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類似物導致〕血ACE↑為活動性指標，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輕重激素反應好，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;