Viral Hepatitis and Liver Disease Website Course

Evaluating Liver Test Abnormalities

Understanding the Pathophysiology of Liver Disease

for Health Care Providers

Primary Biliary Cirrhosis (PBC)

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Introduction

Primary biliary cirrhosis (PBC) is an autoimmune disorder that occurs mainly in middle-aged females. Bile duct injury is secondary to granulomatous destruction of intrahepatic bile ducts.

History

Patients may be entirely asymptomatic. The most common presenting symptoms are fatigue and pruritus. In patients with advanced disease there may be symptoms of deficiency of fat-soluble vitamins (e.g. easy bruising, night blindness, metabolic bone disease). As PBC is frequently associated with Sjogren's syndrome and Raynaud's phenomenon, the presence of dry eyes or skin changes with cold weather should be investigated.

Physical examination

The followings may be present:

  • Xanthomata, xanthelasma
  • Hyperpigmentation
  • Excoriation marks (from scratching)
  • Stigmata of chronic liver disease (particularly if cirrhosis is present)
  • Jaundice

Investigation

  • Anti-mitochondrial antibodies (AMA)
  • Albumin*
  • PT/INR*
  • Platelet level*

*In patients with any chronic liver injury, the presence of thrombocytopenia, coagulopathy or hypoalbuinemia is suggestive of cirrhosis.