Abstract
Primary biliary cirrhosis (PBC) is a slowly progressive, chronic liver disease that causes inflammatory destruction of the bile ducts, bile acid buildup, cirrhosis, and, ultimately, liver failure. Reduction of bile acid available for digestion results in malabsorption of vitamins A, D, E, and K, often causing vitamin deficiencies, hypercholesterolemia, hyperlipidemia, steatorrhea, and osteoporosis.
The etiology of PBC is unknown, but it's suspected to be an autoimmune disorder. Individuals with this condition often have comorbid conditions such as autoimmune thyroiditis, scleroderma, rheumatoid arthritis, or Sjögren's syndrome. About 95% of patients with PBC are women between age 30 and 60. There may be a familial predisposition for PBC, although it's not considered a hereditary disorder. The exact prevalence is unknown, but incidence estimates range from 19 to 151 cases per million. Worldwide, PBC accounts for up to 2% of deaths from cirrhosis.