Updated: 1/28/2019

Primary Biliary Cirrhosis

Topic
Review Topic
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Questions
3
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Evidence
6
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Introduction
  • An autoimmune disease in middle aged women resulting in an autoimmune destruction of bile ducts in the portal triads  
    • hampers the excretion of bile salts
  • Incidence is 8 out of 100,000 people
Presentation
  • Symptoms
    • chronic cholecystitis
    • pruritis (itching)
      • one of the first manifestations
      • caused by excess bile salts deposited in the skin
    • jaundice
    • hyperlipidemia
Evaluation
  • Serology
    • serum alkaline phosphatase levels greater than 1.5x the upper limit of normal
    • typically normal serum total bilirubin levels
  • Non-invasive imaging
    • right upper quadrant ultrasound can be used to first distinguish between intra and extrahepatic biliary obstruction 
  • Anti-mitochondrial antibody
    • 90% sensitive
    • negative in primary sclerosing cholangitis
  • Confirm with biopsy
    • focal duct obliteration with granuloma formation
Differential
  • Polycythemia vera, atopic dermatitis, uremia secondary to hemodialysis,
Treatment
  • Liver transplant
  • Supportive
    • cholestyramine therapy is used to bind salts in the intestine
      • enhancing their excretion
      • decreases puritis
    • Ursodeoxycholic acid   
      • first line treatment
      • decreases symptoms
      • increases transplant-free time
 

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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

(M2.GI.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? Review Topic

QID: 104823
FIGURES:
1

Ledipasvir-sofosbuvir

0%

(0/16)

2

Liver transplant

12%

(2/16)

3

Ribavirin

0%

(0/16)

4

Ursodeoxycholic acid

81%

(13/16)

5

Kasai procedure

0%

(0/16)

M2

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

(M2.GI.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? Review Topic

QID: 104830
FIGURES:
1

Interlobular bile duct destruction

43%

(6/14)

2

Intra and extrahepatic bile duct fibrosis

36%

(5/14)

3

Extrahepatic bile duct obstruction

14%

(2/14)

4

Absent UDP-glucuronyl transferase

0%

(0/14)

5

Decreased bilirubin uptake

0%

(0/14)

M2

Select Answer to see Preferred Response

PREFERRED RESPONSE 1

(M2.GI.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? Review Topic

QID: 106968
FIGURES:
1

Endoscopic balloon dilation

0%

(0/17)

2

Liver transplant

12%

(2/17)

3

Cholecystectomy

0%

(0/17)

4

Ursodeoxycholic acid

82%

(14/17)

5

Intravenous cefazolin

0%

(0/17)

M2

Select Answer to see Preferred Response

PREFERRED RESPONSE 4
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