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

Clinical Features

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

Differential Diagnosis

• Autoimmune hepatitis
• Primary biliary cirrhosis
• IgG4 related disease

Evaluation

• LFTs
  • 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
• Consider serum IgG4 levels

Management

• 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)

Disposition

• Annual Screening for colon cancer in patients with concomitant ulcerative colitis

References

• Current Clinical Medicine, 2nd edition by Cleveland Clinic