Treatment options for primary biliary cirrhosis and primary sclerosing cholangitis |
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Authors: | Cynthia Levy Keith D Lindor |
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Institution: | (1) Mayo Clinic Rochester, 200 1st Street SW-E 19B, 55905 Rochester, MN, USA |
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Abstract: | Opinion statement 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. |
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