Primary Biliary Cirrhosis

Christopher Bowlus, MD, UC Davis, Carlo Selmi, MD, UC Davis
(presentation package by Marion Peters, MD, UCSF)

Cholestasis


Liver Tests

Metabolism    Synthesis
  • drugs
  • toxins
  • urea
  • glucose
  • vitamins
    		•
  • clotting factors
  • albumin
  • globulin
  • lipoproteins
  • cholesterol


    • Cholestasis - Diagnosis


    Cholestasis

    S&S    Tests
  • Pruritis
  • Jaundice
  • Pigmentation
  • Steatorrhea
  • Osteopenia
    		•
  • bile acids
  • alkaline phosphatase
  • serum bilirubin
  • Lipids
  • Fat soluble vitamin deficiency


    • PBC - Clinical


    PBC Diagnosis

    Diagnosis can be made based upon elevated Alk Phos and positive AMA. Biopsy is not necessary for diagnosis, but is for the staging of the disease, especially indications are it may be advanced.

    PBC Clinical Findings

    Early    Late
  • no findings
  • hepatomegaly
  • excoriations
  • hyperpigmentation
  • xanthomata
  • CREST
  • spider angiomata
    		•
  • pathological fractures
  • Portal hypertension
  • GI bleed
  • Ascites
  • muscle wasting


    • PBC not always symptomatic

    In a study of 85 patients at time of diagnosis: Regarding AMA: it is not known which mitochondria are being attacked, or what the relationship of antimitochondrial antibodies is to PBC, only that it is a strong marker of the presence of PBC.

    PBC Clinical Staging

    Stage AMA  IgM  Alk Phos  bili
    1. Asymptomatic + nl ↑ ↑ nl ↑
    2. Symptomatic anicteric + ↑ ↑↑ nl
    3. Symptomatic icteric + ↑ ↑↑ ↑
    4. Cirrhotic + ↑↑ ↑↑ ↑↑


    PBC Risk Factors

    Observation Risk
    Smoking ↑
    No pregnancies ↓
    HRT ↑
    Bone loss ↑
    The following observations are considered the effect of flaws in the methodology of the study.
    Use of nail polish and/or hair dye↑
    Higher income ↑

    Source: Dr. Gershwin's epidemiological study comparing several thousand women with PBC to age-matched women without PBC

    PBC Associated Syndromes

    32% of PBC patients have another autoimmune disease, most commonly seen are Sjogren's and hypothyroiditis, followed distantly by scleroderma.

    PBC Histological Staging

    1. Florid Duct Lesion
    2. Ductular Proliferation
    3. Fibrosis
    4. Cirrhosis
    It is not known which mitochondria are being attacked, or what the relationship of antimitochondrial antibodies is to PBC, only that it is a marker of the presence of PBC.

    Chronic Cholestasis

    It is not known which mitochondria are being attacked, or what the relationship of antimitochondrial antibodies is to PBC, only that it is a marker of the presence of PBC.

    Chronic Cholestasis

    Yearly    Osteodystrophy
  • 25-OH Vitamin D
  • Calcium √ 24 hour
  • urine CA
  • DEXA



  • Vitamin A
    		•
  • Calcium
  • 25 OH Vit D
  • HRT
  • Calcitonin
  • Bisphosphonates


  • Water Soluble Vitamin A


    • Pruritis - Treatment


    Pruritis - Treatment

    Symptomatic Skin

    Pruritis - Treatment


    Pruritis - Treatment - Drugs


    Ursodeoxycholic Acid



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