacitiesareseenaroundthelargenodules(whitearrows),andsmalllow-attenuationspotsthatcorrespondtothespacesbetweenpartiallycoalescentsmallnod-ulesarevisibleperipherally.Thisappearancehasbeentermedthesarcoid“galaxysign”小結(jié)節(jié)環(huán)繞大結(jié)節(jié)——銀河征.Distortionoftherightmajorfissureisalsoseen扭曲的葉間胸膜(blackarrow).Alveolarsarcoid多房型肉瘤patternofairspaceconsolidationinpulmonarysarcoidosis.Axialhigh-resolutionCTscanshowsalveolarconsolidationintheleftupperlobeandpatchysubpleuralalveolaropacitiesintherightupperlobe.Architecturaldistortionandtractionbronchiectasis,signsoffibrosis,alsoarevisible,mainlyintherightupperlobe.Axialunenhancedhigh-resolutionCTscanshowsasymmetricsubpleuralhoneycomb-likecysts不對稱胸膜下蜂窩樣囊腫(ar-rowheads)andarchitecturaldistortionassociatedwithleftfissurenodularity(arrow).Althoughthesefeaturesalsoarecharacteristicofidiopathicpulmonaryfibrosis特發(fā)性肺纖維化,theupper-lobepredominanceofhoneycomb-likecystsandtheperibronchovascularandfissuraldistributionofmicronodulesinthiscaseweremoresuggestiveofsarcoidosis.以上葉為主的蜂窩狀囊,沿支氣管血管束周圍分布并且更提示結(jié)節(jié)病。Miliaryopacitiesinsarcoidosis.粟粒樣結(jié)節(jié)病Axialun-enhancedhigh-resolutionCTscanshowscountlesstinymicronodulesrepresentingmultipleanddiffusegranulomasinarandomdistribution,withbronchialwallthickening.Whenthispatternisseen,thedifferentialdiagnosisshouldincludemiliarytuberculosis,pneumo-coniosis,andmetastaticlesions.無數(shù)的小結(jié)節(jié)表現(xiàn)為隨機(jī)分布的多發(fā)彌漫性肉芽腫,支氣管壁增厚。鑒別診斷應(yīng)包括粟粒性肺結(jié)核,塵肺,和轉(zhuǎn)移性病變。A,High-resolutionCTscanoflungsrevealsill-definedsmallnodularopacitiesinperibronchovascularandissuraldistribution.Smallamountofinterlobularseptalthickening少量的小葉間隔增厚(arrow)isseen.
B,Photomicrographofhistopathologicspecimenshowsnoncaseating?tightlyformedgranulomasandepithelioidhistiocytesandlymphocytes.Perilymphaticdistributionofgranulomasisevident.Normallungparenchymaandsmallregionoffibroticinterlobularseptalthickening(arrow)areseen.組織病理標(biāo)本的顯微照片顯示緊密的肉芽腫和上皮樣細(xì)胞和淋巴細(xì)胞。肉芽腫淋巴分布是顯而易見的。正常肺組織纖維化和小葉間隔增厚(箭頭)。Tracheobronchialandpulmonaryinvolvementofsarcoidosis主支氣管及肺實質(zhì)受侵
(a)Axialcontrast-enhancedCTscanobtainedatthesubcarinallevelshowsasoft-tissuemasswithpunctatecalcificationsthatfillsthelumenoftherightintermediatebronchus(arrow),causingpartialatelectasisoftherightmiddleandrightlowerlobes.Mildbilateralhilarlymphadenopathyandmarkedsub-carinallymphadenopathyalsoareseen.造成右中、右下葉肺不張的部分。雙側(cè)肺門淋巴結(jié)腫大和標(biāo)記隆突淋巴結(jié)腫大也看到。(b)Axialcontrast-enhancedCTscanshowsthatpartofthemassencasesandoccupiestherightlowerlobebronchi,causingpartialatelectasis.Atdiagnosticthoracotomy,sarcoidosiswithtracheobronchialandpulmonaryinvolvementwasfound.開胸活檢示,支氣管及肺實質(zhì)受累Sarcoidosisisamultisystemdisorderthatischaracterizedbynoncaseousepithelioidcellgranulomas,whichmayaffectalmostanyorgan.結(jié)節(jié)病是一種以非干酪樣壞死的上皮細(xì)胞肉芽腫為特點的多系統(tǒng)疾病,幾乎可以累及所有器官。Sarcoidosismaybeasymptomaticorchronic.Itcommonlyimprovesorclearsupspontaneously(自愈或好轉(zhuǎn)).Morethan2/3ofpeoplewithlungsarcoidosishavenosymptomsafter9years.About50%haverelapses.About10%developseriousdisability.支氣管內(nèi)超聲引導(dǎo)下經(jīng)支氣管針吸活檢(EBUS-TBNA)的腫瘤壞死因子(TNF)抑制劑,一個相對較新的類固醇保代理類藥品,已被用于治療難治性疾病。纖維化改變,網(wǎng)狀陰影,肺結(jié)構(gòu)扭曲,牽拉性支擴(kuò),肺容積縮小,兩肺門旁致密影粟粒樣結(jié)節(jié)病Axialun-enhancedhigh-resolutionCTscanshowscountlesstinymicronodulesrepresentingmultipleanddiffusegranulomasinarandomdistribution,withbronchialwallthickening.6MiliaryopacitiesLow-magnificationphotomicrograph2Nodules:micronodules(2–4mmindiameter;welldefined,bilateral);macronodules(≥5mmindiameter,coalescing)無數(shù)的小結(jié)節(jié)表現(xiàn)為隨機(jī)分布的多發(fā)彌漫性肉芽腫,支氣管壁增厚。5Predominantupper-andmiddle-zonelocationsofparenchymalabnormalitiesPhotomicrographsofatransbronchiallungbiopsyspecimenshowaccumulationsofsarcoidgranulomas(*)inthemucosalandsubmucosallayersofbronchiolarepithelium(arrowsinb).8Pleuraldisease:effusion,chylothorax,hemothorax,pneumothorax,pleuralthickening,calcificationPleuralplaquelikeopacities(外周淋巴分布的微結(jié)節(jié))Architecturaldistortionandtractionbronchiectasis,signsoffibrosis,alsoarevisible,mainlyintherightupperlobe.從擴(kuò)大的右氣管旁淋巴結(jié)細(xì)針穿刺活檢標(biāo)本顯微鏡下顯示一組以淋巴細(xì)胞的背景的組織細(xì)胞,具有結(jié)節(jié)病肉芽腫細(xì)胞的結(jié)構(gòu)特征。PathologicCorrelation相關(guān)病理Mildbilateralhilarlymphadenopathyandmarkedsub-carinallymphadenopathyalsoareseen.2Nodules:micronodules(2–4mmindiameter;welldefined,bilateral);macronodules(≥5mmindiameter,coalescing)Typicalfeatures(c)AxialunenhancedCTscan(mediastinalwindow)showspunctatecal-cificationsofhilarlymphnodes(arrows),apatternthatalsooccursinotherchronicgranulomatousdiseases.目前還不清楚是否用于治療肺動脈高壓的藥物治療結(jié)節(jié)病相關(guān)性肺動脈高壓是安全和有效的。
Sarcoidosisofthelungisprimarilyan
interstitiallungdiseaseinwhichtheinflammatoryprocessinvolvesthealveoli,smallbronchi,andsmallbloodvessels.TypicalandAtypicalFeaturesofPulmonarySarcoidosisatHigh-ResolutionCT
Typicalfeatures1Lymphadenopathy:hilar,mediastinal(rightparatracheal),bilateral,symmetric,andwelldefined
2Nodules:micronodules(2–4mmindiameter;welldefined,bilateral);macronodules(≥5mmindiameter,coalescing)
3Lymphangiticspread:peribronchovascular,subpleural,interlobularseptal
4Fibroticchanges:reticularopacities,architecturaldistortion,tractionbronchiectasis,bronchiolectasis,volumelossBilateralperihilaropacities
5Predominantupper-andmiddle-zonelocationsofparenchymalabnormalities結(jié)節(jié)病原本是肺間質(zhì)性病變,累及肺泡,支氣管和小血管淋巴結(jié)增大,兩側(cè)對稱,境界清楚大小結(jié)節(jié)淋巴管播散,支氣管血管鞘,胸膜下,小葉間隔纖維化改變,網(wǎng)狀陰影,肺結(jié)構(gòu)扭曲,牽拉性支擴(kuò),肺容積縮小,兩肺門旁致密影中上肺為主典型特征CTscanshowsmediastinallymphnodeenlargementandareticularpatternproducedbynodularityandthickeningofinterlobularsepta,pleuralsurfaces,andfissures,CT掃描顯示縱隔淋巴結(jié)腫大和形成網(wǎng)狀圖案的小葉間隔增厚,及胸膜表面,及產(chǎn)生的裂縫。
(b)Photomicrographofaspecimenfromfine-needleaspirationbiopsyofanenlargedrightparatracheallymphnodeshowsagroupofhistiocytesagainstalymphocyticbackground,acytologicstructurecharacteristicofsarcoidgranuloma.(c)Photomicrographofalungbiopsyspecimenfromanotherpatientshowsprogressivethickeningoftheinterlobularseptum(*)becauseoftheaccumulationofnumeroussarcoidgranulomas(arrowheads),anappearancethatcorrelateswellwiththeCTfeaturesseenina.從擴(kuò)大的右氣管旁淋巴結(jié)細(xì)針穿刺活檢標(biāo)本顯微鏡下顯示一組以淋巴細(xì)胞的背景的組織細(xì)胞,具有結(jié)節(jié)病肉芽腫細(xì)胞的結(jié)構(gòu)特征。(C)從另一個病人的肺活檢標(biāo)本的顯微照片顯示小葉間隔增厚(*),大量積累的肉芽腫結(jié)節(jié)(箭頭)。expiratoryCTscanobtainedinapatientwithpulmonarysarcoidosisshowsamosaicpatternconsistingofmultipleareasoflowattenuation(arrows)interspersedwithlargerareasofnormallungparenchyma.Thisappearanceisproducedbyairtrapping.Photomicrographsofatransbronchiallungbiopsyspecimenshowaccumulationsofsarcoidgranulomas(*)inthemucosalandsubmucosallayersofbronchiolarepithelium(arrowsinb).呼氣相CT掃描在肺結(jié)節(jié)病患者顯示馬賽克征,由低密度的多個區(qū)域(箭頭)穿插著大面積的正常肺實質(zhì)。這種現(xiàn)象是由空氣滯留產(chǎn)生。一經(jīng)支氣管鏡肺活檢標(biāo)本顯示的積累結(jié)節(jié)病肉芽腫的顯微照片(×)在粘膜和粘膜下的細(xì)支氣管上皮層(箭頭B)。Axialhigh-resolutionCTscanshowsseverallarge,ill-definednodulesandareasofcon-solidationresultingfromtheconfluenceofmultipleparenchymalmicronodulescomposedofnumeroustinygranulomasinbothlungs.Finenodularopacitiesareseenaroundthelargenodules(whitearrows),andsmalllow-attenuationspotsthatcorrespondtothespacesbetweenpartiallycoalescentsmallnod-ulesarevisibleperipherally.Thisappearancehasbeentermedthesarcoid“galaxysign”小結(jié)節(jié)環(huán)繞大結(jié)節(jié)——銀河征.Distortionoftherightmajorfissureisalsoseen扭曲的葉間胸膜(blackarrow).5Predominantupper-andmiddle-zonelocationsofparenchymalabnormalities5Predominantupper-andmiddle-zonelocationsofparenchymalabnormalitiesCT增強(qiáng)掃描(縱隔窗)顯示了典型的雙側(cè)對稱性肺門(箭頭)及隆突下淋巴結(jié)腫大(*)(b)AxialunenhancedCTscan(mediastinalwindow)obtainedattheleveloftheleftpulmonaryarteryshowsenlargementofrightparatrachealandlefthilarlymphnodes(arrows)右氣管旁和左肺門淋巴結(jié)腫大.肺實質(zhì)肉芽腫分布與支氣管血管鞘,胸膜下結(jié)締組織,小葉間隔中淋巴管相關(guān)粟粒樣結(jié)節(jié)病Axialun-enhancedhigh-resolutionCTscanshowscountlesstinymicronodulesrepresentingmultipleanddiffusegranulomasinarandomdistribution,withbronchialwallthickening.Whenthispatternisseen,thedifferentialdiagnosisshouldincludemiliarytuberculosis,pneumo-coniosis,andmetastaticlesions.Low-magnificationphotomicrograph(b)Photomicrographofaspecimenfromfine-needleaspirationbiopsyofanenlargedrightparatracheallymphnodeshowsagroupofhistiocytesagainstalymphocyticbackground,acytologicstructurecharacteristicofsarcoidgranuloma.4Linearopacities:interlobularseptalthickening,intralobularlinearopacities(外周淋巴分布的微結(jié)節(jié))Coronalreformattedimagefromhigh-resolutionCTclearlyshowsupper-lobepredominanceofthemicronodules.Atdiagnosticthoracotomy,sarcoidosiswithtracheobronchialandpulmonaryinvolvementwasfound.Axialhigh-resolutionCTscanshowsalveolarconsolidationintheleftupperlobeandpatchysubpleuralalveolaropacitiesintherightupperlobe.Photomicrographsofatransbronchiallungbiopsyspecimenshowaccumulationsofsarcoidgranulomas(*)inthemucosalandsubmucosallayersofbronchiolarepithelium(arrowsinb).Thisappearancehasbeentermedthesarcoid“galaxysign”小結(jié)節(jié)環(huán)繞大結(jié)節(jié)——銀河征.從擴(kuò)大的右氣管旁淋巴結(jié)細(xì)針穿刺活檢標(biāo)本顯微鏡下顯示一組以淋巴細(xì)胞的背景的組織細(xì)胞,具有結(jié)節(jié)病肉芽腫細(xì)胞的結(jié)構(gòu)特征。一經(jīng)支氣管鏡肺活檢標(biāo)本顯示的積累結(jié)節(jié)病肉芽腫的顯微照片(×)在粘膜和粘膜下的細(xì)支氣管上皮層(箭頭B)。CT上支氣管血管鞘增厚和小結(jié)節(jié)是與包繞氣道血管結(jié)締組織鞘中肉芽腫,胸膜和胸膜下結(jié)節(jié)與Endobronchialultrasound-guidedtransbronchialneedleaspiration(EBUS-TBNA)of大結(jié)節(jié)是肉芽腫病變的融合Smallamountofinterlobularseptalthickening少量的小葉間隔增厚(arrow)isseen.mediastinallymphnodeshasfacilitateddiagnosis,ofteneliminatingtheneedformore-invasiveprocedures,suchasmediastinoscopy.Coronalreformattedimagefromhigh-resolutionCTclearlyshowsupper-lobepredominanceofthemicronodules.Avideo-assistedthoracoscopicsurgicalbiopsywasperformed.(b)Photomicrographofaspecimenfromfine-needleaspirationbiopsyofanenlargedrightparatracheallymphnodeshowsagroupofhistiocytesagainstalymphocyticbackground,acytologicstructurecharacteristicofsarcoidgranuloma.expiratoryCTscanobtainedinapatientwithpulmonarysarcoidosisshowsamosaicpatternconsistingofmultipleareasoflowattenuation(arrows)interspersedwithlargerareasofnormallungparenchyma.肺實質(zhì)肉芽腫分布與支氣管血管鞘,胸膜下結(jié)締組織,小葉間隔中淋巴管相關(guān)(c)AxialunenhancedCTscan(mediastinalwindow)showspunctatecal-cificationsofhilarlymphnodes(arrows),apatternthatalsooccursinotherchronicgranulomatousdiseases.支氣管內(nèi)超聲引導(dǎo)下經(jīng)支氣管針吸活檢(EBUS-TBNA)的Sarcoidosisisamultisystemdisorderthatischaracterizedbynoncaseousepithelioidcellgranulomas,whichmayaffectalmostanyorgan.Oneclusterofnodulesintheperipheryoftheleftupperlobe(arrow)hascoalescedtoformaconglomeratelesion(macronodule).(a)Axialcontrast-enhancedCTscanobtainedatthesubcarinallevelshowsasoft-tissuemasswithpunctatecalcificationsthatfillsthelumenoftherightintermediatebronchus(arrow),causingpartialatelectasisoftherightmiddleandrightlowerlobes.TypicalfeaturesAirbronchiologramswithinregionsofdenseconsolidationonCTimagescorrespondedtobronchiolardilatationwithsurroundingfibrosis支氣管充氣癥是纖維化旁的支擴(kuò)Itcommonlyimprovesorclearsupspontaneously(自愈或好轉(zhuǎn)).(b)Low-powerphotomicrograph(originalmagnification,×10;H-Estain)obtainedathistopathologicanalysisshowsasubpleuralnodulethatisdarkerincolorbecauseofanthracosis(塵肺).Mildbilateralhilarlymphadenopathyandmarkedsub-carinallymphadenopathyalsoareseen.About50%haverelapses.Smallamountofinterlobularseptalthickening少量的小葉間隔增厚(arrow)isseen.Axialunenhancedhigh-resolutionCTscanshowsasymmetricsubpleuralhoneycomb-likecysts不對稱胸膜下蜂窩樣囊腫(ar-rowheads)andarchitecturaldistortionassociatedwithleftfissurenodularity(arrow).纖維化改變,網(wǎng)狀陰影,肺結(jié)構(gòu)扭曲,牽拉性支擴(kuò),肺容積縮小,兩肺門旁致密影(c)AxialunenhancedCTscan(mediastinalwindow)showspunctatecal-cificationsofhilarlymphnodes(arrows),apatternthatalsooccursinotherchronicgranulomatousdiseases.(a)Axialcontrastmaterial–enhancedCTscan(mediastinalwindow)showstypicalbilateralandsymmetrichilar(ar-rows)andsubcarinal(*)lymphadenopathy.(外周淋巴分布的微結(jié)節(jié))這種現(xiàn)象是由空氣滯留產(chǎn)生。纖維化改變,網(wǎng)狀陰影,肺結(jié)構(gòu)扭曲,牽拉性支擴(kuò),肺容積縮小,兩肺門旁致密影Alveolarsarcoid多房型肉瘤patternofairspaceconsolidationinpulmonarysarcoidosis.5Predominantupper-andmiddle-zonelocationsofparenchymalabnormalitiesPhotomicrographsofatransbronchiallungbiopsyspecimenshowaccumulationsofsarcoidgranulomas(*)inthemucosalandsubmucosallayersofbronchiolarepithelium(arrowsinb).TypicalandAtypicalFeaturesofPulmonarySarcoidosisatHigh-ResolutionCTCharacteristicfeaturesofchronicdiseasearedepicted,includingtractionbronchiectasis牽拉性支氣管擴(kuò)張,severearchitecturaldistortion,volumeloss,andinterlobularseptalthickening.Alveolarsarcoid多房型肉瘤patternofairspaceconsolidationinpulmonarysarcoidosis.這種現(xiàn)象是由空氣滯留產(chǎn)生。雙側(cè)肺門淋巴結(jié)腫大和標(biāo)記隆突淋巴結(jié)腫大也看到。結(jié)節(jié)病III期原發(fā)性皮膚惡性黑色素瘤病史。大結(jié)節(jié)是肉芽腫病變的融合Miliaryopacitiesinsarcoidosis.粟粒樣結(jié)節(jié)病Axialun-enhancedhigh-resolutionCTscanshowscountlesstinymicronodulesrepresentingmultipleanddiffusegranulomasinarandomdistribution,withbronchialwallthickening.腫瘤壞死因子(TNF)抑制劑,一個相對較新的類固醇保代理類藥品,已被用于治療難治性疾病。纖維化改變,網(wǎng)狀陰影,肺結(jié)構(gòu)扭曲,牽拉性支擴(kuò),肺容積縮小,兩肺門旁致密影這種現(xiàn)象是由空氣滯留產(chǎn)生。Axialunenhancedhigh-resolutionCTscanshowsasymmetricsubpleuralhoneycomb-likecysts不對稱胸膜下蜂窩樣囊腫(ar-rowheads)andarchitecturaldistortionassociatedwithleftfissurenodularity(arrow).Alveolarsarcoid多房型肉瘤patternofairspaceconsolidationinpulmonarysarcoidosis.Architecturaldistortionandtractionbronchiectasis,signsoffibrosis,alsoarevisible,mainlyintherightupperlobe.5Predominantupper-andmiddle-zonelocationsofparenchymalabnormalitiesslicefromthelowerpartoftherightupperlobeshowsmultipleconfluentgranulomasinfiltratingtheperibronchovascular(arrows)andsubpleural(arrowheads)interstitium.Endobronchialultrasound-guidedtransbronchialneedleaspiration(EBUS-TBNA)of肺實質(zhì)肉芽腫分布與支氣管血管鞘,胸膜下結(jié)締組織,小葉間隔中淋巴管相關(guān)Coronal
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