結節(jié)病典型和不典型型表現(xiàn)第一頁，共二十三頁，編輯于2023年，星期一OrganLungPleuraLymphNodesSkinEyeNasalMucosa（鼻粘膜）Larynx（喉）BoneMarrowSpleenLiver%901-575-90252520515-4050-6060-90OrganKidneyCalciumMetabolismNervousSystemBoneJointsHeartEndocrineGlandsParotidGlandGastrointestinalSystem%Rare1-25525-505Rare10Rare第二頁，共二十三頁，編輯于2023年，星期一Sarcoidosisisamultisystemdisorderthatischaracterizedbynoncaseousepithelioidcellgranulomas,whichmayaffectalmostanyorgan.結節(jié)病是一種以非干酪樣壞死的上皮細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）.Morethan2/3ofpeoplewithlungsarcoidosishavenosymptomsafter9years.About50%haverelapses.About10%developseriousdisability.第三頁，共二十三頁，編輯于2023年，星期一

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結節(jié)病原本是肺間質性病變，累及肺泡，支氣管和小血管淋巴結增大，兩側對稱，境界清楚大小結節(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，肺結構扭曲，牽拉性支擴，肺容積縮小，兩肺門旁致密影中上肺為主典型特征第四頁，共二十三頁，編輯于2023年，星期一

Atypicalfeatures1Lymphadenopathy:unilateral,isolated,anteriorandposteriormediastinal

2Airspaceconsolidation:masslikeopacities,conglomeratemasses,solitarypulmonarynodules,confluentalveolaropacities(alveolarsarcoidpattern)

3Ground-glassopacities

4Linearopacities:interlobularseptalthickening,intralobularlinearopacities

5Fibrocysticchanges:cysts,bullae,blebs,emphysema,honeycomb-likeopacitieswithupper-andmiddle-zonepredominance

單側孤立前后縱隔淋巴結增大肺泡實變：腫塊，實質性結節(jié)，實變融合毛玻璃陰影線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，蜂窩樣改變第五頁，共二十三頁，編輯于2023年，星期一6Miliaryopacities

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

霉菌球第六頁，共二十三頁，編輯于2023年，星期一Duringthepastdecade,advanceshavebeenmadeinthestudyofsarcoidosis

TheMycobacteriumtuberculosiscatalase-peroxidase(mKatG)protein,apotentialantigen,hasbeenidentified,結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.支氣管內超聲引導下經(jīng)支氣管針吸活檢（EBUS-TBNA）的縱隔淋巴結有利于診斷，往往避免更具侵襲性的操作，如縱隔鏡。Geneexpressionanalyseshavemoreclearlydefinedcytokinedysregulationinsarcoidosis

Currently,noreliableprognosticbiomarkershavebeenidentified.目前，還沒有可靠的預后標志物已被確定。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節(jié)病相關性肺動脈高壓是安全和有效的。第七頁，共二十三頁，編輯于2023年，星期一PathologicCorrelation相關病理

Granulomasinthelungparenchymahaveacharacteristicdistributioninrelationtolymphaticsintheperibronchovascularinterstitialspace,subpleuralinterstitialspace,and,toalesserextent,theinterlobularsepta(ie,alymphangiticdistribution)

肺實質肉芽腫分布與支氣管血管鞘，胸膜下結締組織，小葉間隔中淋巴管相關.

ThickenedbronchovascularbundlesandsmallperivascularnodulesseenatCTcorrespondedtogranulomaswithintheconnectivetissuesheathsurroundingpulmonaryairwaysandvessels.PleuralorsubpleuralnoduleswerecorrelatedwithgranulomasadjacenttothevisceralpleuraCT上支氣管血管鞘增厚和小結節(jié)是與包繞氣道血管結締組織鞘中肉芽腫，胸膜和胸膜下結節(jié)與臟層胸膜旁肉芽腫相關。

第八頁，共二十三頁，編輯于2023年，星期一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honeycomb-likepatternofmicroscopiccystsseenatpathologicanalysis.

蜂窩樣改變在顯微鏡下就是很多的小囊第九頁，共二十三頁，編輯于2023年，星期一pulmonarysarcoidosisshowsthetypicalperilymphaticdistributionofmicronodules(arrow).

（外周淋巴分布的微結節(jié)）

Photomicrographofalungbiopsyspecimendemonstratesnumerousepithelioidgranulomas(arrow)surroundingthebronchialwallsandimmediatelybeneaththenormalbronchialepithelium(arrowheads).第十頁，共二十三頁，編輯于2023年，星期一CTscanshowsmultiplemicronoduleswithaperibronchovasculardistributioninbothlungs,predominantlyintheupperandmiddlelobes.Oneclusterofnodulesintheperipheryoftheleftupperlobe(arrow)hascoalescedtoformaconglomeratelesion(macronodule).

Coronalreformattedimagefromhigh-resolutionCTclearlyshowsupper-lobepredominanceofthemicronodules.

Low-magnificationphotomicrographslicefromthelowerpartoftherightupperlobeshowsmultipleconfluentgranulomasinfiltratingtheperibronchovascular(arrows)andsubpleural(arrowheads)interstitium.第十一頁，共二十三頁，編輯于2023年，星期一CTscanshowsmediastinallymphnodeenlargementandareticularpatternproducedbynodularityandthickeningofinterlobularsepta,pleuralsurfaces,andfissures,CT掃描顯示縱隔淋巴結腫大和形成網(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.從擴大的右氣管旁淋巴結細針穿刺活檢標本顯微鏡下顯示一組以淋巴細胞的背景的組織細胞，具有結節(jié)病肉芽腫細胞的結構特征。（C）從另一個病人的肺活檢標本的顯微照片顯示小葉間隔增厚（*），大量積累的肉芽腫結節(jié)（箭頭）。第十二頁，共二十三頁，編輯于2023年，星期一expiratoryCTscanobtainedinapatientwithpulmonarysarcoidosisshowsamosaicpatternconsistingofmultipleareasoflowattenuation(arrows)interspersedwithlargerareasofnormallungparenchyma.Thisappearanceisproducedbyairtrapping.Photomicrographsofatransbronchiallungbiopsyspecimenshowaccumulationsofsarcoidgranulomas(*)inthemucosalandsubmucosallayersofbronchiolarepithelium(arrowsinb).呼氣相CT掃描在肺結節(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。這種現(xiàn)象是由空氣滯留產(chǎn)生。一經(jīng)支氣管鏡肺活檢標本顯示的積累結節(jié)病肉芽腫的顯微照片（×）在粘膜和粘膜下的細支氣管上皮層（箭頭B）。第十三頁，共二十三頁，編輯于2023年，星期一

.(11a)CTscanshowsadiffuseground-glasspatternproducedbymultipleconfluentmicronodules,withassociatedbronchiectasis.CT掃描顯示彌漫性磨玻璃影，由多個融合的結節(jié)產(chǎn)生，伴支氣管擴張(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和伸入肺泡（箭頭）。第十四頁，共二十三頁，編輯于2023年，星期一SarcoidosisinapatientwithahistoryofstageIIIprimarycutaneousmalignantmelanoma.結節(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.第十五頁，共二十三頁，編輯于2023年，星期一Typical(a,b)andatypical(c,d)radiologicfindingsoflymphadenopathyinfourpatientswithsarcoidosis四例結節(jié)病淋巴結病變的影像學表現(xiàn).(a)Axialcontrastmaterial–enhancedCTscan(mediastinalwindow)showstypicalbilateralandsymmetrichilar(ar-rows)andsubcarinal(*)lymphadenopathy.CT增強掃描（縱隔窗）顯示了典型的雙側對稱性肺門（箭頭）及隆突下淋巴結腫大（*）(b)AxialunenhancedCTscan(mediastinalwindow)obtainedattheleveloftheleftpulmonaryarteryshowsenlargementofrightparatrachealandlefthilarlymphnodes(arrows)右氣管旁和左肺門淋巴結腫大.Althoughtherighthilumisnotshown,ittoowasaffected.(c)AxialunenhancedCTscan(mediastinalwindow)showspunctatecal-cificationsofhilarlymphnodes(arrows),apatternthatalsooccursinotherchronicgranulomatousdiseases.(d)Axialcontrast-enhancedCTscanshowsbilateraleggshell-likecalcificationsofhilarandmediastinallymphnodes雙側縱隔、肺門淋巴結蛋殼樣鈣化(arrows),findingsthatwarranttheinclusionofsilicosis矽肺inthedifferentialdiagnosisinthiscase.第十六頁，共二十三頁，編輯于2023年，星期一Axialhigh-resolutionCTscansobtainedattheleveloftheupperlobes(a)andcarina(b)inapatientwithpulmonarysarcoidosisshowafibrotic-cicatricialpatternofdisease,withmultiplelesionsinaperibronchovasculardistribution.Characteristicfeaturesofchronicdiseasearedepicted,includingtractionbronchiectasis牽拉性支氣管擴張,severearchitecturaldistortion,volumeloss,andinterlobularseptalthickening.Coales-centirregularmasslikeopacities(whitearrows)andacalcifiedrightlowerparatra-chealnode(blackarrowinb)alsoareseen.Mosaicattenuation,whichismostvisibleina,presumablyresultsfromairwaydistor-tionduetofibrosis.第十七頁，共二十三頁，編輯于2023年，星期一Axialhigh-resolutionCTscanshowsseverallarge,ill-definednodulesandareasofcon-solidationresultingfromtheconfluenceofmultipleparenchymalmicronodulescomposedofnumeroustinygranulomasinbothlungs.Finenodularopacitiesareseenaroundthelargenodules(whitearrows),andsmalllow-attenuationspotsthatcorrespondtothespacesbetweenpartiallycoalescentsmallnod-ulesarevisibleperipherally.Thisappearancehasbeentermedthesarcoid“galaxysign”小結節(jié)環(huán)繞大結節(jié)——銀河征.Distortionoftherightmajorfissureisalsoseen扭曲的葉間胸膜(blackarrow).第十八頁，共二十三頁，編輯于2023年，星期一Alveolarsarcoid多房型肉瘤patternofairspaceconsolidationinpulmonarysarcoidosis.Axialhigh-resolutionCTscanshowsalveolarconsolidationintheleftupperlobeandpatchysubpleuralalveolaropacitiesintherightupperlobe.Architecturaldistortionandtractionbronchiectasis,signsoffibrosis,alsoarevisible,mainlyintherightupperlobe.第十九頁，共二十三頁，編輯于2023年，星期一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節(jié)病。第二十頁，共二十三頁，編輯于2023年，星期一Miliaryopacitiesinsarcoidosis.粟粒樣結節(jié)病Axialun-enhancedhigh-resolutionCTscanshowscountlesstinymicronodulesrepresentingmultipleanddiffusegranulomasinarandomdistribution,withbronchialwallthickening.Whenthispatternisseen,thedifferentialdiagnosisshouldincludemiliarytuberculosis,pneumo-coniosis,