結(jié)節(jié)病在胃腸道的表現(xiàn)課件

What’sthis？1畢錫文Sarcoidosisindigestivesystem2Multisystemdisease

3Lung

5Lung

6Lung

7Lung

8Skin

10Salivarygland

12NS

14Larynx

15Muscle

16Nose

17Sarcoidosisindigestivesystem18Esophagus

ClinicalmanifestationsDysphagiaWeightlossAchalasialikesymptoms20Esophagus

ImagingPlaquelikemucosallesions,mucosalirregularitiesStricturesEsophagealdilatationBulkymediastinal

lymphadenopathyDecreasedperistalsis(蠕動(dòng)減弱)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（腸道狹窄或淋巴結(jié)壓迫）33CASE134353637CASE2Markedcircumferentialthickeningoftheterminalileum

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

midlinepelvicactivityrepresentsthebladder(B).39Colon&RectumRareSigmoidcolonismostcommonsitePathologictypesStrictureandnarrowingPlaquelikelesionsUlcersFoldthickening（結(jié)腸皺襞增厚）FocalnodularityPolypoidlesionsHistologicevidenceofcolonicinvolvementhasbeenfoundingrosslynormalmucosa40Colon&Rectum

ClinicalmanifestationsAbdominalpain（>50%pts）DiarrheaTenesmus（里急后重）Hematochezia（血便）Distention(腹脹)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

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)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↑c(diǎn)anbehelpfulindifferentialdiagnosisCTorMRIrevealhepatomegalyandgranulomasrepresentedbymultiplehypointensenodules.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↑（主要由腹膜間皮細(xì)胞在炎癥時(shí)分泌）Needbiopsytobedifferentiatedbetweencarcinomatosisandtuberculousperitonitis64LymphomaORsarcoidosis？6566Sarcoidosis:singlebulkymesentericlymphnodemimickingalymphoma

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