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1: Curr Treat Options Gastroenterol. 2003 Apr;6(2):93-103.Click here to read Links

Treatment Options for Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis.

Levy C, Lindor KD.

Mayo Clinic Rochester, 200 1st Street SW-E 19B, Rochester, MN 55905, USA. Levy.cynthia@mayo.edu

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are chronic cholestatic liver diseases that affect 0.5 to 40 per 100,000 and 1 to 6 per 100,000 Americans, respectively. Prompt recognition and management of the clinical manifestations of these diseases is essential for the patients' well-being and ultimate outcome. Ursodeoxycholic acid (UDCA), 13 to 15 mg/kg per day, is the standard therapy for PBC and should be offered to every patient. It has been shown to slow progression of the disease and prevent the need for liver transplantation, which is the last recourse for patients with end-stage disease. However, there is no effective therapy for PSC yet. Patients are managed symptomatically, with surgical or endoscopic interventions as needed in cases of significant biliary obstruction. Complications of chronic cholestasis are seen in both PBC and PSC, with pruritus and fatigue being the most common complaints. The first choice for the treatment of pruritus is still cholestyramine, starting at 4 g/d. The pathogenesis of fatigue is poorly understood in this population; unrecognized hypothyroidism should be excluded. The use of antidepressants is currently under evaluation, but there is no specific therapy for fatigue as of yet. For prevention of severe osteoporosis, we recommend supplementation with 800 IU vitamin D and 1500 mg calcium/d. In patients with PBC and established osteoporosis, the use of alendronate and vitamin K appears to cause an increase in bone mineral density. Further studies are necessary before either of these drugs is routinely recommended. Finally, fat-soluble vitamin deficiencies are noted with more advanced disease. We recommend that serum levels be checked in high-risk patients, and that vitamins are replaced as appropriate with water-soluble supplements. However, other causes of malabsorption must be ruled out, including pancreatic insufficiency and celiac sprue.

PMID: 12628068 [PubMed - as supplied by publisher]

Patient Drug Information

  • Cholestyramine Resin (Locholest® , Locholest® Light, Questran® , ...)

    Cholestyramine is used with diet changes (restriction of cholesterol and fat intake) to reduce the amount of cholesterol and certain fatty substances in your blood. Accumulation of cholesterol and fats along the walls of...

  • Ursodiol (Actigall® )

    Ursodiol is used to dissolve gallstones in patients who do not want surgery or cannot have surgery to remove gallstones. Ursodiol is also used to prevent the formation of gallstones in overweight patients who are losing ...

  • Alendronate (Fosamax® , Fosamax Plus D® )

    Alendronate is used to treat and prevent osteoporosis (a condition in which the bones become thin and weak and break easily) in women who have undergone menopause ('change of life,' end of menstrual periods) and to treat...

  • Source: AHFS Consumer Medication Information