Primary biliary cirrhosis is chronic, progressive, autoimmune, cholestatic liver disease more common in middle-aged women. It is characterized by destruction of small to medium bile ducts, leading to cholestasis and frequently, end-stage liver disease.
Diagnostic features are chronic biochemical cholestasis, presence of antimitochondrial antibodies and the characteristic liver biopsy findings.
At present, the diagnosis is most often made in an asymptomatic patient who presents with abnormal lab results on a routine checkup or as part of workup for an associated illness.



Annual Re-evaluation for Certain Patients

  • Patients positive for antimitochondrial antibody (AMA), but have a normal alkaline phosphatase (ALP) level, should have their liver biochemistry rechecked yearly
  • A small study has shown that most antimitochondrial antibody (AMA)-positive asymptomatic individuals w/ normal alkaline phosphatase (ALP) eventually develop evidence of cholestasis &/or cholestatic symptoms after several years


Prevention of Complications

  • Assess primary biliary cirrhosis (PBC) activity & progression w/ tests for albumin, total bilirubin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), prothrombin time (PT) every 3-6 months
  • Assess esophageal/gastric varices complication w/ an upper gastrointestinal endoscopy annually & liver cirrhosis w/ an abdominal ultrasound & serum alpha-fetoprotein every 6 months


  • Pruritus has a significant effect on a patient’s life & is often refractory to medical treatment


  • Cholestyramine is the main drug used to treat cholestasis-associated pruritus
  • Action: Binds bile acids in the gut lumen promoting its fecal excretion
  • Most effective in patients w/ intact gallbladders if taken before & after breakfast, because this is when the largest amount of bile is available for binding by the drug
  • Drug takes effect w/in 1-4 days of starting therapy
  • Effect is optimal w/ daily treatment
  • There should be an interval of ≥4 hours between intake of Cholestyramine & other medications because Cholestyramine can also bind other oral drugs


  • May be used to control mild pruritus early in the course of the disease
    • Should be used w/ caution in patients w/ cirrhosis or signs of encephalopathy because antihistamines can depress brain function further
  • Not typically very effective & most of the relief results from sedation


  • May be used as a 2nd-line agent in patients unresponsive to Cholestyramine
  • Long-term treatment may result in improvement in liver biochemistry
  • Disadvantage is that Rifampicin is not effective in all patients & if it is effective, the effect on pruritus is only noticeable after about 1 month
  • Usual dose given: 150 mg PO 8-12 hourly


  • Other medications for the treatment of pruritus are Sertraline, Rifaximin, opioid antagonists eg Naltrexone, Naloxone; Dronabinol, Rifampicin, & Metronidazole

Portal Hypertension

  • Portal hypertension may develop earlier than cirrhosis from nodular regenerative hyperplasia
  • Nonselective beta-blockers may help relieve portal hypertension
  • Patients may also benefit from shunt surgery
  • Patients should be screened for varices upon diagnosis of primary biliary cirrhosis (PBC) & every 3 years thereafter
  • Prophylactic measures to prevent bleeding should be carried out in patients w/ varices

Metabolic Bone Disease

  • Osteoporosis is often subtle & can only be detected by measuring bone mineral density using dual energy x-ray absorptiometry
  • Patient’s bone mineral density should be assessed at the time of diagnosis of primary biliary cirrhosis (PBC) & every 1-2 years thereafter
  • All primary biliary cirrhosis (PBC) patients should be advised to engage in regular wt-bearing exercise & if required, to stop smoking & drinking alcohol
  • In patients w/ evidence of osteoporosis, bisphosphonates are of benefit
  • Vitamin D & calcium (Vit D & Ca) supplementation should be given
  • Estrogen hormone replacement therapy (HRT) may be needed in certain patients
    • Transdermal administration may be the preferred route

Sicca Syndrome

  • Symptoms of the sicca syndrome should be elicited by direct questioning
  • Patients w/ dry eyes should be given artificial tears to prevent complications eg corneal ulceration
  • Patients w/ dry mouth should be given saliva substitutes & undergo monitoring of oral health
    • Pilocarpine or Cevimeline can be given to patients w/ dry mouth or dry eyes who are unresponsive to other therapies
  • Liq should be given w/ food & medications to ease swallowing
  • Lubricating jelly & moisturizers may be used in female patients w/ dyspareunia

Malabsorption of Fat-Soluble Vitamins

  • Replacement of fat-soluble vitamins (eg Vitamin K) may be given using their water-soluble form
  • If bilirubin level >2 mg/dL, monitor vitamins A, D, K yearly


  • A complication of chronic cholestasis, hyperlipidemia seen in primary biliary cirrhosis (PBC) is apparently not associated w/ cardiovascular adverse effects
  • Statins & fibrates may be given to patients w/ primary biliary cirrhosis (PBC)
    • Fibrates may occasionally cause a paradoxical increase in serum cholesterol levels

Thyroid Dysfunction

  • Thyroid-stimulating hormone (TSH) should be determined at the time of diagnosis of primary biliary cirrhosis (PBC) & regularly thereafter ie yearly

Raynaud’s Syndrome

  • More of an issue for patients in cold climates
  • Patient should be advised to avoid exposure of extremities to cold & to stop smoking
  • Ca antagonists may relieve extremity symptoms but may worsen esophageal dysmotility
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