结节病典型和不典型型表现

OrganLungPleuraLymphNodesSkinEyeNasalMucosa（鼻粘膜）Larynx（喉）BoneMarrowSpleenLiver%901-575-90252520515-4050-6060-90OrganKidneyCalciumMetabolismNervousSystemBoneJointsHeartEndocrineGlandsParotidGlandGastrointestinalSystem%Rare1-25525-505Rare10Rare当前第1页\共有22页\编于星期五\11点Sarcoidosisisamultisystemdisorderthatischaracterizedbynoncaseousepithelioidcellgranulomas,whichmayaffectalmostanyorgan.结节病是一种以非干酪样坏死的上皮细胞肉芽肿为特点的多系统疾病，几乎可以累及所有器官。Sarcoidosismaybeasymptomaticorchronic.Itcommonlyimprovesorclearsupspontaneously（自愈或好转）.Morethan2/3ofpeoplewithlungsarcoidosishavenosymptomsafter9years.About50%haverelapses.About10%developseriousdisability.当前第2页\共有22页\编于星期五\11点

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结节病原本是肺间质性病变，累及肺泡，支气管和小血管淋巴结增大，两侧对称，境界清楚大小结节淋巴管播散，支气管血管鞘，胸膜下，小叶间隔纤维化改变，网状阴影，肺结构扭曲，牵拉性支扩，肺容积缩小，两肺门旁致密影中上肺为主典型特征当前第3页\共有22页\编于星期五\11点

Atypicalfeatures1Lymphadenopathy:unilateral,isolated,anteriorandposteriormediastinal

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

3Ground-glassopacities

4Linearopacities:interlobularseptalthickening,intralobularlinearopacities

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

单侧孤立前后纵隔淋巴结增大肺泡实变：肿块，实质性结节，实变融合毛玻璃阴影线状阴影小叶间隔增厚小叶年线状阴影不典型表现囊性纤维化改变：囊，大泡，小泡，肺气肿，蜂窝样改变当前第4页\共有22页\编于星期五\11点6Miliaryopacities

粟粒样改变

7Airwayinvolvement:mosaicattenuationpattern,tracheobronchialabnormalities,atelectasis

气道累及：马赛克改变，气管支气管异常，肺不张8Pleuraldisease:effusion,chylothorax,hemothorax,pneumothorax,pleuralthickening,calcificationPleuralplaquelikeopacities

9Mycetoma,aspergilloma

霉菌球当前第5页\共有22页\编于星期五\11点Duringthepastdecade,advanceshavebeenmadeinthestudyofsarcoidosis

TheMycobacteriumtuberculosiscatalase-peroxidase(mKatG)protein,apotentialantigen,hasbeenidentified,结核分枝杆菌过氧化氢酶过氧化物酶（mkatg）蛋白，作为一个潜在的抗原已被确定。PETscanninghasprovenvaluableinlocatingoccultsitesfordiagnosticbiopsy.PET扫描已被证明在定位活检诊断隐匿性病灶的价值

Endobronchialultrasound-guidedtransbronchialneedleaspiration(EBUS-TBNA)ofmediastinallymphnodeshasfacilitateddiagnosis,ofteneliminatingtheneedformore-invasiveprocedures,suchasmediastinoscopy.支气管内超声引导下经支气管针吸活检（EBUS-TBNA）的纵隔淋巴结有利于诊断，往往避免更具侵袭性的操作，如纵隔镜。Geneexpressionanalyseshavemoreclearlydefinedcytokinedysregulationinsarcoidosis

Currently,noreliableprognosticbiomarkershavebeenidentified.目前，还没有可靠的预后标志物已被确定。Thetumornecrosisfactor(TNF)inhibitors,arelativelynewclassofsteroid-sparingagents,havebeenusedinpatientswithrefractorydisease.Itisunclearwhethermedicationsusedtotreatpulmonaryarterialhypertensionaresafeandeffectiveforthetreatmentofsarcoidosis-associatedpulmonaryhypertension.肿瘤坏死因子（TNF）抑制剂，一个相对较新的类固醇保代理类药品，已被用于治疗难治性疾病。目前还不清楚是否用于治疗肺动脉高压的药物治疗结节病相关性肺动脉高压是安全和有效的。当前第6页\共有22页\编于星期五\11点PathologicCorrelation相关病理

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

肺实质肉芽肿分布与支气管血管鞘，胸膜下结缔组织，小叶间隔中淋巴管相关.

ThickenedbronchovascularbundlesandsmallperivascularnodulesseenatCTcorrespondedtogranulomaswithintheconnectivetissuesheathsurroundingpulmonaryairwaysandvessels.PleuralorsubpleuralnoduleswerecorrelatedwithgranulomasadjacenttothevisceralpleuraCT上支气管血管鞘增厚和小结节是与包绕气道血管结缔组织鞘中肉芽肿，胸膜和胸膜下结节与脏层胸膜旁肉芽肿相关。

当前第7页\共有22页\编于星期五\11点Ground-glassopacitiesrepresentedanaccumulationofmanygranulomatouslesions,withorwithoutfibrosis,inthealveolarseptaandaroundthesmallvessels.Noalveolitiswasseen

肺泡间隔小血管周围大量肉芽肿是毛玻璃阴影主要原因，可伴纤维化，但没有肺泡炎。

Largeparenchymalnodules(>1cmindiameter)representedcoalescentgranulomas

大结节是肉芽肿病变的融合

AirbronchiologramswithinregionsofdenseconsolidationonCTimagescorrespondedtobronchiolardilatationwithsurroundingfibrosis

支气管充气症是纤维化旁的支扩honeycomb-likepatternofmicroscopiccystsseenatpathologicanalysis.

蜂窝样改变在显微镜下就是很多的小囊当前第8页\共有22页\编于星期五\11点pulmonarysarcoidosisshowsthetypicalperilymphaticdistributionofmicronodules(arrow).

（外周淋巴分布的微结节）

Photomicrographofalungbiopsyspecimendemonstratesnumerousepithelioidgranulomas(arrow)surroundingthebronchialwallsandimmediatelybeneaththenormalbronchialepithelium(arrowheads).当前第9页\共有22页\编于星期五\11点CTscanshowsmultiplemicronoduleswithaperibronchovasculardistributioninbothlungs,predominantlyintheupperandmiddlelobes.Oneclusterofnodulesintheperipheryoftheleftupperlobe(arrow)hascoalescedtoformaconglomeratelesion(macronodule).

Coronalreformattedimagefromhigh-resolutionCTclearlyshowsupper-lobepredominanceofthemicronodules.

Low-magnificationphotomicrographslicefromthelowerpartoftherightupperlobeshowsmultipleconfluentgranulomasinfiltratingtheperibronchovascular(arrows)andsubpleural(arrowheads)interstitium.当前第10页\共有22页\编于星期五\11点CTscanshowsmediastinallymphnodeenlargementandareticularpatternproducedbynodularityandthickeningofinterlobularsepta,pleuralsurfaces,andfissures,CT扫描显示纵隔淋巴结肿大和形成网状图案的小叶间隔增厚，及胸膜表面，及产生的裂缝。

(b)Photomicrographofaspecimenfromfine-needleaspirationbiopsyofanenlargedrightparatracheallymphnodeshowsagroupofhistiocytesagainstalymphocyticbackground,acytologicstructurecharacteristicofsarcoidgranuloma.(c)Photomicrographofalungbiopsyspecimenfromanotherpatientshowsprogressivethickeningoftheinterlobularseptum(*)becauseoftheaccumulationofnumeroussarcoidgranulomas(arrowheads),anappearancethatcorrelateswellwiththeCTfeaturesseenina.从扩大的右气管旁淋巴结细针穿刺活检标本显微镜下显示一组以淋巴细胞的背景的组织细胞，具有结节病肉芽肿细胞的结构特征。（C）从另一个病人的肺活检标本的显微照片显示小叶间隔增厚（*），大量积累的肉芽肿结节（箭头）。当前第11页\共有22页\编于星期五\11点expiratoryCTscanobtainedinapatientwithpulmonarysarcoidosisshowsamosaicpatternconsistingofmultipleareasoflowattenuation(arrows)interspersedwithlargerareasofnormallungparenchyma.Thisappearanceisproducedbyairtrapping.Photomicrographsofatransbronchiallungbiopsyspecimenshowaccumulationsofsarcoidgranulomas(*)inthemucosalandsubmucosallayersofbronchiolarepithelium(arrowsinb).呼气相CT扫描在肺结节病患者显示马赛克征，由低密度的多个区域（箭头）穿插着大面积的正常肺实质。这种现象是由空气滞留产生。一经支气管镜肺活检标本显示的积累结节病肉芽肿的显微照片（×）在粘膜和粘膜下的细支气管上皮层（箭头B）。当前第12页\共有22页\编于星期五\11点

.(11a)CTscanshowsadiffuseground-glasspatternproducedbymultipleconfluentmicronodules,withassociatedbronchiectasis.CT扫描显示弥漫性磨玻璃影，由多个融合的结节产生，伴支气管扩张(11b)Magnifiedaxialhigh-resolutionCTscanoftherightlungclearlydepictsseparatenodulesinasubpleural(blackarrow)andfissural(whitearrow)distributionandalongthebronchovascularbundles(arrowheads)放大的轴位高分辨率CT扫描清楚显示右肺胸膜下区分胸膜下的结节（黑色箭头）和沿支气管血管束分布的“裂”（白色箭头）.(11c)High-powerphotomicrographshowsanaccumulationofinterstitialgranulomas(white*),whichcausesathickenedappearanceoftheinteralveolarsepta,andacinargranulomas(black*),whichformintheinterstitiumofthealveolarwallandprotrudeintothealveoli(arrowheads).高倍镜下涂片显示间质肉芽肿集聚（白*），使肺小泡壁的增厚，和腺泡肉芽肿（黑色），形成在肺泡壁的间质和伸入肺泡（箭头）。当前第13页\共有22页\编于星期五\11点SarcoidosisinapatientwithahistoryofstageIIIprimarycutaneousmalignantmelanoma.结节病III期原发性皮肤恶性黑色素瘤病史。(a)Contrast-enhancedCTscansshowpulmonarynodules(arrow)insubpleural(right)andfissural(left)regions.Avideo-assistedthoracoscopicsurgicalbiopsywasperformed.(b)Low-powerphotomicrograph(originalmagnification,×10;H-Estain)obtainedathistopathologicanalysisshowsasubpleuralnodulethatisdarkerincolorbecauseofanthracosis（尘肺）.(c)Photo-micrographobtainedathigherpower(originalmagnification,×100;H-Estain)showsmultiplenonnecroticgranulomas（坏死性肉芽肿）(arrows)expandingtheinterstitiumthatsurroundsthesubpleuralnoduleinb.当前第14页\共有22页\编于星期五\11点Typical(a,b)andatypical(c,d)radiologicfindingsoflymphadenopathyinfourpatientswithsarcoidosis四例结节病淋巴结病变的影像学表现.(a)Axialcontrastmaterial–enhancedCTscan(mediastinalwindow)showstypicalbilateralandsymmetrichilar(ar-rows)andsubcarinal(*)lymphadenopathy.CT增强扫描（纵隔窗）显示了典型的双侧对称性肺门（箭头）及隆突下淋巴结肿大（*）(b)AxialunenhancedCTscan(mediastinalwindow)obtainedattheleveloftheleftpulmonaryarteryshowsenlargementofrightparatrachealandlefthilarlymphnodes(arrows)右气管旁和左肺门淋巴结肿大.Althoughtherighthilumisnotshown,ittoowasaffected.(c)AxialunenhancedCTscan(mediastinalwindow)showspunctatecal-cificationsofhilarlymphnodes(arrows),apatternthatalsooccursinotherchronicgranulomatousdiseases.(d)Axialcontrast-enhancedCTscanshowsbilateraleggshell-likecalcificationsofhilarandmediastinallymphnodes双侧纵隔、肺门淋巴结蛋壳样钙化(arrows),findingsthatwarranttheinclusionofsilicosis矽肺inthedifferentialdiagnosisinthiscase.当前第15页\共有22页\编于星期五\11点Axialhigh-resolutionCTscansobtainedattheleveloftheupperlobes(a)andcarina(b)inapatientwithpulmonarysarcoidosisshowafibrotic-cicatricialpatternofdisease,withmultiplelesionsinaperibronchovasculardistribution.Characteristicfeaturesofchronicdiseasearedepicted,includingtractionbronchiectasis牵拉性支气管扩张,severearchitecturaldistortion,volumeloss,andinterlobularseptalthickening.Coales-centirregularmasslikeopacities(whitearrows)andacalcifiedrightlowerparatra-chealnode(blackarrowinb)alsoareseen.Mosaicattenuation,whichismostvisibleina,presumablyresultsfromairwaydistor-tionduetofibrosis.当前第16页\共有22页\编于星期五\11点Axialhigh-resolutionCTscanshowsseverallarge,ill-definednodulesandareasofcon-solidationresultingfromtheconfluenceofmultipleparenchymalmicronodulescomposedofnumeroustinygranulomasinbothlungs.Finenodularopacitiesareseenaroundthelargenodules(whitearrows),andsmalllow-attenuationspotsthatcorrespondtothespacesbetweenpartiallycoalescentsmallnod-ulesarevisibleperipherally.Thisappearancehasbeentermedthesarcoid“galaxysign”小结节环绕大结节——银河征.Distortionoftherightmajorfissureisalsoseen扭曲的叶间胸膜(blackarrow).当前第17页\共有22页\编于星期五\11点Alveolarsarcoid多房型肉瘤patternofairspaceconsolidationinpulmonarysarcoidosis.Axialhigh-resolutionCTscanshowsalveolarconsolidationintheleftupperlobeandpatchysubpleuralalveolaropacitiesintherightupperlobe.Architecturaldistortionandtractionbronchiectasis,signsoffibrosis,alsoarevisible,mainlyintherightupperlobe.当前第18页\共有22页\编于星期五\11点Axialunenhancedhigh-resolutionCTscanshowsasymmetricsubpleuralhoneycomb-likecysts不对称胸膜下蜂窝样囊肿(ar-rowheads)andarchitecturaldistortionassociatedwithleftfissurenodularity(arrow).Althoughthesefeaturesalsoarecharacteristicofidiopathicpulmonaryfibrosis特发性肺纤维化,theupper-lobepredominanceofhoneycomb-likecystsandtheperibronchovascularandfissuraldistributionofmicronodulesinthiscaseweremoresuggestiveofsarcoidosis.以上叶为主的蜂窝状囊，沿支气管血管束周围分布并且更提示结节病。当前第19页\共有22页\编于星期五\11点Miliaryopacitiesinsarcoidosis.粟粒样结节病Axialun-enhancedhigh-resolutionCTscanshowscountlesstinymicronodulesrepresentingmultipleanddiffusegranulomasinarandomdistribution,withbronchialwallthickening.Whenthispatternisseen,thedifferentialdiagnosisshouldincludemiliarytuberculosis,pneumo-coniosis,