结节病在胃肠道的表现

What’sthis？1整理课件毕锡文Sarcoidosisindigestivesystem2整理课件Multisystemdisease

3整理课件Lung

4整理课件Lung

5整理课件Lung

6整理课件Lung

7整理课件Lung

8整理课件Lymphnodes

9整理课件Skin

10整理课件Eye

结膜肉芽肿虹膜肉芽肿11整理课件Salivarygland

12整理课件Bone

13整理课件NS

14整理课件Larynx

15整理课件Muscle

16整理课件Nose

17整理课件Sarcoidosisindigestivesystem18整理课件Esophagus

ExtremelyrarePathologictypesSuperficialmucosalinvolvementMyopathicinvolvementExtrinsicesophagealcompressionfrommediastinal

lymphadenopathyAchalasialikeesophagealinvolvementsecondarytodirectinfiltrationofLESorentericnervoussystem19整理课件Esophagus

ClinicalmanifestationsDysphagiaWeightlossAchalasialikesymptoms20整理课件Esophagus

ImagingPlaquelikemucosallesions,mucosalirregularitiesStricturesEsophagealdilatationBulkymediastinal

lymphadenopathyDecreasedperistalsis(蠕动减弱)21整理课件MildlydilatedoesophagealbodyBariumhold-upinthedistaloesophagus

Abird-beakappearanceoftheoesophagogastricjunction

22整理课件Stomach

Themostcommonform,10%ptswithsarcoidosishavegastricinvolvmentonautopsyAntrumisthemostcommonsitePathologictypesSubclinicalgastricsarcoidosisUlcerativegastricsarcoidosisInfiltrativegastricsarcoidosisPolypoidgastricsarcoidosis23整理课件Stomach

PathologictypesSubclinicalgastricsarcoidosisThemostcommontypeofgastricsarcoidosisMostlyasymptomatic,incidentallydiscoveredwithgastricmucosalbiopsyMucosamayappearnormal(sarcoidosisrisesfrommuscularlayer)HyperemicwithsuperficialnodularityAtrophicgastritisinchroniccases,usuallybenign24整理课件Stomach

Pathologictypes2.UlcerativegastricsarcoidosisCanpresentthroughoutthestomachOccurmorefrequentlyinantralmucosa,pylorus,andlessercurvatureEpigastricpain\upperGIbleeding\obstructionBothbenignandmalignant-lookingulcershavebeenreported25整理课件Stomach

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

plastica-likeappearance→shouldbedifferentiatedfromgastriccarcinoma4.PolypoidgastricsarcoidosisRareMultipleorsingle26整理课件Stomach

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

TheleastcommonformPathologictypesGranulomatousenteritis〔isolatedorasapartofdisseminatedGItractsarcoidosis〕ObstructionVillousatrophy→malabsorption32整理课件Smallintestine

ClinicalmanifestationsChronicdiarrheaAbdominalpainNauseaVomitingMalabsorption→protein-losingenteropathy→peripheraledemaGIhemorrhageMegaloblasticanemia→folatedeficiencyormalabsorptionofvitaminB12withterminalilealdiseaseorachlorhydria〔胃酸缺乏〕Obstruction→intrinsicorextrinsic〔肠道狭窄或淋巴结压迫〕33整理课件CASE134整理课件35整理课件36整理课件37整理课件CASE2Markedcircumferentialthickeningoftheterminalileum

38整理课件CASE267Gawhole-bodyscanlungs(∗)nose(straightarrow)Lacrimalandparotidglandshemipelvis

midlinepelvicactivityrepresentsthebladder(B).39整理课件Colon&RectumRareSigmoidcolonismostcommonsitePathologictypesStrictureandnarrowingPlaquelikelesionsUlcersFoldthickening〔结肠皱襞增厚〕FocalnodularityPolypoidlesionsHistologicevidenceofcolonicinvolvementhasbeenfoundingrosslynormalmucosa40整理课件Colon&Rectum

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

Extremelyrare,only1caseamong50,000appendectomyspecimensAppendicitisPerforationAbscessformation46整理课件DiagnosisofGIsarcoidosis

Difficult,isolatedGIsarcoidosisisevenmorehardThediagnosisofGItractsarcoidosisissuggestedinapatientwithsystemicsarcoidosiswithGIsymptomsDemonstrationofnoncaseating

granulomainGItractisnecessary3. OthercausesofGItractgranuloma

shouldbeexcluded:Tuberculosis,fungalinfections,schistosomiasisVasculitis,ForeignbodyreactionsRadiationinjuryCrohn’sdiseaseMicroscopiccolitisWhipple’sdiseaseLymphomaandcarcinoma47整理课件DiagnosisofGIsarcoidosis

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整理课件

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整理课件CytoplasmicSchaumannbody50整理课件Liver

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

Chronicinflammationandfibrosis→portalhypertension,Budd-Chiarisyndrome,cirrhosis,andcarcinoma51整理课件Liver

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

granulomasintheportalspacethroughexternalcompressionofbileductsfromgranulomasinextrahepaticlymphnodes.52整理课件LiverLabtestALPand/orγ-GT↑,correlatehighlywithcholestasisandliverinvolvement50%ofasymptomaticptshavemildly↑ALT&ASTHyperglobulinemiaisalsocommonACE↑,60%ofpatientswithactivesarcoidosis,butlesssoinchronicsarcoidosis,andpatientsoncorticosteroids.NormalACElevelsdonotruleoutdiseaseACE↑canbehelpfulindifferentialdiagnosisCTorMRIrevealhepatomegalyandgranulomasrepresentedbymultiplehypointensenodules.53整理课件54整理课件55整理课件Spleen

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

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

RadiologicalimagingDilatationofthecommonbileductandpancreaticductSolitaryill-definedpancreaticmassesMultiplemasseswithlowT1,mildhighT2onMRIEnlargedlymphnode61整理课件62整理课件63整理课件PeritoneumRareThemostfrequentclinicalpresentationisexudativeascites(bothbloodyandnon-bloody)andabdominalpainSingleormultiplelesionsCA125↑〔主要由腹膜间皮细胞在炎症时分泌〕Needbiopsytobedifferentiatedbetweencarcinomatosisandtuberculousperitonitis64整理课件LymphomaORsarcoidosis？65整理课件66整理课件Sarcoidosis:singlebulkymesentericlymphnodemimickingalymphoma

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