Autoimmunehepatitis(AIH)hasrarelybeendescribedasanautoimmuneparaneoplasticsyndromeofthymoma.ThiscaseistheseventhcaseofAIHrevealedbycholestasisfewyearsafterthediagnosisofthymomaandthefirstcasetreatedwithchemotherapyalone.Wereportinthispaperanewapproachtothisrareseverecondition.A29year-oldmanpresentedwithchestpainanddyspneawithahistoryofthymomasurgicallyremoved4yearsago.CTscanshowedtherecurrenceofananteriormediastinalmass.Biologyshowedelevatedliverenzymesandprofoundcholestasis.Nosignofviralortoxichepatitisorbileductabnormalitieswereobserved.Autoimmuneantibodies,exceptfortheanti-nuclearantibody,werenegative.LiverbiopsyshowedactivechronicAIH.ThepatientwasdiagnosedwithrecurrentthymomawithAIHandunderwent6cyclesofchemotherapy.Acompleteresponseonthymomaandcholestasiswasobtainedafter10monthsoffollow-up.SteroidsandimmunosuppressorsarethestandardtreatmentforAIH.Theeffectofchemotherapyasaspecifictreatmentofthisparaneoplasticsyndromeneedstobeconsidered.
