Primary sclerosing cholangitis
Primary Sclerosing Cholangitis (PSC)
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
DDx
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
See Also
Source
Current Clinical Medicine, 2nd edition by Cleveland Clinic