結(jié)節(jié)病在胃腸道的表現(xiàn)

What’sthis？1整理課件畢錫文Sarcoidosisindigestivesystem2整理課件Multisystemdisease

3整理課件Lung

4整理課件Lung

5整理課件Lung

6整理課件Lung

7整理課件Lung

8整理課件Lymphnodes

9整理課件Skin

10整理課件Eye

結(jié)膜肉芽腫虹膜肉芽腫11整理課件Salivarygland

12整理課件Bone

13整理課件NS

14整理課件Larynx

15整理課件Muscle

16整理課件Nose

17整理課件Sarcoidosisindigestivesystem18整理課件Esophagus

ExtremelyrarePathologictypesSuperficialmucosalinvolvementMyopathicinvolvementExtrinsicesophagealcompressionfrommediastinal

lymphadenopathyAchalasialikeesophagealinvolvementsecondarytodirectinfiltrationofLESorentericnervoussystem19整理課件Esophagus

ClinicalmanifestationsDysphagiaWeightlossAchalasialikesymptoms20整理課件Esophagus

ImagingPlaquelikemucosallesions,mucosalirregularitiesStricturesEsophagealdilatationBulkymediastinal

lymphadenopathyDecreasedperistalsis(蠕動(dòng)減弱)21整理課件MildlydilatedoesophagealbodyBariumhold-upinthedistaloesophagus

Abird-beakappearanceoftheoesophagogastricjunction

22整理課件Stomach

Themostcommonform,10%ptswithsarcoidosishavegastricinvolvmentonautopsyAntrumisthemostcommonsitePathologictypesSubclinicalgastricsarcoidosisUlcerativegastricsarcoidosisInfiltrativegastricsarcoidosisPolypoidgastricsarcoidosis23整理課件Stomach

PathologictypesSubclinicalgastricsarcoidosisThemostcommontypeofgastricsarcoidosisMostlyasymptomatic,incidentallydiscoveredwithgastricmucosalbiopsyMucosamayappearnormal(sarcoidosisrisesfrommuscularlayer)HyperemicwithsuperficialnodularityAtrophicgastritisinchroniccases,usuallybenign24整理課件Stomach

Pathologictypes2.UlcerativegastricsarcoidosisCanpresentthroughoutthestomachOccurmorefrequentlyinantralmucosa,pylorus,andlessercurvatureEpigastricpain\upperGIbleeding\obstructionBothbenignandmalignant-lookingulcershavebeenreported25整理課件Stomach

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

plastica-likeappearance→shouldbedifferentiatedfromgastriccarcinoma4.PolypoidgastricsarcoidosisRareMultipleorsingle26整理課件Stomach

ClinicalmanifestationsEpigastricpain(75%),usuallypostprandialNauseaVomitingBloatingEarlysatietyWeightloss25%ptswithupperGIbleeding,sometimessevereObstruction:extensiveretroperitonealadenopathymayextrinsicallycompressthegastricantrum27整理課件StomachLabtestGastricaspiratesACElevel>serumAnti-parietalcellAb〔+〕AntiH+/K+ATPasepumpAb〔+〕Derangedparietalcellfunction→Gastricacid↓→serumgastrin↑28整理課件considerablenodularityofthemucosainthegastricantrum.Thispatienthadpulmonarysarcoidosis,andendoscopicbiopsyspecimensrevealednoncaseatinggranulomasinthestomach29整理課件Inthispatient,moreadvancedgastricsarcoidosisismanifestedbymarkedantralnarrowinganddeformity30整理課件31整理課件Smallintestine

TheleastcommonformPathologictypesGranulomatousenteritis〔isolatedorasapartofdisseminatedGItractsarcoidosis〕ObstructionVillousatrophy→malabsorption32整理課件Smallintestine

ClinicalmanifestationsChronicdiarrheaAbdominalpainNauseaVomitingMalabsorption→protein-losingenteropathy→peripheraledemaGIhemorrhageMegaloblasticanemia→folatedeficiencyormalabsorptionofvitaminB12withterminalilealdiseaseorachlorhydria〔胃酸缺乏〕Obstruction→intrinsicorextrinsic〔腸道狹窄或淋巴結(jié)壓迫〕33整理課件CASE134整理課件35整理課件36整理課件37整理課件CASE2Markedcircumferentialthickeningoftheterminalileum

38整理課件CASE267Gawhole-bodyscanlungs(?)nose(straightarrow)Lacrimalandparotidglandshemipelvis

midlinepelvicactivityrepresentsthebladder(B).39整理課件Colon&RectumRareSigmoidcolonismostcommonsitePathologictypesStrictureandnarrowingPlaquelikelesionsUlcersFoldthickening〔結(jié)腸皺襞增厚〕FocalnodularityPolypoidlesionsHistologicevidenceofcolonicinvolvementhasbeenfoundingrosslynormalmucosa40整理課件Colon&Rectum

ClinicalmanifestationsAbdominalpain〔>50%pts〕DiarrheaTenesmus〔里急后重〕Hematochezia〔血便〕Distention(腹脹)Obstruction〔mostlycausedbycompressionoflymphadenopathy〕ConstipationWeightloss41整理課件CASE142整理課件CASE143整理課件CASE144整理課件CASE2Irregularnarrowingoftherectosigmoidduetosarcoidosishastheappearanceofinflammatorydiseaseormalignancy45整理課件Appendix

Extremelyrare,only1caseamong50,000appendectomyspecimensAppendicitisPerforationAbscessformation46整理課件DiagnosisofGIsarcoidosis

Difficult,isolatedGIsarcoidosisisevenmorehardThediagnosisofGItractsarcoidosisissuggestedinapatientwithsystemicsarcoidosiswithGIsymptomsDemonstrationofnoncaseating

granulomainGItractisnecessary3. OthercausesofGItractgranuloma

shouldbeexcluded:Tuberculosis,fungalinfections,schistosomiasisVasculitis,ForeignbodyreactionsRadiationinjuryCrohn’sdiseaseMicroscopiccolitisWhipple’sdiseaseLymphomaandcarcinoma47整理課件DiagnosisofGIsarcoidosis

LabtestsCBC：一系or三系下降〔脾亢or骨髓浸潤(rùn)〕高血鈣，高尿鈣〔無(wú)高血鈣也可高尿鈣，由于marcophage產(chǎn)生VitD類(lèi)似物導(dǎo)致〕血ACE↑為活動(dòng)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(xì)胞攝取鎵，縱膈和雙側(cè)肺門(mén)攝取增高(λ征)；淚腺、腮腺、唾液腺高濃聚〔熊貓臉〕48整理課件

SarcoidosisORCrohn？

SarcoidosisCrohn瘺管和肛周病變少見(jiàn)多見(jiàn)ACE↑N肺部或淋巴結(jié)結(jié)節(jié)病多有多無(wú)Schaumannbodies可有無(wú)浸潤(rùn)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(jiàn)49整理課件CytoplasmicSchaumannbody50整理課件Liver

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

Chronicinflammationandfibrosis→portalhypertension,Budd-Chiarisyndrome,cirrhosis,andcarcinoma51整理課件Liver

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

granulomasintheportalspacethroughexternalcompressionofbileductsfromgranulomasinextrahepaticlymphnodes.52整理課件LiverLabtestALPand/orγ-GT↑,correlatehighlywithcholestasisandliverinvolvement50%ofasymptomaticptshavemildly↑ALT&ASTHyperglobulinemiaisalsocommonACE↑,60%ofpatientswithactivesarcoidosis,butlesssoinchronicsarcoidosis,andpatientsoncorticosteroids.NormalACElevelsdonotruleoutdiseaseACE↑c(diǎn)anbehelpfulindifferentialdiagnosisCTorMRIrevealhepatomegalyandgranulomasrepresentedbymultiplehypointensenodules.53整理課件54整理課件55整理課件Spleen

Mostofteninthepresenceofsystemicdiseaseratherthanasanisolatedentity24-53%ofptsisinvolvedonFNAUsuallyasymptomatic,Splenomegaly〔5-14%〕,abdominalpain,andhematologicabnormalitiessuchasleukopenia〔20%〕.SplenicinfiltrationcanbehomogeneousorisolatedasmultiplegranulomatousnodulesCTorMRIaslow-attenuatinglesions,andcaneasilybeconfusedwithlymphoma,metastasesorinfection56整理課件57整理課件58整理課件59整理課件Pancreas

1–3%ofcasesonautopsy,rarelypresentssymptomaticallyAbdominalpain,weightloss,obstructivejaundice,nauseaandvomiting.ElevatedamylaseandlipasearepossibleDiffuselynodular〔50%〕,orapancreaticmass〔50%〕,mostlyintheheadofthepancreas.60整理課件Pancreas

RadiologicalimagingDilatationofthecommonbileductandpancreaticductSolitaryill-definedpancreaticmassesMultiplemasseswithlowT1,mildhighT2onMRIEnlargedlymphnode61整理課件62整理課件63整理課件PeritoneumRareThemostfrequentclinicalpresentationisexudativeascites(bothbloodyandnon-bloody)andabdominalpainSingleormultiplelesionsCA125↑〔主要由腹膜間皮細(xì)胞在炎癥時(shí)分泌〕Needbiopsytobedifferentiatedbetweencarcinomatosisandtuberculousperitonitis64整理課件LymphomaORsarcoidosis？65整理課件66整理課件Sarcoidosis:singlebulkymesentericlymphnodemimickingalymphoma

FazziP,SolfanelliS,MorelliG,etal.Sarcoidosis1995;12:7