Clinical Investigation Of Primary Biliary Cirrhosis With Sj(?)gren's Syndrome

Objective 0To investigate the incidence,clinical characteristics,treatment and follow-up of primary biliary cirrhosis(PBC)with Sj(o|¨)gren's syndrome(SS).MethodsWe analyzed retrospectively the clinical manifestations,laboratory,auxiliary examination, treatment and follow-up of hospitalized patients with PBC and SS.Results1.There were 47 cases with SS in patients who were diagnosed with PBC of 170 cases. We selected 34 cases to study.2.In these 34 cases,the main clinical symptoms were jaundice,dry mouth,splenomegaly, hepatomegaly,dryness of eye.The alkaline phosphatase(ALP),γ-glutamyltransferase(GGT), alanine aminotransferase(ALT),aspartate aminotransferase(AST)of almost cases elevated. There were 21 patients whose antinuclear antibodies were positive.3.Follow-up revealed that there were five patients died.Fifteen patients were lost to follow-up.Ten patients stopped taking medicine or received herbal treatment.Four patients received ursodeoxycholic acid(UDCA)combination methylprednisolone,mycophenolate mofetil(MMF)with cyclophosphamide(CTX)at least one year.All of these four cases,the indexes decreased,including ALT,AST,ALP,GGT,total bilirubin(TB)and erythrocyte sedimentation rate(ESR).There was no severely adverse effect in all patients.Conclusions1.PBC with SS occurs frequently.The patients' clinical manifestations include both PBC and SS.2.Combination UDCA,methylprednisolone,MMF with CTX for PBC with SS is effective with few side effect.