Updated: 7/22/2019

Secondary Billiary Cholangitis

Review Topic
  • A 46-year-old obese woman presents to her gastrointestinal doctor for colicky right upper quadrant pain and pruritus. She denies having any weight loss, fevers, or chills. She does report having pruritus worse at night, as well as fatigue and light-colored stool. Labs show elevated alkaline phosphatase. A right upper quadrant ultrasound shows intrahepatic dilation of bile ducts and stones in the gallbladder. She is scheduled for a consultation with general surgery and started on ursodeoxycholic acid and cholestyramine.
  • Overview
    • secondary biliary cholangitis resulting from longstanding extrahepatic biliary obstruction
  • Epidemiology
    • risk factors
      • obstructive lesions
        • gallstones
        • biliary strictures
        • malignancy
          • pancreatic cancer
        • inflammation
          • pancreatitis
  • Pathogenesis
    • extrahepatic obstruction of the bile ducts causes ↑ pressure in the intrahepatic ducts
      • this causes injury in the biliary tree, including fibrosis
  • Symptoms
    • common symptoms
      • fatigue
      • pruritus
        • worse at night
      • dark urine
      • light-colored stool
      • right upper quadrant pain
  • Physical exam
    • inspection
      • jaundice
      • hepatosplenomegaly
      • may have xanthomas or xanthelasma
  • Ultrasound
    • indication
      • to assess for hepatobiliary disease, including the etiology of extrahepatic obstruction
    • views
      • right upper quadrant
    • findings
      • gallstones
      • intrahepatic vs extrahepatic ductal dilation
  • Serum labs
    • liver function tests showing cholestasis pattern
      • ↑ direct bilirubin
      • ↑ alkaline phosphatase
    • ↑ cholesterol
  • Primary biliary cholangitis
    • key distinguishing factor
      • + autoantibodies (anti-mitochondrial antibody)
  • Hemochromatosis
    • key distinguishing factor
      • triad of cirrhosis, diabetes, and bronze skin pigmentation
  • Wilson disease
    • key distinguishing factors
      • patients typically present before age 40
      • cirrhosis, neurologic disease, psychiatric disease, and Kayser-Fleischer rings in the eyes
  • Management approach
    • treat underlying condition
      • i.e., cholecystectomy for cholelithiasis
  • Medical
    • ursodeoxycholic acid
      • indications
        • all patients
    • cholestyramine
      • indications
        • severe pruritus refractory to other therapies
  • Ascending cholangitis

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