primary biliary cirrhosis, a chronic inflammatory condition of the liver. It is characterized by generalized pruritus; enlargement and hardening of the liver; weight loss; and diarrhea with pale, bulky stools. Petechiae, epistaxis, or hemorrhage resulting from hypoprothrombinemia may also be evident. Pathological fractures and collapsed vertebrae may develop as the result of the associated malabsorption of vitamin D and calcium. Xanthomas commonly develop when the serum cholesterol level exceeds 450 mg/dL. The cause of primary biliary cirrhosis is unknown, although it is associated with autoimmune disorders. The condition most often affects women 40 to 60 years of age. The diagnosis is confirmed by liver biopsy and cholangiography. Antibody is nearly always present. Jaundice, dark urine, pale stools, and cutaneous xanthosis may occur in the later stages of this disease. Treatment commonly includes the administration of fat-soluble vitamins A, D, E, and K to prevent and correct deficiencies caused by malabsorption. Life expectancy is about 5 years for symptomatic patients after the onset of jaundice. Compare secondary biliary cirrhosis.
