Primary biliary cholangitis(PBC)is a chronic cholestatic liver disease characterized by immune-driven destruction of small intrahepatic bile ducts leading a proportion of patients to hepatic failure over the years.Dia...Primary biliary cholangitis(PBC)is a chronic cholestatic liver disease characterized by immune-driven destruction of small intrahepatic bile ducts leading a proportion of patients to hepatic failure over the years.Diagnosis at early stages in concert with ursodeoxycholic acid treatment has been linked with prevention of disease progression in the majority of cases.Diagnosis of PBC in a patient with cholestasis relies on the detection of disease-specific autoantibodies,including anti-mitochondrial antibodies,and disease-specific anti-nuclear antibodies targeting sp100 and gp210.These autoantibodies assist the diagnosis of the disease,and are amongst few autoantibodies the presence of which is included in the diagnostic criteria of the disease.They have also become important tools evaluating disease prognosis.Herein,we summarize existing data on detection of PBC-related autoantibodies and their clinical significance.Moreover,we provide insight on novel autoantibodies and their possible prognostic role in PBC patients.展开更多
Background and Aims:The diagnostic value of primary biliary cholangitis(PBC)-specific antibodies in patients with elevated alkaline phosphatase(ALP)and gamma-glutamyl transferase(GGT)levels,and other identifiable caus...Background and Aims:The diagnostic value of primary biliary cholangitis(PBC)-specific antibodies in patients with elevated alkaline phosphatase(ALP)and gamma-glutamyl transferase(GGT)levels,and other identifiable causes,was unclear.Our study aimed to determine whether etiological treatments in PBC-specific antibody-positive patients could improve liver biochemical tests,thereby distinguishing them from individuals with PBC.Methods:We enrolled patients who were positive for PBC-specific antibodies and elevated ALP and/or GGT levels but with other identifiable etiologies.Changes in liver biochemistry following non-ursodeoxycholic acid etiological treatments were monitored.Results:A total of 155 patients with positive PBC-specific antibodies and elevated ALP and/or GGT levels due to non-PBC diseases were enrolled.Among them,100 patients were diagnosed with non-PBC liver diseases,mainly metabolic-associated fatty liver disease,drug-induced liver injury,and autoimmune hepatitis.Additionally,55 patients had non-liver diseases,predominantly connective tissue diseases.The median follow-up duration was 15.9(4.7-25.6)months.Among 141 patients who completed follow-up after receiving etiological treatments,85.1%(120/141)showed improvement in ALP and/or GGT levels,with 51.8%(73/141)achieving normalization of both ALP and GGT.However,68 patients continued to exhibit elevated ALP and/or GGT,with 55 patients displaying isolated GGT elevation and 11 patients showing liver histological changes not consistent with PBC.Conclusions:PBC-specific antibodies,along with elevated ALP and GGT levels,may occur in various non-PBC diseases.Etiological treatments may improve or even resolve cholestatic biochemistry.For these patients,initiating etiological treatment rather than immediately starting ursodeoxycholic acid therapy would be justified.展开更多
文摘Primary biliary cholangitis(PBC)is a chronic cholestatic liver disease characterized by immune-driven destruction of small intrahepatic bile ducts leading a proportion of patients to hepatic failure over the years.Diagnosis at early stages in concert with ursodeoxycholic acid treatment has been linked with prevention of disease progression in the majority of cases.Diagnosis of PBC in a patient with cholestasis relies on the detection of disease-specific autoantibodies,including anti-mitochondrial antibodies,and disease-specific anti-nuclear antibodies targeting sp100 and gp210.These autoantibodies assist the diagnosis of the disease,and are amongst few autoantibodies the presence of which is included in the diagnostic criteria of the disease.They have also become important tools evaluating disease prognosis.Herein,we summarize existing data on detection of PBC-related autoantibodies and their clinical significance.Moreover,we provide insight on novel autoantibodies and their possible prognostic role in PBC patients.
基金supported by a grant from the National Natural Science Foundation of China(No.82100633).
文摘Background and Aims:The diagnostic value of primary biliary cholangitis(PBC)-specific antibodies in patients with elevated alkaline phosphatase(ALP)and gamma-glutamyl transferase(GGT)levels,and other identifiable causes,was unclear.Our study aimed to determine whether etiological treatments in PBC-specific antibody-positive patients could improve liver biochemical tests,thereby distinguishing them from individuals with PBC.Methods:We enrolled patients who were positive for PBC-specific antibodies and elevated ALP and/or GGT levels but with other identifiable etiologies.Changes in liver biochemistry following non-ursodeoxycholic acid etiological treatments were monitored.Results:A total of 155 patients with positive PBC-specific antibodies and elevated ALP and/or GGT levels due to non-PBC diseases were enrolled.Among them,100 patients were diagnosed with non-PBC liver diseases,mainly metabolic-associated fatty liver disease,drug-induced liver injury,and autoimmune hepatitis.Additionally,55 patients had non-liver diseases,predominantly connective tissue diseases.The median follow-up duration was 15.9(4.7-25.6)months.Among 141 patients who completed follow-up after receiving etiological treatments,85.1%(120/141)showed improvement in ALP and/or GGT levels,with 51.8%(73/141)achieving normalization of both ALP and GGT.However,68 patients continued to exhibit elevated ALP and/or GGT,with 55 patients displaying isolated GGT elevation and 11 patients showing liver histological changes not consistent with PBC.Conclusions:PBC-specific antibodies,along with elevated ALP and GGT levels,may occur in various non-PBC diseases.Etiological treatments may improve or even resolve cholestatic biochemistry.For these patients,initiating etiological treatment rather than immediately starting ursodeoxycholic acid therapy would be justified.
