Primary Biliary Cholangitis (Primary Biliary Cirrhosis) Medication

Updated: Jun 03, 2016
  • Author: Nikolaos T Pyrsopoulos, MD, PhD, MBA, FACP, AGAF; Chief Editor: BS Anand, MD  more...
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Medication

Medication Summary

The goals of treatment are to slow the progression rate of the disease and to alleviate the symptoms (eg, pruritus, osteoporosis, sicca syndrome). Liver transplantation appears to be the only life-saving procedure.

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Bile acids

Class Summary

UDCA is the major medication used to slow the progression of the disease. Patients with early disease have clinical, biochemical, and histologic improvement. Reports suggest that UDCA delays the need for transplantation or delays death. The efficacy of this medication in late stages (ie, cirrhosis) is questionable.

Ursodiol (Actigall)

Displaces endogenous bile acids from enterohepatic circulation, stabilizes hepatocellular membranes, and reduces the abnormal expression of HLA class I and II molecules on hepatocytes.

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Farnesoid X Receptor Agonist

Class Summary

The farnesoid X receptor (FXR) is a nuclear receptor expressed in the liver, intestine, kidney, and adipose tissue that regulates a wide variety of target genes critically involved in the control of bile acid synthesis and transport, lipid metabolism, and glucose homeostasis.

Obeticholic acid (Ocaliva)

Farnesoid X receptor (FXR) agonist. FXR activation suppresses de novo synthesis of bile acids in hepatocytes as well as increases transport of bile acids out of hepatocytes, thereby reducing exposure of the hepatocytes to bile acid. It is indicated for primary biliary cholangitis (PBC) in combination with ursodeoxycholic acid (UDCA) in adults with an inadequate response to UDCA for at least 1 y or as monotherapy in adults unable to tolerate UDCA.

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Immunosuppressants

Class Summary

Inhibit key factors that mediate immune reactions.

Methotrexate (Rheumatrex, Folex)

Results of various trials suggest improvement in biochemical and histologic findings after treatment.

Prednisolone (Delta-Cortef)

May improve symptoms, biochemical findings, and histologic findings. Great concern exists about corticosteroid-induced osteoporosis.

Cyclosporine (Sandimmune, Neoral)

Has some therapeutic potential.

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Anti-inflammatory agents

Class Summary

Decrease leukocyte motility and phagocytosis in inflammatory responses.

Colchicine

Has been administered to these patients, but results are controversial.

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Antipruritics

Class Summary

Pruritus is often refractory to medical therapy and significantly impacts patients' quality of life. Antihistamines are first-line agents to relieve pruritus in early stages. Cholestyramine and colestipol are effective in sequestering bile salts in the enteric lumen. A 1- to 4-day delay is expected before itching remits.

Cholestyramine (Questran)

Should be administered 1 h before meals.

Rifampin (Rifadin, Rimactane)

Precise mechanism of action is unclear. May involve inhibition of bile acid uptake into hepatocytes and facilitation of excretion of dihydroxy and monohydroxy bile acids and toxic bile acids. Used in patients who are not responding to cholestyramine.

Colestipol (Colestid)

Forms a soluble complex after binding to bile acid, increasing fecal loss of bile acid–bound low-density lipoprotein cholesterol.

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Antihistamines

Class Summary

These agents are the first line of medication for patients with mild-to-moderate pruritus. Use caution in patients with cirrhosis and signs of encephalopathy because antihistamines can further depress brain function.

Hydroxyzine hydrochloride (Vistaril, Atarax)

Antagonizes H1 receptors in periphery. May suppress histamine activity in subcortical region of CNS.

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