结节病在胃肠道的表现

What’sthis？1精选ppt毕锡文Sarcoidosisindigestivesystem2精选pptMultisystemdisease

3精选pptLung

4精选pptLung

5精选pptLung

6精选pptLung

7精选pptLung

8精选pptLymphnodes

9精选pptSkin

10精选pptEye

结膜肉芽肿虹膜肉芽肿11精选pptSalivarygland

12精选pptBone

13精选pptNS

14精选pptLarynx

15精选pptMuscle

16精选pptNose

17精选pptSarcoidosisindigestivesystem18精选pptEsophagus

ExtremelyrarePathologictypesSuperficialmucosalinvolvementMyopathicinvolvementExtrinsicesophagealcompressionfrommediastinal

lymphadenopathyAchalasialikeesophagealinvolvementsecondarytodirectinfiltrationofLESorentericnervoussystem19精选pptEsophagus

ClinicalmanifestationsDysphagiaWeightlossAchalasialikesymptoms20精选pptEsophagus

ImagingPlaquelikemucosallesions,mucosalirregularitiesStricturesEsophagealdilatationBulkymediastinal

lymphadenopathyDecreasedperistalsis(蠕动减弱)21精选pptMildlydilatedoesophagealbodyBariumhold-upinthedistaloesophagus

Abird-beakappearanceoftheoesophagogastricjunction

22精选pptStomach

Themostcommonform,10%ptswithsarcoidosishavegastricinvolvmentonautopsyAntrumisthemostcommonsitePathologictypesSubclinicalgastricsarcoidosisUlcerativegastricsarcoidosisInfiltrativegastricsarcoidosisPolypoidgastricsarcoidosis23精选pptStomach

PathologictypesSubclinicalgastricsarcoidosisThemostcommontypeofgastricsarcoidosisMostlyasymptomatic,incidentallydiscoveredwithgastricmucosalbiopsyMucosamayappearnormal(sarcoidosisrisesfrommuscularlayer)HyperemicwithsuperficialnodularityAtrophicgastritisinchroniccases,usuallybenign24精选pptStomach

Pathologictypes2.UlcerativegastricsarcoidosisCanpresentthroughoutthestomachOccurmorefrequentlyinantralmucosa,pylorus,andlessercurvatureEpigastricpain\upperGIbleeding\obstructionBothbenignandmalignant-lookingulcershavebeenreported25精选pptStomach

Pathologictypes3.InfiltrativegastricsarcoidosisLocalizedtype→thedistalpartofthestomach→smooth,coned-shapedantralnarrowinganddeformityDiffusedtype→linitis

plastica-likeappearance→shouldbedifferentiatedfromgastriccarcinoma4.PolypoidgastricsarcoidosisRareMultipleorsingle26精选pptStomach

ClinicalmanifestationsEpigastricpain(75%),usuallypostprandialNauseaVomitingBloatingEarlysatietyWeightloss25%ptswithupperGIbleeding,sometimessevereObstruction:extensiveretroperitonealadenopathymayextrinsicallycompressthegastricantrum27精选pptStomachLabtestGastricaspiratesACElevel>serumAnti-parietalcellAb〔+〕AntiH+/K+ATPasepumpAb〔+〕Derangedparietalcellfunction→Gastricacid↓→serumgastrin↑28精选pptconsiderablenodularityofthemucosainthegastricantrum.Thispatienthadpulmonarysarcoidosis,andendoscopicbiopsyspecimensrevealednoncaseatinggranulomasinthestomach29精选pptInthispatient,moreadvancedgastricsarcoidosisismanifestedbymarkedantralnarrowinganddeformity30精选ppt31精选pptSmallintestine

TheleastcommonformPathologictypesGranulomatousenteritis〔isolatedorasapartofdisseminatedGItractsarcoidosis〕ObstructionVillousatrophy→malabsorption32精选pptSmallintestine

ClinicalmanifestationsChronicdiarrheaAbdominalpainNauseaVomitingMalabsorption→protein-losingenteropathy→peripheraledemaGIhemorrhageMegaloblasticanemia→folatedeficiencyormalabsorptionofvitaminB12withterminalilealdiseaseorachlorhydria〔胃酸缺乏〕Obstruction→intrinsicorextrinsic〔肠道狭窄或淋巴结压迫〕33精选pptCASE134精选ppt35精选ppt36精选ppt37精选pptCASE2Markedcircumferentialthickeningoftheterminalileum

38精选pptCASE267Gawhole-bodyscanlungs(∗)nose(straightarrow)Lacrimalandparotidglandshemipelvis

midlinepelvicactivityrepresentsthebladder(B).39精选pptColon&RectumRareSigmoidcolonismostcommonsitePathologictypesStrictureandnarrowingPlaquelikelesionsUlcersFoldthickening〔结肠皱襞增厚〕FocalnodularityPolypoidlesionsHistologicevidenceofcolonicinvolvementhasbeenfoundingrosslynormalmucosa40精选pptColon&Rectum

ClinicalmanifestationsAbdominalpain〔>50%pts〕DiarrheaTenesmus〔里急后重〕Hematochezia〔血便〕Distention(腹胀)Obstruction〔mostlycausedbycompressionoflymphadenopathy〕ConstipationWeightloss41精选pptCASE142精选pptCASE143精选pptCASE144精选pptCASE2Irregularnarrowingoftherectosigmoidduetosarcoidosishastheappearanceofinflammatorydiseaseormalignancy45精选pptAppendix

Extremelyrare,only1caseamong50,000appendectomyspecimensAppendicitisPerforationAbscessformation46精选pptDiagnosisofGIsarcoidosis

Difficult,isolatedGIsarcoidosisisevenmorehardThediagnosisofGItractsarcoidosisissuggestedinapatientwithsystemicsarcoidosiswithGIsymptomsDemonstrationofnoncaseating

granulomainGItractisnecessary3. OthercausesofGItractgranuloma

shouldbeexcluded:Tuberculosis,fungalinfections,schistosomiasisVasculitis,ForeignbodyreactionsRadiationinjuryCrohn’sdiseaseMicroscopiccolitisWhipple’sdiseaseLymphomaandcarcinoma47精选pptDiagnosisofGIsarcoidosis

LabtestsCBC：一系or三系下降〔脾亢or骨髓浸润〕高血钙，高尿钙〔无高血钙也可高尿钙，由于marcophage产生VitD类似物导致〕血ACE↑为活动性指标，与病情相关Kveim-siltebach皮试〔不常用〕Ga67核素扫描：巨噬细胞摄取镓，纵膈和双侧肺门摄取增高(λ征)；泪腺、腮腺、唾液腺高浓聚〔熊猫脸〕48精选ppt

SarcoidosisORCrohn？

SarcoidosisCrohn瘘管和肛周病变少见多见ACE↑N肺部或淋巴结结节病多有多无Schaumannbodies可有无浸润深度肠壁浅层全层病变程度粘膜破坏和炎症反应轻重激素反应好，数天可缓解较前者差*结节病和Crohn病可合并存在，虽然极少见49精选pptCytoplasmicSchaumannbody50精选pptLiver

>50%ofptswithsarcoidosishavehepaticinvolvementbybiopsyand67–70%byautopsyOnly10-30%havelaboratoryevidenceofliverdiseaseClinicalmanifestationsUsuallyasymptomatic,rarelyorgandysfunctionFeverandarthralgias,althoughnotspecific,arepresentinthemajorityofindividualswithactivehepaticsarcoidosis

Chronicinflammationandfibrosis→portalhypertension,Budd-Chiarisyndrome,cirrhosis,andcarcinoma51精选pptLiver

ClinicalmanifestationsPruritis-commonRightupperquadrantabdominalpain-commonHepatomegaly-20%ptsclinicallyand50%ptsonCTJaundice-rareintrahepatic

granulomasintheportalspacethroughexternalcompressionofbileductsfromgranulomasinextrahepaticlymphnodes.52精选pptLiverLabtestALPand/orγ-GT↑,correlatehighlywithcholestasisandliverinvolvement50%ofasymptomaticptshavemildly↑ALT&ASTHyperglobulinemiaisalsocommonACE↑,60%ofpatientswithactivesarcoidosis,butlesssoinchronicsarcoidosis,andpatientsoncorticosteroids.NormalACElevelsdonotruleoutdiseaseACE↑canbehelpfulindifferentialdiagnosisCTorMRIrevealhepatomegalyandgranulomasrepresentedbymultiplehypointensenodules.53精选ppt54精选ppt55精选pptSpleen

Mostofteninthepresenceofsystemicdiseaseratherthanasanisolatedentity24-53%ofptsisinvolvedonFNAUsuallyasymptomatic,Splenomegaly〔5-14%〕,abdominalpain,andhematologicabnormalitiessuchasleukopenia〔20%〕.SplenicinfiltrationcanbehomogeneousorisolatedasmultiplegranulomatousnodulesCTorMRIaslow-attenuatinglesions,andcaneasilybeconfusedwithlymphoma,metastasesorinfection56精选ppt57精选ppt58精选ppt59精选pptPancreas

1–3%ofcasesonautopsy,rarelypresentssymptomaticallyAbdominalpain,weightloss,obstructivejaundice,nauseaandvomiting.ElevatedamylaseandlipasearepossibleDiffuselynodular〔50%〕,orapancreaticmass〔50%〕,mostlyintheheadofthepancreas.60精选pptPancreas

RadiologicalimagingDilatationofthecommonbileductandpancreaticductSolitaryill-definedpancreaticmassesMultiplemasseswithlowT1,mildhighT2onMRIEnlargedlymphnode61精选ppt62精选ppt63精选pptPeritoneumRareThemostfrequentclinicalpresentationisexudativeascites(bothbloodyandnon-bloody)andabdominalpainSingleormultiplelesionsCA125↑〔主要由腹膜间皮细胞在炎症时分泌〕Needbiopsytobedifferentiatedbetweencarcinomatosisandtuberculousperitonitis64精选pptLymphomaORsarcoidosis？65精选ppt66精选pptSarcoidosis:singlebulkymesentericlymphnodemimickingalymphoma

FazziP,SolfanelliS,MorelliG,etal.Sarcoidosis1995;