結(jié)節(jié)病在胃腸道的表現(xiàn)

What’sthis？1精選ppt畢錫文Sarcoidosisindigestivesystem2精選pptMultisystemdisease

3精選pptLung

4精選pptLung

5精選pptLung

6精選pptLung

7精選pptLung

8精選pptLymphnodes

9精選pptSkin

10精選pptEye

結(jié)膜肉芽腫虹膜肉芽腫11精選pptSalivarygland

12精選pptBone

13精選pptNS

14精選pptLarynx

15精選pptMuscle

16精選pptNose

17精選pptSarcoidosisindigestivesystem18精選pptEsophagus

ExtremelyrarePathologictypesSuperficialmucosalinvolvementMyopathicinvolvementExtrinsicesophagealcompressionfrommediastinal

lymphadenopathyAchalasialikeesophagealinvolvementsecondarytodirectinfiltrationofLESorentericnervoussystem19精選pptEsophagus

ClinicalmanifestationsDysphagiaWeightlossAchalasialikesymptoms20精選pptEsophagus

ImagingPlaquelikemucosallesions,mucosalirregularitiesStricturesEsophagealdilatationBulkymediastinal

lymphadenopathyDecreasedperistalsis(蠕動減弱)21精選pptMildlydilatedoesophagealbodyBariumhold-upinthedistaloesophagus

Abird-beakappearanceoftheoesophagogastricjunction

22精選pptStomach

Themostcommonform,10%ptswithsarcoidosishavegastricinvolvmentonautopsyAntrumisthemostcommonsitePathologictypesSubclinicalgastricsarcoidosisUlcerativegastricsarcoidosisInfiltrativegastricsarcoidosisPolypoidgastricsarcoidosis23精選pptStomach

PathologictypesSubclinicalgastricsarcoidosisThemostcommontypeofgastricsarcoidosisMostlyasymptomatic,incidentallydiscoveredwithgastricmucosalbiopsyMucosamayappearnormal(sarcoidosisrisesfrommuscularlayer)HyperemicwithsuperficialnodularityAtrophicgastritisinchroniccases,usuallybenign24精選pptStomach

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

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

plastica-likeappearance→shouldbedifferentiatedfromgastriccarcinoma4.PolypoidgastricsarcoidosisRareMultipleorsingle26精選pptStomach

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

TheleastcommonformPathologictypesGranulomatousenteritis〔isolatedorasapartofdisseminatedGItractsarcoidosis〕ObstructionVillousatrophy→malabsorption32精選pptSmallintestine

ClinicalmanifestationsChronicdiarrheaAbdominalpainNauseaVomitingMalabsorption→protein-losingenteropathy→peripheraledemaGIhemorrhageMegaloblasticanemia→folatedeficiencyormalabsorptionofvitaminB12withterminalilealdiseaseorachlorhydria〔胃酸缺乏〕Obstruction→intrinsicorextrinsic〔腸道狹窄或淋巴結(jié)壓迫〕33精選pptCASE134精選ppt35精選ppt36精選ppt37精選pptCASE2Markedcircumferentialthickeningoftheterminalileum

38精選pptCASE267Gawhole-bodyscanlungs(?)nose(straightarrow)Lacrimalandparotidglandshemipelvis

midlinepelvicactivityrepresentsthebladder(B).39精選pptColon&RectumRareSigmoidcolonismostcommonsitePathologictypesStrictureandnarrowingPlaquelikelesionsUlcersFoldthickening〔結(jié)腸皺襞增厚〕FocalnodularityPolypoidlesionsHistologicevidenceofcolonicinvolvementhasbeenfoundingrosslynormalmucosa40精選pptColon&Rectum

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

Extremelyrare,only1caseamong50,000appendectomyspecimensAppendicitisPerforationAbscessformation46精選pptDiagnosisofGIsarcoidosis

Difficult,isolatedGIsarcoidosisisevenmorehardThediagnosisofGItractsarcoidosisissuggestedinapatientwithsystemicsarcoidosiswithGIsymptomsDemonstrationofnoncaseating

granulomainGItractisnecessary3. OthercausesofGItractgranuloma

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

LabtestsCBC：一系or三系下降〔脾亢or骨髓浸潤〕高血鈣，高尿鈣〔無高血鈣也可高尿鈣，由于marcophage產(chǎn)生VitD類似物導(dǎo)致〕血ACE↑為活動性指標(biāo)，與病情相關(guān)Kveim-siltebach皮試〔不常用〕Ga67核素掃描：巨噬細胞攝取鎵，縱膈和雙側(cè)肺門攝取增高(λ征)；淚腺、腮腺、唾液腺高濃聚〔熊貓臉〕48精選ppt

SarcoidosisORCrohn？

SarcoidosisCrohn瘺管和肛周病變少見多見ACE↑N肺部或淋巴結(jié)結(jié)節(jié)病多有多無Schaumannbodies可有無浸潤深度腸壁淺層全層病變程度粘膜破壞和炎癥反應(yīng)輕重激素反應(yīng)好，數(shù)天可緩解較前者差*結(jié)節(jié)病和Crohn病可合并存在，雖然極少見49精選pptCytoplasmicSchaumannbody50精選pptLiver

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

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

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

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

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

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

RadiologicalimagingDilatationofthecommonbileductandpancreaticductSolitaryill-definedpancreaticmassesMultiplemasseswithlowT1,mildhighT2onMRIEnlargedlymphnode61精選ppt62精選ppt63精選pptPeritoneumRareThemostfrequentclinicalpresentationisexudativeascites(bothbloodyandnon-bloody)andabdominalpainSingleormultiplelesionsCA125↑〔主要由腹膜間皮細胞在炎癥時分泌〕Needbiopsytobedifferentiatedbetweencarcinomatosisandtuberculousperitonitis64精選pptLymphomaORsarcoidosis？65精選ppt66精選pptSarcoidosis:singlebulkymesentericlymphnodemimickingalymphoma

FazziP,SolfanelliS,MorelliG,etal.Sarcoidosis1995;