Primary biliary cholangitis,formerly known as primary biliary cirrhosis,is a chronic,autoimmune,and cholestatic disease ameliorating the biliary epithelial system causing fibrosis and end-stage liver disease,over time...Primary biliary cholangitis,formerly known as primary biliary cirrhosis,is a chronic,autoimmune,and cholestatic disease ameliorating the biliary epithelial system causing fibrosis and end-stage liver disease,over time.Patients range from an asymptomatic phase early in the disease course,to symp-toms of decompensated cirrhosis later in its course.This review focuses on the current consensus on the epidemiology,diagnosis,and management of patients with primary biliary cholangitis.We also discuss established medical manage-ment as well as novel and investigational therapeutics in the pipeline for management of PBC.展开更多
To explore the impact of ursodeoxycholic acid(UDCA)on severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection and clinical outcomes in patients with autoimmune liver disease(AILD).Patients diagnosed with ...To explore the impact of ursodeoxycholic acid(UDCA)on severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection and clinical outcomes in patients with autoimmune liver disease(AILD).Patients diagnosed with AILD were enrolled and divided into a UDCA group and a non-UDCA group based on whether they received UDCA treatment.Relevant data were collected regarding AILD diagnosis,treatment,biochemical indicators,and imaging examination.The incidence of SARS-CoV-2 infection and the prognosis of AILD patients were observed.A total of 1,138 patients completed follow-up.The usage rate of hormone(P=0.003)and immunosuppressant(P=0.001)used for treating AILD in the non-UDCA group was markedly lower than in the UDCA group.The UDCA usage rate was markedly lower in SARS-CoV-2 infected patients than in uninfected patients(P=0.003).The rate of SARS-CoV-2 infection in the non-UDCA group was significantly higher than in the UDCA group(P=0.018).Logistic regression analysis showed that UDCA use(P=0.003)was correlated to a lower incidence of SARS-CoV-2,while immunosuppressant use(P=0.017)increased the incidence.Recovery time from SARS-CoV-2 infection was markedly longer for those receiving UDCA treatment than those in the non-UDCA group(P=0.018).UDCA is associated with low SARS-CoV-2 incidence in AILD patients,while immunosuppressant increases its incidence instead.Patients receiving UDCA treatment have a longer recovery time after being infected.展开更多
Background and Aims:Hypercholesterolemia is a common finding in patients with primary biliary cholangitis(PBC)and is a well-defined risk factor for cardiovascular disease.However,studies have been mixed on whether PBC...Background and Aims:Hypercholesterolemia is a common finding in patients with primary biliary cholangitis(PBC)and is a well-defined risk factor for cardiovascular disease.However,studies have been mixed on whether PBC patients do,in fact,have higher cardiovascular risk.The aim of this study is to review the current literature and provide an evidence-based assessment of cardiovascular risk in PBC patients.Methods:We performed a systematic literature search on PubMed re-garding patients with PBC and cardiovascular events from the database inception to July 1,2017.A total of 33 articles ful-filling our inclusion criteria were found.Results:The majority of the studies evaluated yielded no statistically significant dif-ference in cardiovascular disease in the PBC population com-pared to the general public.However,some reports found a statistically significantly increase in coronary artery disease.Several studies have looked at the specific lipid profile of pa-tients with PBC with hypocholesteremia.While these lipid ab-normalities differ by stage of disease,there is evidence to suggest that the specific lipid profile in PBC may have lower atherogenicity than in patients with hypercholesterolemia without PBC.Studies looking at patients with PBC with other risk factors for cardiovascular disease,such as hypertension and metabolic syndrome,have consistently found a higher risk for cardiovascular disease in these patients.Statin treat-ment is effective in reducing lipid levels and possibly improv-ing endothelial inflammation in patients with PBC with hypercholesterolemia.Conclusions:There is not enough evidence to suggest an increased risk of cardiovascular dis-ease in patients with PBC with hypercholesterolemia,except for those individuals with concomitant features of metabolic syndrome.In patients with PBC with no additional cardiovas-cular risk factors,individual risk/benefit discussion on lipid-lowering treatment should be considered.展开更多
Cholestatic liver diseases(CLDs)encompass a variety of disorders of abnormal bile formation and/or flow.CLDs often lead to progressive hepatic insult and injury and following the development of cirrhosis and associate...Cholestatic liver diseases(CLDs)encompass a variety of disorders of abnormal bile formation and/or flow.CLDs often lead to progressive hepatic insult and injury and following the development of cirrhosis and associated complications.Many such complications are clinically silent until they manifest with severe sequelae,including but not limited to life-altering symptoms,metabolic disturbances,cirrhosis,and hepatobiliary diseases as well as other malignancies.Primary sclerosing cholangitis(PSC)and primary biliary cholangitis(PBC)are the most common CLDs,and both relate to mutual as well as unique complications.This review provides an overview of PSC and PBC,with a focus on preventive measures aimed to reduce the incidence and severity of disease-related complications.展开更多
Primary biliary cholangitis(PBC)is an autoimmune disease that causes progressive destruction of the intrahepatic bile ducts.Although the pathogenesis of PBC is not completely understood,it is characterized by cholest...Primary biliary cholangitis(PBC)is an autoimmune disease that causes progressive destruction of the intrahepatic bile ducts.Although the pathogenesis of PBC is not completely understood,it is characterized by cholestatic inflammation resulting in a spectrum of cholangitis,varying degrees of fibrosis,and,potentially,cirrhosis.PBC affects all races;however,there have been few clinical studies of the disease in Asian countries,particularly in Southeast Asia,suggesting that PBC may be underreported in the Asian population.Here,we review the epidemiology,natural history,predictive and diagnostic features,and current and emerging treatments for PBC.Our goal is also to highlight current studies from Asian countries to provide a better understanding of PBC in this patient population.展开更多
文摘Primary biliary cholangitis,formerly known as primary biliary cirrhosis,is a chronic,autoimmune,and cholestatic disease ameliorating the biliary epithelial system causing fibrosis and end-stage liver disease,over time.Patients range from an asymptomatic phase early in the disease course,to symp-toms of decompensated cirrhosis later in its course.This review focuses on the current consensus on the epidemiology,diagnosis,and management of patients with primary biliary cholangitis.We also discuss established medical manage-ment as well as novel and investigational therapeutics in the pipeline for management of PBC.
基金the National Key Research and Development Program(2022YFC2603500,2022YFC2603505)Beijing Municipal Health Commission high-level public health technical personnel construction project,discipline leader-03-26,Beijing Hospitals Authority Clinical medicine Development of special funding support(XMLX 202127)+1 种基金the capital health research and development of special public health project(2022-1-2172)The Digestive Medical Coordinated Development Center of Beijing Hospitals Authority(XXZ0302).
文摘To explore the impact of ursodeoxycholic acid(UDCA)on severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection and clinical outcomes in patients with autoimmune liver disease(AILD).Patients diagnosed with AILD were enrolled and divided into a UDCA group and a non-UDCA group based on whether they received UDCA treatment.Relevant data were collected regarding AILD diagnosis,treatment,biochemical indicators,and imaging examination.The incidence of SARS-CoV-2 infection and the prognosis of AILD patients were observed.A total of 1,138 patients completed follow-up.The usage rate of hormone(P=0.003)and immunosuppressant(P=0.001)used for treating AILD in the non-UDCA group was markedly lower than in the UDCA group.The UDCA usage rate was markedly lower in SARS-CoV-2 infected patients than in uninfected patients(P=0.003).The rate of SARS-CoV-2 infection in the non-UDCA group was significantly higher than in the UDCA group(P=0.018).Logistic regression analysis showed that UDCA use(P=0.003)was correlated to a lower incidence of SARS-CoV-2,while immunosuppressant use(P=0.017)increased the incidence.Recovery time from SARS-CoV-2 infection was markedly longer for those receiving UDCA treatment than those in the non-UDCA group(P=0.018).UDCA is associated with low SARS-CoV-2 incidence in AILD patients,while immunosuppressant increases its incidence instead.Patients receiving UDCA treatment have a longer recovery time after being infected.
文摘Background and Aims:Hypercholesterolemia is a common finding in patients with primary biliary cholangitis(PBC)and is a well-defined risk factor for cardiovascular disease.However,studies have been mixed on whether PBC patients do,in fact,have higher cardiovascular risk.The aim of this study is to review the current literature and provide an evidence-based assessment of cardiovascular risk in PBC patients.Methods:We performed a systematic literature search on PubMed re-garding patients with PBC and cardiovascular events from the database inception to July 1,2017.A total of 33 articles ful-filling our inclusion criteria were found.Results:The majority of the studies evaluated yielded no statistically significant dif-ference in cardiovascular disease in the PBC population com-pared to the general public.However,some reports found a statistically significantly increase in coronary artery disease.Several studies have looked at the specific lipid profile of pa-tients with PBC with hypocholesteremia.While these lipid ab-normalities differ by stage of disease,there is evidence to suggest that the specific lipid profile in PBC may have lower atherogenicity than in patients with hypercholesterolemia without PBC.Studies looking at patients with PBC with other risk factors for cardiovascular disease,such as hypertension and metabolic syndrome,have consistently found a higher risk for cardiovascular disease in these patients.Statin treat-ment is effective in reducing lipid levels and possibly improv-ing endothelial inflammation in patients with PBC with hypercholesterolemia.Conclusions:There is not enough evidence to suggest an increased risk of cardiovascular dis-ease in patients with PBC with hypercholesterolemia,except for those individuals with concomitant features of metabolic syndrome.In patients with PBC with no additional cardiovas-cular risk factors,individual risk/benefit discussion on lipid-lowering treatment should be considered.
基金This work was supported in part by the United States National Center for Advancing Translational Sciences grant UL1TR000135.
文摘Cholestatic liver diseases(CLDs)encompass a variety of disorders of abnormal bile formation and/or flow.CLDs often lead to progressive hepatic insult and injury and following the development of cirrhosis and associated complications.Many such complications are clinically silent until they manifest with severe sequelae,including but not limited to life-altering symptoms,metabolic disturbances,cirrhosis,and hepatobiliary diseases as well as other malignancies.Primary sclerosing cholangitis(PSC)and primary biliary cholangitis(PBC)are the most common CLDs,and both relate to mutual as well as unique complications.This review provides an overview of PSC and PBC,with a focus on preventive measures aimed to reduce the incidence and severity of disease-related complications.
文摘Primary biliary cholangitis(PBC)is an autoimmune disease that causes progressive destruction of the intrahepatic bile ducts.Although the pathogenesis of PBC is not completely understood,it is characterized by cholestatic inflammation resulting in a spectrum of cholangitis,varying degrees of fibrosis,and,potentially,cirrhosis.PBC affects all races;however,there have been few clinical studies of the disease in Asian countries,particularly in Southeast Asia,suggesting that PBC may be underreported in the Asian population.Here,we review the epidemiology,natural history,predictive and diagnostic features,and current and emerging treatments for PBC.Our goal is also to highlight current studies from Asian countries to provide a better understanding of PBC in this patient population.
