Primary sclerosing cholangitis

Primary Sclerosing Cholangitis (PSC)

Background

Autoimmune Dz typically seen in young men

Progressive inflammatory destruction of medium and large bile ducts

Most (80%) cases are associated with inflammatory bowel dz, typically ulcerative colitis 10% of pts with ulcerative colitis have PSC

Increased risk of colon CA in pts with Ulcerative colitis and PSC (more than UC alone) Increased risk of cholangiocarcinoma Prevalence is 1 to 6 per 100,000 in the U.S

Clinical Features

Generally asymptomatic but can present with abd pain, jaundice, cholangitis, or puritis

Mean age at presentation: 30-40 

Diagnosis

Alkaline phosphatase is usually elevated with mild elevations in aminotransferases

Bilirubin is typically normal, except when common hepatic duct or common bile duct is involved and in late stages of dz

Dx made by ERCP or MRCP, which demonstrates strictures or beading of the intrahepatic or extrahepatic bile ducts

Liver biopsy typically shows pericholangitis and periductual fibrosis but is often not diagnostic in early dz

Perinuclear antineutrophil cytoplasmic antibody (pANCA) positive in 2/3rds of cases

Treatment

 High dose Ursodeoxycholic acid (UDCA), 25-28mg/kg/day, may improve liver chemistries but does not slow dz progression and may actually hasten development of portal HTN

 Periodic dilation of strictures via ERCP or percutaneous route

Liver Transplant should be offered to those with advanced liver dz or repeated bouts of cholangitis (Dz can recur after transplantation)

Disposition

 Annual Screening for colon cancer pts with concomitant UC