Primary sclerosing cholangitis(PSC)is a rare,chronic,cholestatic liver disease of uncertain etiology characterized biochemically by cholestasis and histologically and cholangiographically by fibroobliterative inflamma...Primary sclerosing cholangitis(PSC)is a rare,chronic,cholestatic liver disease of uncertain etiology characterized biochemically by cholestasis and histologically and cholangiographically by fibroobliterative inflammation of the bile ducts.In a clinically significant proportion of patients,PSC progresses to cirrhosis,end-stage liver disease,and/or hepatobiliary cancer,though the disease course can be highly variable.Despite clinical trials of numerous pharmacotherapies over several decades,safe and effective medical therapy remains to be established.Liver transplantation is an option for select patients with severe complications of PSC,and its outcomes are generally favorable.Periodic surveillance testing for pre-as well as post-transplant patients is a cornerstone of preventive care and health maintenance.Here we provide an overview of PSC,including its epidemiology,etiopathogenesis,clinical features,associated disorders,surveillance,and emerging potential therapies.展开更多
Primary sclerosing cholangitis(PSC)is an immune-related chronic cholangiopathy associated with high rates of progression to liver cirrhosis and the need for liver transplantation.Since PSC is frequently associated wit...Primary sclerosing cholangitis(PSC)is an immune-related chronic cholangiopathy associated with high rates of progression to liver cirrhosis and the need for liver transplantation.Since PSC is frequently associated with inflammatory bowel disease(IBD),several studies have investigated the role of the gut-liver axis in PSC and emerging evidence indicates that gut and bile microbiota are associated with the onset and progression of PSC[reviewed in(1)].展开更多
Primary sclerosing cholangitis(PSC)is a chronic,idiopathic,cholestatic liver disease characterized by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts.It can affect individuals of all age g...Primary sclerosing cholangitis(PSC)is a chronic,idiopathic,cholestatic liver disease characterized by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts.It can affect individuals of all age groups and gender,has no established pharmacotherapy,and is associated with a variety of neoplastic(e.g.cholangiocarcinoma)and non-neoplastic(e.g.dominant strictures)hepatobiliary complications.Given these considerations,endoscopy plays a major role in the care of patients with PSC.In this review,we discuss and provide updates regarding endoscopic considerations in the management of hepatobiliary manifestations and complications of PSC.Where evidence is limited,we suggest pragmatic approaches based on currently available data and expert opinion.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
基金This work was supported by US NIH grant R21DK100897(to Dr.C.L.Bowlus).
文摘Primary sclerosing cholangitis(PSC)is a rare,chronic,cholestatic liver disease of uncertain etiology characterized biochemically by cholestasis and histologically and cholangiographically by fibroobliterative inflammation of the bile ducts.In a clinically significant proportion of patients,PSC progresses to cirrhosis,end-stage liver disease,and/or hepatobiliary cancer,though the disease course can be highly variable.Despite clinical trials of numerous pharmacotherapies over several decades,safe and effective medical therapy remains to be established.Liver transplantation is an option for select patients with severe complications of PSC,and its outcomes are generally favorable.Periodic surveillance testing for pre-as well as post-transplant patients is a cornerstone of preventive care and health maintenance.Here we provide an overview of PSC,including its epidemiology,etiopathogenesis,clinical features,associated disorders,surveillance,and emerging potential therapies.
基金Funding:DFG Emmy Noether Program to MS(SI 19834/1)ERC Starting Grant REVERT to MS,BMBF PACE Therapy Grant to MSEinstein Foundation EC3R to MS.
文摘Primary sclerosing cholangitis(PSC)is an immune-related chronic cholangiopathy associated with high rates of progression to liver cirrhosis and the need for liver transplantation.Since PSC is frequently associated with inflammatory bowel disease(IBD),several studies have investigated the role of the gut-liver axis in PSC and emerging evidence indicates that gut and bile microbiota are associated with the onset and progression of PSC[reviewed in(1)].
基金This work was completed in part through T32 fellowship funding support for Dr.J.H.Tabibian from the United States National Institutes of Health(grant DK007198).
文摘Primary sclerosing cholangitis(PSC)is a chronic,idiopathic,cholestatic liver disease characterized by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts.It can affect individuals of all age groups and gender,has no established pharmacotherapy,and is associated with a variety of neoplastic(e.g.cholangiocarcinoma)and non-neoplastic(e.g.dominant strictures)hepatobiliary complications.Given these considerations,endoscopy plays a major role in the care of patients with PSC.In this review,we discuss and provide updates regarding endoscopic considerations in the management of hepatobiliary manifestations and complications of PSC.Where evidence is limited,we suggest pragmatic approaches based on currently available data and expert opinion.
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.