Primary Sclerosing Cholangitis Medication

  • Author: Vikas Khurana, MD, FACP, FACG; Chief Editor: Julian Katz, MD   more...
 
Updated: Apr 5, 2012
 

Medication Summary

No pharmacologic therapy has been proven effective for primary sclerosing cholangitis (PSC). Drug therapy is aimed at treating symptoms and managing complications. Immunosuppressants, chelators, and steroids are used in an attempt to control the disease process but have not shown significant benefit.Ursodeoxycholic acid improves the liver function profile in some patients and, in conjunction with endoscopic dilation, has shown a survival benefit in some studies. Trials using ursodeoxycholic acid in higher doses and earlier in the disease course are ongoing.

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Immunosuppressant agents

Class Summary

With the possibility of an autoimmune pathogenesis for PSC, immunosuppressive therapy has been used in treatment of PSC. Results of therapeutic trials, however, have been disappointing.

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Azathioprine (Imuran, Azasan)

 

Azathioprine antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. It may decrease proliferation of immune cells, which results in lower autoimmune activity.

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Cyclosporine (Sandimmune, Neoral)

 

Cyclosporine is a cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions, such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft versus host disease for a variety of organs. For both children and adults, dosing is based on ideal body weight.

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Prednisone

 

Prednisone is an immunosuppressant for the treatment of autoimmune disorders. It may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear activity. It stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.

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Methotrexate (Trexall, Rheumatrex)

 

Methotrexate is an antimetabolite used in the treatment of certain neoplastic diseases, severe psoriasis, and adult rheumatoid arthritis. It inhibits dihydrofolic acid reductase. Dihydrofolates must be reduced to tetrahydrofolates by this enzyme before they can be used as carriers of single-carbon groups in the synthesis of purine nucleotides and thymidylate. Methotrexate therefore interferes with DNA synthesis, repair, and cellular replication.

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Chelators

Class Summary

Observation of increased serum, urinary, and hepatic copper concentrations in patients with primary sclerosing cholangitis has prompted the use of penicillamine, which is a chelator.

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Penicillamine (Cuprimine, Depen)

 

Penicillamine is a chelating agent that is recommended for the removal of excess copper in patients with Wilson disease. It depresses circulating IgM rheumatoid factor and T-cell activity but not B-cell activity.

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Gallstone Solubilizing Agents

Class Summary

Ursodiol is thought to remove toxic bile acids from the enterohepatic circulation and to offer protection to the bile duct from injury.

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Ursodiol (Actigall)

 

Ursodiol suppresses hepatic synthesis and secretion of cholesterol and inhibits intestinal absorption of cholesterol. It may displace natural, toxic, and endogenous bile acids from the enterohepatic circulation and provide a cytoprotective effect, which may lead to decreased cholestasis and improved liver functions.

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Lipid-Lowering Agents, Other

Class Summary

Cholestyramine is thought to decrease pruritus by combining with bile acids in the intestine and by causing them to be excreted because of nonreabsorption.

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Cholestyramine (Prevalite, Questran)

 

Cholestyramine forms a nonabsorbable complex with bile acids in the intestine, which, in turn, inhibits enterohepatic reuptake of intestinal bile salts. In patients with partial biliary obstruction, the reduction of serum bile acid levels by cholestyramine reduces excess bile acids deposited in dermal tissue, which decreases pruritus.

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Contributor Information and Disclosures
Author

Vikas Khurana, MD, FACP, FACG  Assistant Professor, Department of Medicine, Division of Gastroenterology and Hepatology, Graduate Hospital, Gastroenterology Associates, PC

Disclosure: Nothing to disclose.

Coauthor(s)

Tejinder Singh, MD  Lead Physician, Section of Emergency Services, Overton Brooks Veterans Affairs Medical Center

Disclosure: Nothing to disclose.

Praveen K Roy, MD, AGAF  Gastroenterologist, Ochsner Clinic Foundation; Adjunct Associate Research Scientist, Lovelace Respiratory Research Institute; Editor-in-Chief, The Internet Journal of Gasteroenterology; Editorial Board, Signal Transduction Insights; Editorial Board, The Internet Journal of Epidemiology; Editorial Board, Gastrointestinal Endoscopy Review Letter

Praveen K Roy, MD, AGAF is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and American Society of Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Additional Contributors

Simmy Bank, MD Chair, Professor, Department of Internal Medicine, Division of Gastroenterology, Long Island Jewish Hospital, Albert Einstein College of Medicine

Disclosure: Nothing to disclose.

David Greenwald, MD Associate Professor of Clinical Medicine, Fellowship Program Director, Department of Medicine, Division of Gastroenterology, Montefiore Medical Center, Albert Einstein College of Medicine

David Greenwald, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, and New York Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References

  1. Gotthardt D, Chahoud F, Sauer P. Primary sclerosing cholangitis: diagnostic and therapeutic problems. Dig Dis. 2011;29 Suppl 1:41-5. [Medline].

  2. Shneider BL. Diagnostic and therapeutic challenges in pediatric primary sclerosing cholangitis. Liver Transpl. Dec 5 2011;[Medline].

  3. Trottier J, Bialek A, Caron P, Straka RJ, Heathcote J, Milkiewicz P, et al. Metabolomic profiling of 17 bile acids in serum from patients with primary biliary cirrhosis and primary sclerosing cholangitis: A pilot study. Dig Liver Dis. Dec 8 2011;[Medline].

  4. Okada H, Mizuno M, Yamamoto K, Tsuji T. Primary sclerosing cholangitis in Japanese patients: association with inflammatory bowel disease. Acta Med Okayama. Oct 1996;50(5):227-35. [Medline].

  5. Kim WR, Therneau TM, Wiesner RH, et al. A revised natural history model for primary sclerosing cholangitis. Mayo Clin Proc. Jul 2000;75(7):688-94. [Medline].

  6. Mayo Foundation for Medical Education and Research. The Revised Natural History Model for Primary Sclerosing Cholangitis. Available at http://www.mayoclinic.org/gi-rst/mayomodel3.html. Accessed March 30, 2012.

  7. Shetty K, Rybicki L, Carey WD. The Child-Pugh classification as a prognostic indicator for survival in primary sclerosing cholangitis. Hepatology. May 1997;25(5):1049-53. [Medline].

  8. Ananthakrishnan AN, Beaulieu DB, Ulitsky A, Zadvornova Y, Skaros S, Johnson K, et al. Does primary sclerosing cholangitis impact quality of life in patients with inflammatory bowel disease?. Inflamm Bowel Dis. Jul 27 2009;[Medline].

  9. Abbas G, Lindor KD. Cholangiocarcinoma in primary sclerosing cholangitis. J Gastrointest Cancer. Aug 25 2009;epub ahead of print. [Medline].

  10. Jesudian AB, Jacobson IM. Screening and diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis. Rev Gastroenterol Disord. Spring 2009;9(2):E41-7. [Medline].

  11. Ludwig J, Barham SS, LaRusso NF, et al. Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hepatology. Nov-Dec 1981;1(6):632-40. [Medline].

  12. Lindor KD, Kowdley KV, Luketic VA, Harrison ME, McCashland T, Befeler AS, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology. May 20 2009;[Medline].

  13. Shi J, Li Z, Zeng X, Lin Y, Xie WF. Ursodeoxycholic acid in primary sclerosing cholangitis: meta-analysis of randomized controlled trials. Hepatol Res. May 7 2009;[Medline].

  14. Eckhauser FE, Colleti LM, Knol JA. The changing role of surgery for sclerosing cholangitis. Dig Dis. May-Jun 1996;14(3):180-91. [Medline].

  15. Graziadei IW, Wiesner RH, Batts KP, et al. Recurrence of primary sclerosing cholangitis following liver transplantation. Hepatology. Apr 1999;29(4):1050-6. [Medline].

  16. Graziadei IW, Wiesner RH, Marotta PJ, et al. Long-term results of patients undergoing liver transplantation for primary sclerosing cholangitis. Hepatology. Nov 1999;30(5):1121-7. [Medline].

  17. Lian L, Menon KV, Shen B, Remzi F, Kiran RP. Inflammatory bowel disease complicated by primary sclerosing cholangitis and cirrhosis: is restorative proctocolectomy safe?. Dis Colon Rectum. Jan 2012;55(1):79-84. [Medline].

  18. Angulo P, Peter JB, Gershwin ME, et al. Serum autoantibodies in patients with primary sclerosing cholangitis. J Hepatol. Feb 2000;32(2):182-7. [Medline].

  19. Borkje B, Vetvik K, Odegaard S, et al. Chronic pancreatitis in patients with sclerosing cholangitis and ulcerative colitis. Scand J Gastroenterol. May 1985;20(4):539-42. [Medline].

  20. Broome U, Lofberg R, Veress B, Eriksson LS. Primary sclerosing cholangitis and ulcerative colitis: evidence for increased neoplastic potential. Hepatology. Nov 1995;22(5):1404-8. [Medline].

  21. Cangemi JR, Wiesner RH, Beaver SJ, et al. Effect of proctocolectomy for chronic ulcerative colitis on the natural history of primary sclerosing cholangitis. Gastroenterology. Mar 1989;96(3):790-4. [Medline].

  22. Chapman RW, Arborgh BA, Rhodes JM, et al. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut. Oct 1980;21(10):870-7. [Medline].

  23. Farrant JM, Hayllar KM, Wilkinson ML, et al. Natural history and prognostic variables in primary sclerosing cholangitis. Gastroenterology. Jun 1991;100(6):1710-7. [Medline].

  24. Helzberg JH, Petersen JM, Boyer JL. Improved survival with primary sclerosing cholangitis. A review of clinicopathologic features and comparison of symptomatic and asymptomatic patients. Gastroenterology. Jun 1987;92(6):1869-75. [Medline].

  25. Johnson GK, Geenen JE, Venu RP, et al. Endoscopic treatment of biliary tract strictures in sclerosing cholangitis: a larger series and recommendations for treatment. Gastrointest Endosc. Jan-Feb 1991;37(1):38-43. [Medline].

  26. Lee YM, Kaplan MM. Management of primary sclerosing cholangitis. Am J Gastroenterol. Mar 2002;97(3):528-34. [Medline].

  27. Lee YM, Kaplan MM. Primary sclerosing cholangitis. N Engl J Med. Apr 6 1995;332(14):924-33. [Medline].

  28. Lundqvist K, Broome U. Differences in colonic disease activity in patients with ulcerative colitis with and without primary sclerosing cholangitis: a case control study. Dis Colon Rectum. Apr 1997;40(4):451-6. [Medline].

  29. MacCarty RL, LaRusso NF, Wiesner RH, Ludwig J. Primary sclerosing cholangitis: findings on cholangiography and pancreatography. Radiology. Oct 1983;149(1):39-44. [Medline].

  30. Olsson R, Danielsson A, Jarnerot G, et al. Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis. Gastroenterology. May 1991;100(5 Pt 1):1319-23. [Medline].

  31. Rabinovitz M, Gavaler JS, Schade RR, et al. Does primary sclerosing cholangitis occurring in association with inflammatory bowel disease differ from that occurring in the absence of inflammatory bowel disease? A study of sixty-six subjects. Hepatology. Jan 1990;11(1):7-11. [Medline].

  32. Rosen CB, Nagorney DM, Wiesner RH, et al. Cholangiocarcinoma complicating primary sclerosing cholangitis. Ann Surg. Jan 1991;213(1):21-5. [Medline].

  33. Schrumpf E, Elgjo K, Fausa O, et al. Sclerosing cholangitis in ulcerative colitis. Scand J Gastroenterol. 1980;15(6):689-97. [Medline].

  34. Shepherd HA, Selby WS, Chapman RW, et al. Ulcerative colitis and persistent liver dysfunction. Q J Med. Autumn 1983;52(208):503-13. [Medline].

  35. Taylor BV, Wijdicks EF, Poterucha JJ, Weisner RH. Chronic inflammatory demyelinating polyneuropathy complicating liver transplantation. Ann Neurol. Nov 1995;38(5):828-31. [Medline].

  36. Terblanche J, Allison HF, Northover JM. An ischemic basis for biliary strictures. Surgery. Jul 1983;94(1):52-7. [Medline].

  37. UNOS Update. Primary liver disease of liver transplant recipients, 1991 and 1992. 1993;9(8):27-9.

  38. van Milligen de Wit AW, van Deventer SJ, Tytgat GN. Immunogenetic aspects of primary sclerosing cholangitis: implications for therapeutic strategies. Am J Gastroenterol. Jun 1995;90(6):893-900. [Medline].

  39. Wiesner RH. Current concepts in primary sclerosing cholangitis. Mayo Clin Proc. Oct 1994;69(10):969-82. [Medline].

 
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Periductal onion skin fibrosis seen in primary sclerosing cholangitis.
Endoscopic retrograde cholangiopancreatography performed in a patient with abnormal liver function test results shows multiple intrahepatic bile duct strictures and beading.
Double-contrast barium enema (same patient as in the previous image) shows filiform polyps and an ahaustral colon resulting from ulcerative colitis.
Percutaneous transhepatic cholangiogram shows dilatation, stricturing, and beading of the intrahepatic bile ducts. Note the surgical clips from a previous cholecystectomy.
T-tube cholangiogram shows irregularity of the common bile duct, stricturing, beading, and dilatation of the intrahepatic bile ducts. Note a calculus in the termination of the left hepatic duct (arrow).
Magnetic resonance cholangiopancreatography shows a normal-sized common bile duct, but strictures of both the left and right ducts are noted as well as a dilated proximal left hepatic duct.
Technetium-99m iminodiacetic acid scan shows retention of the radionuclide proximal to strictures in the distribution of the left hepatic duct. Note the lack of filling of the gallbladder because of a previous cholecystectomy. Isotope has entered the small bowel.
 
 
 
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