Updated: 12/21/2019

Primary Biliary Cholangitis

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Questions
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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
  • 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
    • 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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Questions (4)
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(M2.GI.15.32) A 36-year-old female with a history of celiac disease presents to her primary care physician because of recent changes to her health. She states that over the past several months she has noticed her eyes and skin seem to have become more yellow in color and she has also noticed increased itchiness all over her body. On exam, the patients eyes are shown to be as demonstrated in Figure A and an additional finding is observed around her eyes as demonstrated in Figure B. The physician obtains liver function tests which demonstrate an increased direct bilirubin and an increased alkaline phosphatase. Further testing demonstrates increased serum mitochondrial antibodies and a liver biopsy is obtained as demonstrated in Figure C. What is the first-line treatment for this patient's condition?

QID: 104823
FIGURES:
1

Ledipasvir-sofosbuvir

0%

(0/17)

2

Liver transplant

12%

(2/17)

3

Ribavirin

0%

(0/17)

4

Ursodeoxycholic acid

82%

(14/17)

5

Kasai procedure

0%

(0/17)

M 7 D

Select Answer to see Preferred Response

(M2.GI.15.39) A 65-year-old female with a history of rheumatoid arthritis presents to her primary care physician because she is concerned about her skin. She states that over the past few months she has become extremely itchy, in spite of applying numerous creams and lotions. She states that she has not noticed any visible changes or rashes on her skin and feels well apart from mild fatigue. On exam, the physician appreciates hepatosplenomegaly and observes the findings shown in Figure A. Laboratory testing reveals elevated antimitochondrial antibodies and a liver biopsy is obtained which is demonstrated in Figure B. What is the most likely underlying pathophysiology of this patient's disease?

QID: 104830
FIGURES:
1

Interlobular bile duct destruction

38%

(6/16)

2

Intra and extrahepatic bile duct fibrosis

31%

(5/16)

3

Extrahepatic bile duct obstruction

25%

(4/16)

4

Absent UDP-glucuronyl transferase

0%

(0/16)

5

Decreased bilirubin uptake

0%

(0/16)

M 5 D

Select Answer to see Preferred Response

(M2.GI.15.4823) A 41-year-old woman presents to her primary care physician reporting fatigue for the past 3 months. On further questioning, she notes dry eyes and dry mouth, as well as whole-body itching for the past 3 months. She denies any change in skin color. For the past year, she has had a persistently elevated alkaline phosphatase around 300 IU/L (normal 40 to 120 IU/L) as well as elevated total bilirubin at 2.4 (normal 0.3 to 1.9 mg/dL) and direct bilirubin at 0.7 (normal 0 to 0.3 mg/dL). Her aminotransferase levels have been within normal limits and RUQ ultrasound was unremarkable. Further testing revealed a positive anti-mitochondrial antibody titer, and a liver biopsy was performed (Figure A). Which of the following is the most appropriate treatment for this patient at this time?

QID: 106968
FIGURES:
1

Endoscopic balloon dilation

0%

(0/22)

2

Liver transplant

9%

(2/22)

3

Cholecystectomy

0%

(0/22)

4

Ursodeoxycholic acid

77%

(17/22)

5

Intravenous cefazolin

9%

(2/22)

M 7 C

Select Answer to see Preferred Response

Evidence (6)
EXPERT COMMENTS (7)
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