Primary sclerosing cholangitis

Background

Autoimmune disease typically seen in young men
Progressive inflammation and fibrosis of intra/extra hepatic bile ducts
Most (80%) cases are associated with inflammatory bowel disease, typically ulcerative colitis, 10% of patients with ulcerative colitis have PSC
Increased risk of colon cancer in patients 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

Generally asymptomatic but can present with fatigue, abdominal pain, jaundice, cholangitis, puritis, weight loss, or fever
Mean age at presentation: 30-40

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

High dose Ursodeoxycholic acid (UDCA), 25-30mg/kg/day
- May improve liver chemistries but does not slow disease progression and may actually hasten development of portal hypertension
Periodic dilation of strictures via ERCP or percutaneous route
Liver Transplant should be offered to those with advanced liver disease or repeated bouts of cholangitis (disease can recur after transplantation)

Annual Screening for colon cancer in patients with concomitant ulcerative colitis