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PulmonarySarcoidosis:TypicalManifestationsatHigh-ResolutionCTwithPathologicCorrelationRecentAdvancesinSarcoidosis

结节病CT典型表现结节病进展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-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-magnificationphotomicrographslicefromthelowerpartoftherightupperlobeshowsmultipleconfluentgranulomasinfiltratingtheperibronchovascular(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)。

.(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)。Typical(a,b)andatypical(c,d)radiologicfindingsoflymphadenopathyinfourpatientswithsarcoidosis四例结节病淋巴结病变的影像学表现.(a)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)obtained

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