Updated: 9/3/2019

Primary Biliary Cholangitis

Topic
Review Topic
0
0
Questions
3
0
0
Evidence
3
0
0
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 muslce 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
    • 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
 

Please rate topic.

Average 4.8 of 6 Ratings

Thank you for rating! Please vote below and help us build the most advanced adaptive learning platform in medicine

The complexity of this topic is appropriate for?
How important is this topic for board examinations?
How important is this topic for clinical practice?
Questions (3)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
Calculator

Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

You have 100% on this question.
Just skip this one for now.

(M1.GI.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? Review Topic

QID: 108499
Type in at least one full word to see suggestions list
1

Anti-neutrophilic cytoplasmic antibodies (ANCA)

20%

(43/217)

2

Rheumatoid factor

6%

(13/217)

3

Anti-gliadin antibody

5%

(10/217)

4

Anti-centromere antibody

15%

(32/217)

5

Anti-mitochondrial antibody

52%

(113/217)

M1

Select Answer to see Preferred Response

SUBMIT RESPONSE 5
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
ARTICLES (4)
Topic COMMENTS (11)
Private Note