结节病在胃肠道的表现课件

What’sthis？1毕锡文Sarcoidosisindigestivesystem2Multisystemdisease

3Lung

5Lung

6Lung

7Lung

8Skin

10Salivarygland

12NS

14Larynx

15Muscle

16Nose

17Sarcoidosisindigestivesystem18Esophagus

ClinicalmanifestationsDysphagiaWeightlossAchalasialikesymptoms20Esophagus

ImagingPlaquelikemucosallesions,mucosalirregularitiesStricturesEsophagealdilatationBulkymediastinal

lymphadenopathyDecreasedperistalsis(蠕动减弱)21Stomach

Themostcommonform,10%ptswithsarcoidosishavegastricinvolvmentonautopsyAntrumisthemostcommonsitePathologictypesSubclinicalgastricsarcoidosisUlcerativegastricsarcoidosisInfiltrativegastricsarcoidosisPolypoidgastricsarcoidosis23Stomach

PathologictypesSubclinicalgastricsarcoidosisThemostcommontypeofgastricsarcoidosisMostlyasymptomatic,incidentallydiscoveredwithgastricmucosalbiopsyMucosamayappearnormal(sarcoidosisrisesfrommuscularlayer)HyperemicwithsuperficialnodularityAtrophicgastritisinchroniccases,usuallybenign24Stomach

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

plastica-likeappearance→shouldbedifferentiatedfromgastriccarcinoma4.PolypoidgastricsarcoidosisRareMultipleorsingle26Stomach

ClinicalmanifestationsEpigastricpain(75%),usuallypostprandialNauseaVomitingBloatingEarlysatietyWeightloss25%ptswithupperGIbleeding,sometimessevereObstruction:extensiveretroperitonealadenopathymayextrinsicallycompressthegastricantrum27StomachLabtestGastricaspiratesACElevel>serumAnti-parietalcellAb（+）AntiH+/K+ATPasepumpAb（+）Derangedparietalcellfunction→Gastricacid↓→serumgastrin↑28Inthispatient,moreadvancedgastricsarcoidosisismanifestedbymarkedantralnarrowinganddeformity3031Smallintestine

TheleastcommonformPathologictypesGranulomatousenteritis（isolatedorasapartofdisseminatedGItractsarcoidosis）ObstructionVillousatrophy→malabsorption32Smallintestine

ClinicalmanifestationsChronicdiarrheaAbdominalpainNauseaVomitingMalabsorption→protein-losingenteropathy→peripheraledemaGIhemorrhageMegaloblasticanemia→folatedeficiencyormalabsorptionofvitaminB12withterminalilealdiseaseorachlorhydria（胃酸不足）Obstruction→intrinsicorextrinsic（肠道狭窄或淋巴结压迫）33CASE134353637CASE2Markedcircumferentialthickeningoftheterminalileum

38CASE267Gawhole-bodyscanlungs(∗)nose(straightarrow)Lacrimalandparotidglandshemipelvis

midlinepelvicactivityrepresentsthebladder(B).39Colon&RectumRareSigmoidcolonismostcommonsitePathologictypesStrictureandnarrowingPlaquelikelesionsUlcersFoldthickening（结肠皱襞增厚）FocalnodularityPolypoidlesionsHistologicevidenceofcolonicinvolvementhasbeenfoundingrosslynormalmucosa40Colon&Rectum

ClinicalmanifestationsAbdominalpain（>50%pts）DiarrheaTenesmus（里急后重）Hematochezia（血便）Distention(腹胀)Obstruction（mostlycausedbycompressionoflymphadenopathy）ConstipationWeightloss41CASE142CASE143CASE144CASE2Irregularnarrowingoftherectosigmoidduetosarcoidosishastheappearanceofinflammatorydiseaseormalignancy45Appendix

Extremelyrare,only1caseamong50,000appendectomyspecimensAppendicitisPerforationAbscessformation46DiagnosisofGIsarcoidosis

Difficult,isolatedGIsarcoidosisisevenmorehardThediagnosisofGItractsarcoidosisissuggestedinapatientwithsystemicsarcoidosiswithGIsymptomsDemonstrationofnoncaseating

granulomainGItractisnecessary3. OthercausesofGItractgranuloma

shouldbeexcluded:Tuberculosis,fungalinfections,schistosomiasisVasculitis,ForeignbodyreactionsRadiationinjuryCrohn’sdiseaseMicroscopiccolitisWhipple’sdiseaseLymphomaandcarcinoma47DiagnosisofGIsarcoidosis

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

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49CytoplasmicSchaumannbody50Liver

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

Chronicinflammationandfibrosis→portalhypertension,Budd-Chiarisyndrome,cirrhosis,andcarcinoma51Liver

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

granulomasintheportalspacethroughexternalcompressionofbileductsfromgranulomasinextrahepaticlymphnodes.52LiverLabtestALPand/orγ-GT↑,correlatehighlywithcholestasisandliverinvolvement50%ofasymptomaticptshavemildly↑ALT&ASTHyperglobulinemiaisalsocommonACE↑,60%ofpatientswithactivesarcoidosis,butlesssoinchronicsarcoidosis,andpatientsoncorticosteroids.NormalACElevelsdonotruleoutdiseaseACE↑canbehelpfulindifferentialdiagnosisCTorMRIrevealhepatomegalyandgranulomasrepresentedbymultiplehypointensenodules.535455Spleen

Mostofteninthepresenceofsystemicdiseaseratherthanasanisolatedentity24-53%ofptsisinvolvedonFNAUsuallyasymptomatic,Splenomegaly（5-14%）,abdominalpain,andhematologicabnormalitiessuchasleukopenia（20%）.SplenicinfiltrationcanbehomogeneousorisolatedasmultiplegranulomatousnodulesCTorMRIaslow-attenuatinglesions,andcaneasilybeconfusedwithlymphoma,metastasesorinfection56575859Pancreas

1–3%ofcasesonautopsy,rarelypresentssymptomaticallyAbdominalpain,weightloss,obstructivejaundice,nauseaandvomiting.ElevatedamylaseandlipasearepossibleDiffuselynodular（50%）,orapancreaticmass（50%）,mostlyintheheadofthepancreas.60Pancreas

RadiologicalimagingDilatationofthecommonbileductandpancreaticductSolitaryill-definedpancreaticmassesMultiplemasseswithlowT1,mildhighT2onMRIEnlargedlymphnode616263PeritoneumRareThemostfrequentclinicalpresentationisexudative

ascites(bothbloodyandnon-bloody)andabdominalpainSingleormultiplelesionsCA125↑（主要由腹膜间皮细胞在炎症时分泌）Needbiopsytobedifferentiatedbetweencarcinomatosisandtuberculousperitonitis64LymphomaORsarcoidosis？6566Sarcoidosis:singlebulkymesentericlymphnodemimickingalymphoma

FazziP,SolfanelliS,MorelliG,etal.Sarcoidosis1995;12:75–77.Apreviouslyhealthymanpresentedwithacuteabdominalpainthatextendedfromtheleftlumbarar