Biliary Tract: Primary Sclerosing Cholangitis (PSC)

Basics

• Chronic Autoimmune Fibrosis of the Biliary Tract
• Affects Intrahepatic Bile Ducts (15%), Extrahepatic Bile Ducts (10%) or Both (75%)
• May See Multiple Strictures Throughout the Entire Biliary Tree
• Most Common in Men
• Strongly Associated with IBD (Especially Ulcerative Colitis)
  • Prevalence: 60-90%
  • No Improvement After Colon Resection
• High Risk of Cholangiocarcinoma (10-20%)

Presentation

• Causes Biliary Stricture with Chronic Cholestasis & Eventual Cirrhosis
• Commonly ASx at Diagnosis
• Sx: Fatigue, Pruritis, Abdominal Pain & Jaundice
• May Show Signs of Decompensated Liver Failure if Presenting Late

Diagnosis

• Dx: MRCP/ERCP (“Beaded” Bile Ducts)
• Labs:
  • Elevated LFT’s; Particularly Alkaline Phosphatase
  • Atypical P-ANCA (Perinuclear Antineutrophil Cytoplasmic Antibody)
  • Always Check CA 19-9 for CA
  • Always Check IgG4
• Immediate Colonoscopy on Dx to Evaluate for IBD

Treatment

• Temporary/Sx Relief:
  • Ursodeoxycholic Acid (UDCA)
  • If Dominant Extrahepatic Strictures: Consider ERCP & Stent
  • Other Potential Medical Options: Cyclosporine, Methotrexate, Azathioprine or ABX
  • Avoid:
    • Generally Avoid Choledochojejunostomy as TXP is Preferred
    • No Benefit: Cholestyramine or Colchicine
• Definitive Tx: Liver TXP

Cancer Screening After Diagnosis

• Abdominal US or MRI/MRCP Every 6-12 Months
• CA 19-9 Every 1 Year
• Colonoscopy at Diagnosis & Every 1-2 Years