Cholangitis, Primary Sclerosing

• • Associated with ulcerative colitis 40-60%, pancreatitis 12-25%, diabetes mellitus 5-10%, and rarely other autoimmune disorders

  • Onset during fourth or fifth decade of life

  • Increased risk for cholangiocarcinoma

Symptoms and Signs

• • Intermittent jaundice

  • Fever

  • Right upper quadrant pain

  • Pruritus

Laboratory Findings

• • Elevated alkaline phosphatase

  • Hyperbilirubinemia

  • Leukocytosis

Imaging Findings

• • Right upper quadrant US, ERCP, and magnetic resonance cholangiopancreatography (MRCP) may show multiple dilatations and strictures of extrahepatic biliary ducts

• • Cholangiocarcinoma

  • Presence of cirrhosis

Rule Out

• • Cholangiocarcinoma

• • History and physical exam

  • Liver function tests

  • Abdominal US

  • ERCP

  • Liver biopsy if question of cirrhosis

  • Brushings by percutaneous transhepatic cholangiography (PTC) or ERCP if question of malignancy

When to Admit

• • Cholangitis

Surgery

• • Balloon dilatation of multiple strictures

  • Resection of dominant stricture followed by biliary reconstruction

  • Liver transplantation (preferably before onset of cirrhosis)

Contraindications

• • Cirrhosis

Medications

• • Ursodiol (improves liver function and histology but no difference in 5-year clinical outcome)

Treatment Monitoring

• • Alkaline phosphatase levels

Complications

• • Cholangitis

  • Recurrent strictures

  • Primary nonfunction

  • Allograft rejection

  • Recurrent stricture post-transplant

Prognosis

• • 85% survival rate 5 years post transplant

  • 71% actuarial survival at 5 years for resection of dominant stricture (only 20% if cirrhosis present)

  • 43% long-term success with balloon therapy for multiple strictures

References