AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma

Abstract

WHAT’S NEW SINCE THE 2010 GUIDELINES? Inclusion of guidance for the diagnosis and management of cholangiocarcinoma (CCA) in patients with and without primary sclerosing cholangitis (PSC) (Figures 5, 8, and 9). Introduction of the term relevant stricture, defined as any biliary stricture of the common hepatic duct or hepatic ducts associated with signs or symptoms of obstructive cholestasis and/or bacterial cholangitis (Table 1). In patients with equivocal MRI with cholangiopancreatography (MRI/MRCP) findings, a repeated high‐quality MRI/MRCP should be performed for diagnostic purposes. Endoscopic retrograde cholangiopancreatography (ERCP) should be avoided for the diagnosis of PSC (Figure 2). In patients with PSC without known inflammatory bowel disease (IBD), diagnostic colonoscopy with histological sampling should be performed and may be repeated every 5 years if IBD is not initially detected. Colon cancer surveillance should begin at age 15 years in patients with PSC and IBD. New clinical risk tools for PSC are available for risk stratification, but probabilities of events in individual patients should be interpreted with caution (Figure 4 and Table 3). All patients with PSC should be considered for participation in clinical trials; however, ursodeoxycholic acid (13–23 mg/kg/day) can be considered and continued if well tolerated with a meaningful improvement in alkaline phosphatase (γ‐glutamyl transferase in children) and/or symptoms with 12 months of treatment. ERCP with biliary brushings for cytology and fluorescent in situ hybridization analysis should be obtained in all patients with suspected perihilar or distal CCA. There is a new United Network for Organ Sharing policy regarding standardization of Model for End‐Stage Liver Disease exceptions for patients with PSC and recurrent cholangitis. Liver transplantation following neoadjuvant therapy is recommended for patients with perihilar CCA < 3 cm in radial diameter that is unresectable or arising in the setting of PSC and in the absence of intrahepatic or extrahepatic metastasis (Figure 9). TABLE 1 - Definitions in PSC PSC Chronic, cholestatic liver disease likely of autoimmune origin characterized by inflammation and fibrosis of intrahepatic and/or extrahepatic bile ducts, leading to the formation of bile duct strictures, and frequently associated with IBD Small‐duct PSC Less common variant of PSC that is characterized by typical cholestatic and histological features of PSC but with normal bile ducts on cholangiography PSC–AIH overlap Concurrent diagnostic features of PSC and clinical, biochemical, and histological features of AIH Secondary sclerosing cholangitis Biliary strictures due to identifiable causes that can result in secondary biliary cirrhosis IgG4 sclerosing cholangitis Biliary strictures due to elevated IgG4‐positive plasma cells in tissue and serum IgG4 elevation frequently associated with pancreatic involvement Dominant stricture A biliary stricture on ERCP with a diameter of ≤1.5 mm in the common bile duct or of ≤1 mm in the hepatic duct High‐grade stricture A biliary stricture on MRI with cholangiopancreatography with >75% reduction in the common bile duct or hepatic ducts Relevant stricture Any biliary stricture of the common bile duct or hepatic ducts associated with signs or symptoms of obstructive cholestasis and/or bacterial cholangitis INTRODUCTION AND SCOPE OF GUIDANCE Primary sclerosing cholangitis (PSC) is a cholangiopathy characterized by chronic fibroinflammatory damage of the biliary tree and is frequently associated with inflammatory bowel disease (IBD). The majority of patients with PSC have fibrotic biliary strictures on cholangiogram, whereas a minority have small‐duct PSC, characterized by a normal cholangiogram but with histological features of PSC on liver biopsy. A small percentage have overlapping features of autoimmune hepatitis (PSC‐AIH). PSC affects both male and female individuals and can occur at any age. PSC is considered an autoimmune disease, though the pathophysiology remains poorly understood. PSC frequently results in cholestatic liver damage, cirrhosis, and liver failure and can recur in 20%–30% of patients PSC the risk of cholangiocarcinoma (CCA) and cancer is therapy for PSC, and clinical with a of diagnostic for A of new biliary strictures, is in Table for the of Liver guidance a to the diagnosis and management of PSC and CCA. are by of the of the of the and of the if of results the of and In guidance by of an and guidance on and analysis of the on the with by the at all of guidance The to a guidance on a of not available to the of a meaningful In to the of to the 2010 new for the of biliary strictures, an on for diagnosis retrograde cholangiopancreatography (ERCP) and liver of and for clinical and management of OF PSC of PSC have The majority of to have in and of and are and have that the and of PSC may be of the a PSC to the United and the United to be by PSC at to of PSC is the of and with a ag