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Updated: Dec 14 2021

Primary Biliary Cholangitis

Images
https://upload.medbullets.com/topic/120168/images/primary_biliary_cirrhosis_intermed_mag.jpg
  • Snapshot
    • A 45-year-old woman presents to her primary care physician for months of fatigue and generalized pruritus. She had been already examined in the dermatology clinic, when scabies and tinea were ruled out. On physical exam, she is noted to be jaundiced with scleral icterus and mild hepatomegaly. Laboratory results reveal a mild transaminitis and positive anti-mitochondrial antibodies but negative anti-nuclear antibody and anti-smooth-muscle antibodies. She is started on ursodeoxycholic acid and scheduled for an ultrasound to evaluate her hepatobiliary system.
  • Introduction
    • Overview
      • primary biliary cholangitis (PBC) is a granulomatous autoimmune condition resulting in destruction of the interlobular bile ducts
        • also known as primary biliary cirrhosis
    • Associated conditions
      • other autoimmune diseases
        • CREST syndrome
        • rheumatoid arthritis
        • celiac disease
        • Hashimoto thyroiditis
        • scleroderma
  • Epidemiology
    • Demographics
      • female > male
      • middle-aged (>40 years of age)
    • Risk factors
      • family history
  • ETIOLOGY
    • Pathogenesis
      • autoimmune attack on anti-mitochondrial antigens on biliary epithelial cells
      • autoantibodies
        • anti-mitochondrial antibodies (AMAs)
  • Presentation
    • Symptoms
      • common symptoms
        • fatigue
        • pruritus
          • worse at night
          • secondary to bile salts deposited in the skin
        • dark urine
        • light-colored stool
        • right upper quadrant pain
    • Physical exam
      • inspection
        • jaundice
        • hepatosplenomegaly
        • may have xanthomas or xanthelasma
  • Imaging
    • Ultrasound
      • indication
        • to assess for hepatobiliary disease
      • views
        • right upper quadrant
      • findings
        • cirrhosis, including increased nodularity, atrophy, or hypertrophy
        • distinguish between intra and extrahepatic biliary obstruction
    • ERCP (endoscopic retrograde cholangiopancreatography)
      • indication
        • ultrasound is inconclusive
      • findings
        • multifocal strictures and dilation of bile ducts
        • “string of beads”
  • Studies
    • Serum labs
      • liver function tests showing cholestasis pattern
        • ↑ direct bilirubin
        • ↑ alkaline phosphatase
      • autoantibodies
        • + anti-mitochondrial antibody (AMA)
          • 90-95% sensitivity
          • > 99% specificity
        • + anti-smooth muscle antibody (ASMA)
          • in 50% of patients
        • + anti-nuclear antibody (ANA)
          • in 50% of patients
      • ↑ IgM
      • ↑ cholesterol
    • Invasive studies
      • liver biopsy
        • indication
          • in AMA-negative patients in whom clinical suspicion is strong for PBC
        • findings
          • nonsuppurative cholangitis and destruction of interlobular biliary ducts
          • degenerating bile duct with lymphoid structures and granulomas
    • Diagnosis with 2 or more of
      • alkaline phosphatase > 1.5x upper limit of normal for 6 months or more
      • + AMA in serum
      • liver biopsy showing signs of PBC
  • Differential
    • Hemochromatosis
      • key distinguishing factor
        • triad of cirrhosis, diabetes, and skin bronze pigmentation
    • Wilson disease
      • key distinguishing factors
        • patients typically present before age 40
        • cirrhosis, neurologic disease, psychiatric disease, and Kayser-Fleischer rings in the eyes
  • Treatment
    • Lifestyle
      • avoidance of alcohol
      • vaccination against hepatitis A and B
    • Medical
      • ursodeoxycholic acid
        • indications
          • all patients
      • cholestyramine
        • indications
          • pruritus
    • Surgical
      • liver transplantation
        • indications
          • decompensated liver disease
          • severe pruritus refractory to other therapies
  • Complications
    • Portal hypertension
    • Cirrhosis
    • Osteoporosis
      • monitor with DEXA scan
      • supplement with vitamin D3
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