Characteristic Findings of Primary Sclerosing Cholangitis on Endoscopic Retrograde Cholangiography: Which is the Most Common Finding?

Abstract

BackgroundPrimary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease and one of the most common indications for liver transplantation in adults. There are conflicting data regarding characteristic findings of PSC disease on endoscopic retrograde cholangiography (ERCP). We undertook this study to clarify whether there is a specific pattern of involvement of the biliary tract in patients with PSC and to evaluate features of PSC disease on ERCP in order to be able to manage this disease better.MethodsThis retrospective study included 45 patients admitted to Taleghani Hospital in 2004–2010 and diagnosed to have PSC on the basis of typical cholangiographic findings in combination with clinical and laboratory data. Patients suspected to have secondary sclerosing cholangitis were excluded. Demographic and clinical data were recorded, along with cholangiographic findings and the frequency of large duct and small duct PSC.ResultsForty-five patients of mean age 34.8 (range 15–66) years were included. Twenty-nine patients (64.4%) had inflammatory bowel disease, and the frequency of large duct PSC and small duct PSC was 93.4% and 6.6%, respectively. The intrahepatic ducts alone were involved in 11 (24.4%) patients and the extrahepatic ducts were involved in 14 (31.1%), with 17 (37.7%) patients having both intrahepatic and extrahepatic PSC. Three (6.6%) patients did not have bile duct involvement on ERCP, and their disease was diagnosed by liver biopsy as small duct PSC. The most common type of cholangiographic feature of intrahepatic duct involvement was type 2, found in 15 (33.3%) patients, with type 3 being the most common type of extrahepatic duct involvement and detected in 16 (35.5%) patients.ConclusionOur study demonstrates that the most common PSC finding on ERCP is involvement of both the extrahepatic and intrahepatic bile ducts, with small duct PSC being less common than large duct PSC.

The role of natural killer cells in liver inflammation

“…Dysregulation of the liver’s immune milieu due to both genetic and environmental factors can lead to autoimmune targeting of liver-specific compartments. Three main autoimmune diseases have been described in the liver: primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) [ 38 ].…”

“…PSC occurs equally in both males and females but is more often subclinical in females [ 43 , 44 ]. PSC is characterised by destruction of both intra- and extra-hepatic bile ducts leading to fibrosis and diffuse inflammation [ 38 ]. The frequencies of HLA-Bw4 and HLA-C2, ligands for the inhibitory NK cell receptors KIR3DL1 and KIR2DL1, respectively, were reduced in PSC patients compared to healthy controls, indicating a potential role for NK cells in the disease [ 45 ].…”

Primary Sclerosing Cholangitis Associated With Ulcerative Colitis Coexisting With Cholangiocarcinoma: A Case Report

“…The bile duct strictures are usually short, annular, or band-like, and long confluent strictures may be found in advanced cases. Both extrahepatic and IHBD involvement is reported in 75% of patients [ 16 ].…”