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1、Diagnosisandtreatmentofautoimmunehepatitis(AIH)Kai-ChunFanIntroductionAIHisaprogressiveinflammatoryhepatitisofunknownetiologythatisresponsivetoimmunosuppressivetherapy.Itischaracterizedbythepresenceofinterfacehepatitisandportalplasmacellinfiltrationonhistologicexamination,hypergammaglobulin
2、emiaandautoantibodies.Patientsarefrequentlyasymptomatic(34%).Anacuteonsetofillnessiscommon(40%).Thefulminantpresentationispossible.Womenareaffectedmorethanmen(genderratio3.6:1),andallagesandethnicgroupsaresusceptible.Diagnosticcriteriainadults(recommendationsoftheinternationalAIHGroup)Nogen
3、eticliverdisease:normalα1antitrypsinphenotype(noα1antitrypsindeficiency);normalserumcopper,ceruloplasmin(noWillsondisease),ironand/orferritin(nohemochromatosis)Noactiveviralinfection:nomarkersofcurrentinfectionwithhepatitisA,BandCvirusesNotoxicoralcoholinjury:norecentuseofhepatotoxicdrugsan
4、ddailyalcohol<25gDiagnosticcriteriainadults(recommendationsoftheinternationalAIHGroup)Laboratoryfeatures:Predominantserumaminotransferaseabnormality;γ-globulinorIgG≥1.5timesnormalAutoantibodies:antinuclearantibodies(ANA),smoothmuscleantibodies(SMA),antibodiestoliver/kidneymicrosometype1(ant
5、i-LKM1)≥1:80inadults;noantimitochondrialantibodies(AMA)Histologicfinding:interfacehepatitis;nobiliarylesions(primarysclerosingcholangitis,primarybiliarycirrhosis,autoimmunecholangitis,ortheoverlapsyndromes),granulomas,orprominentchangessuggestiveofanotherdisease(steatosis,ironoverload,Wills
6、ondisease,chronichepatitisC,drugtoxicity,etc)DiagnosticscoringsystemforAIHinadults(recommendationsoftheinternationalAIHGroup)CategoryFactorScoreGenderFemale+2AKP/ASTorALTratio<1.5+3>3-2γ-globulinorIgG>2timesnormal+31.5-2.0timesnormal+21.0-1.5timesnormal+1<1.0timesnormal+0Diagnosticscoringsy
7、stemforAIHinadultsANA,SMA,anti-LKM1≥1:80+31:80+21:40+1<1:40+0AMApositive-4MarkersofcurrentinfectionwithhepatitisA,BandCvirusesNegative+3Positive-3HepatotoxicdrugsNo+1Yes-4DiagnosticscoringsystemforAIHinadultsAlcohol<25g/d+2>60g/d-2Concurrentimmunedisease