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PRIMARY BILIARY CHOLANGITIS

Primary biliary cholangitis (formerly primary biliary cirrhosis) is a 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.


 

Introduction

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

Signs and Symptoms

Fatigue

  • The most common symptom seen in 50-78% of patients
  • Associated with depression, obsessive-compulsive disorder and sleep disturbances
  • Symptom does not correlate with severity of liver disease

Pruritus

  • Prevalence rate is 20-70% in patients with PBC
  • A circadian rhythm may be noted with worse symptoms at night
  • May be so severe as to cause severe emotional disturbance
  • May be present even in patients with good liver function
  • Declines in severity with 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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