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