Updated: 12/11/2019

Primary Biliary Cholangitis

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Questions
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Evidence
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Topic
Snapshot
  • A 40-year-old woman presents to the urgent care for excessive fatigue and jaundice. She reports that she had felt fatigued for months. Recently, she noticed that her skin is more orange. On physical exam, she is noted to have scleral icterus and jaundice. Laboratory results reveal transaminitis. Anti-smooth muscle antibodies were negative, but a liver biopsy showed granulomatous inflammation of the bile ducts.
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
  • Epidemiology
    • demographics
      • female > male
      • middle-aged (>40 years of age)
    • risk factors
      • family history
  • Pathogenesis
    • autoimmune attack on anti-mitochondrial antigens on biliary epithelial cells
    • autoantibodies
      • anti-mitochondrial antibodies (AMAs)
  • Associated conditions
    • other autoimmune diseases
      • CREST syndrome
      • rheumatoid arthritis
      • celiac disease
      • Hashimoto thyroiditis
      • scleroderma
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, or concern for gallstones
    • findings
      • extrahepatic ducts appear grossly normal
      • contrast to “string of beads” in primary sclerosing cholangitis
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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Questions (4)
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(M1.GI.17.4720) A 47-year-old Caucasian woman presents with a 2-month history of general fatigue, slight jaundice, and mild itching. She has also noticed that her urine has been darker and stools have been lighter in color recently. She denies any fevers, chills, or alcohol use. She has no significant past medical or surgical history and is not taking any medications. She recalls that her mother saw a doctor for eye and mouth dryness but cannot remember the name of her diagnosis. She denies any illicit drug use, recent change in diet, or recent travel. On physical exam, her abdomen is soft and non-distended. There is right upper quadrant tenderness to deep palpation but a negative Murphy’s sign. Her laboratory findings were significant for increased liver enzymes, direct bilirubin, and alkaline phosphatase with normal levels of iron and ceruloplasmin. Ultrasound revealed no stones in the gallbladder or common bile duct and endoscopic retrograde cholangiopancreatography (ERCP) revealed normal extrahepatic biliary ducts. Which of the following findings is most likely to also be found in this patient?

QID: 108499
1

Anti-neutrophilic cytoplasmic antibodies (ANCA)

18%

(48/268)

2

Rheumatoid factor

6%

(15/268)

3

Anti-gliadin antibody

4%

(12/268)

4

Anti-centromere antibody

13%

(34/268)

5

Anti-mitochondrial antibody

56%

(151/268)

M 2 C

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Evidence (5)
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