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OrganLungPleuraLymphNodesSkinEyeNasalMucosa 鼻粘膜 Larynx 喉 BoneMarrowSpleenLiver 901 575 90252520515 4050 6060 90 OrganKidneyCalciumMetabolismNervousSystemBoneJointsHeartEndocrineGlandsParotidGlandGastrointestinalSystem Rare1 25525 505Rare10Rare 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 Sarcoidosisofthelungisprimarilyaninterstitiallungdiseaseinwhichtheinflammatoryprocessinvolvesthealveoli smallbronchi andsmallbloodvessels TypicalandAtypicalFeaturesofPulmonarySarcoidosisatHigh ResolutionCTTypicalfeatures1Lymphadenopathy hilar mediastinal rightparatracheal bilateral symmetric andwelldefined2Nodules micronodules 2 4mmindiameter welldefined bilateral macronodules 5mmindiameter coalescing 3Lymphangiticspread peribronchovascular subpleural interlobularseptal4Fibroticchanges reticularopacities architecturaldistortion tractionbronchiectasis bronchiolectasis volumelossBilateralperihilaropacities5Predominantupper 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 Atypicalfeatures1Lymphadenopathy unilateral isolated anteriorandposteriormediastinal2Airspaceconsolidation masslikeopacities conglomeratemasses solitarypulmonarynodules confluentalveolaropacities alveolarsarcoidpattern 3Ground glassopacities4Linearopacities 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 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 calcificationPleuralplaquelikeopacities9Mycetoma aspergilloma霉菌球 Duringthepastdecade advanceshavebeenmadeinthestudyofsarcoidosisTheMycobacteriumtuberculosiscatalase 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 GeneexpressionanalyseshavemoreclearlydefinedcytokinedysregulationinsarcoidosisCurrently 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 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 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 pulmonarysarcoidosisshowsthetypicalperilymphaticdistributionofmicronodules arrow 外周淋巴分布的微结节 Photomicrographofalungbiopsyspecimendemonstratesnumerousepithelioidgranulomas arrow surroundingthebronchialwallsandimmediatelybeneaththenormalbronchialepithelium arrowheads CTscanshowsmultiplemicronoduleswithaperibronchovasculardistributioninbothlungs predominantlyintheupperandmiddlelobes Oneclusterofnodulesintheperipheryoftheleftupperlobe arrow hascoalescedtoformaconglomeratelesion macronodule Coronalreformattedimagefromhigh resolutionCTclearlyshowsupper lobepredominanceofthemicronodules Low magnificationphotomicrograph slicefromthelowerpartoftherightupperlobeshowsmultipleconfluentgranulomasinfiltratingtheperibronchovascular arrows andsubpleural arrowheads interstitium 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 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 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 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 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 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 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 Alveolarsarcoid多房型肉瘤patternofairspaceconsolidationinpulmonarysarcoidosis Axialhigh resolutionCTscanshowsalveolarconsolidationintheleftupperlobeandpatchysubpleuralalveolaropacitiesintherightupperlobe Architecturaldistortionandtractionbronchiectasis signsoffibrosis alsoarevisible mainlyintherightupperlobe 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 Miliaryopacitiesinsarcoidosis 粟粒样结节病Axialun enhancedhigh resolutionCTscanshowscountlesstinymicronodulesrepresentingmultipleanddiffusegranulomasinarandomdistribution withbronchialwallthickening Whenthispatternisseen thedifferentialdiagnosisshouldincludemiliarytuberculosis pneumo coniosis andmetastaticlesions 无数的小结节表
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