Primary sclerosing cholangitis

Background

• Autoimmune Dz typically seen in young men
• Progressive inflammation and fibrosis of intra/extra hepatic 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 fatigue, abd pain, jaundice, cholangitis, puritis, weight loss, or fever
• Mean age at presentation: 30-40 

Diagnosis

• Cholangiography
• 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

Work-Up

• 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 in late stages of dz
• Perinuclear antineutrophil cytoplasmic antibody (pANCA) positive in 2/3rds of cases

Treatment

• High dose Ursodeoxycholic acid (UDCA), 25-30mg/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 in pts with concomitant UC

Source

Current Clinical Medicine, 2nd edition by Cleveland Clinic