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PRIMARY BILIARY CIRRHOSIS

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.


 

Introduction

  • Primary biliary cirrhosis (PBC) is an autoimmune disease of a chronic & progressive nature, characterized by destruction of small to medium bile ducts, leading to cholestasis & frequently, end-stage liver disease
    • Diagnostic features of primary biliary cirrhosis (PBC) are the chronic biochemical cholestasis, presence of antimitochondrial antibodies (AMA) & the characteristic liver biopsy findings
  • Suggested to have environmental &/or genetic factors affecting its development
  •  Primary biliary cirrhosis (PBC) is more common in Northern Europeans & the majority of the patients are middle-aged women
    •  Men w/ the disease are more likely to develop hepatocellular carcinoma (HCC)

Signs and Symptoms

Patients may Present w/ Symptoms Attributable to Liver Disease

Fatigue

  • Seen in 20-85% of patients
  • Associated w/ depression, obsessive-compulsive disorder & sleep disturbances
  • Symptom does not correlate w/ severity of liver disease

Pruritus

  • Prevalence rate is 20-75% in patients w/ primary biliary cirrhosis (PBC) 
  • A circadian rhythm may be noted in pruritus due to cholestasis
  • May be so severe as to cause severe emotional disturbance
  • May be present even in patients w/ good liver function
  • Declines in severity w/ time from diagnosis

Jaundice

  • Present in 10-60% of patients due to cholestasis

Right Upper Quadrant Pain

  • Occurs in 8-17% of patients
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