Skip to main content

Advertisement

Log in

Primary sclerosing cholangitis treated by endoscopic biliary dilation: Review and long-term follow-up evaluation

  • Published:
Current Gastroenterology Reports Aims and scope Submit manuscript

Abstract

Primary sclerosing cholangitis (PSC) is an important cause of chronic liver disease. We review the management of PSC and report a 20-year follow-up of our initial 10 patients. This is the longest detailed follow-up of a group of PSC patients to date. We discuss the clinical course and results of endoscopic management in these patients and relate these data to management of PSC in general. We compare the actual survival of these patients to predicted survival scores based on the Mayo multicenter survival model. Although our patients presented with cholangitis, which typically reflects advanced stages of liver disease, their survival compares favorably with expected survival in unselected PSC patients. Endoscopic balloon dilation of PSC patients presenting with biliary strictures and cholangitis may have long-term benefit in addition to short-term symptomatic relief.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References and Recommended Reading

  1. MacFaul G, Chapman R: Sclerosing cholangitis. Curr Opin Gastroenterol 2004, 20:275–280.

    Article  PubMed  Google Scholar 

  2. Bambha K, Kim W, Talwalkar J, et al.: Incidence, clinical spectrum and outcomes of primary sclerosing cholangitis in a united states community. Gastroenterology 2003, 125:1364–1369. This paper is the first to supply information from a US population base that provides an estimate of the incidence and prevalence of PSC. The authors suggest that the prevalence is greater than previously suspected.

    Article  PubMed  Google Scholar 

  3. Talwalkar J, Lindor K: Primary sclerosing cholangitis. Inflamm Bowel Dis 2005, 11:62–72.

    Article  PubMed  Google Scholar 

  4. Graziadei I, Wiesner R, Marotta P, et al.: Long term results of patients undergoing liver transplantation for primary sclerosing cholangitis. Hepatology 1999, 30:1121–1127.

    Article  PubMed  CAS  Google Scholar 

  5. Klompmaker I, Haagsma E, Verwer R, et al.: Primary sclerosing cholangitis and liver transplantation. Scand J Gastroenterol 1996, 31(Suppl 218):98–102.

    Google Scholar 

  6. Wiesner R, Porayko M, Dickson E, et al.: Selection and timing of liver transplantation in primary biliary cirrhosis and primary sclerosing cholangitis. Hepatology 1992, 16:1290–1299. This paper describes the analysis of risk factors in the computation of a Cox regression model that can be applied to therapeutic approaches and risk categorization of patient groups.

    PubMed  CAS  Google Scholar 

  7. Schrumpf E, Abdelnoor M, Fausa O, et al.: Risk factors in primary sclerosing cholangitis. J Hepatol 1994, 21:1061–1066.

    Article  PubMed  CAS  Google Scholar 

  8. Farges O, Malassagne B, Sebagh M, et al.: Primary sclerosing cholangitis: liver transplantation or biliary surgery. Surgery 1995, 117:146–155.

    Article  PubMed  CAS  Google Scholar 

  9. Blendis L, Lurie Y: Primary sclerosing cholangitis: A premalignant condition. Gastroenterology 2002, 123:647–648.

    Article  PubMed  Google Scholar 

  10. Burak K, Angulo P, Pasha T, et al.: Incidence and risk factors for cholangiocarcinoma in primary sclerosing cholangitis. Am J Gastroenterol 2004, 99:523–526.

    Article  PubMed  Google Scholar 

  11. Ahrendt S, Pitt H, Nakeeb gnA, et al.: Diagnosis and management of cholangiocarcinoma in primary sclerosing cholangitis. J Gastrointest Surg 1999, 357–367.

  12. Fisher A, Theise ND, Min A, et al.: CA19-9 does not predict cholangiocarcinoma in patients with primary sclerosing cholangitis undergoing liver transplantation. Liver Transplant Surg 1995, 1:94–98.

    Article  CAS  Google Scholar 

  13. Siqueira E, Schoen R, Silverman W, et al.: Detecting cholangiocarcinoma in patients with primary sclerosing cholangitis. Gastrointest Endosc 2002, 56:40–47. This paper discusses the most accurate tests for the detection of CCA.

    Article  PubMed  Google Scholar 

  14. United Network for Organ Sharing (UNOS): Transplant patient data; 2005 (Data as of August 15, 2004; Personal communication).

  15. Johnson G, Geenen J, Venu R, Hogan W: Endoscopic treatment of biliary duct strictures in sclerosing cholangitis: follow-up assessment of a new therapeutic approach. Gastrointest Endosc 1987, 33:9–12.

    PubMed  CAS  Google Scholar 

  16. Lindor K, Larusso N: Primary sclerosing cholangitis. In Diseases of the Liver. Edited by Schiff E, Sorrell M, Maddrey W. Philadelphia: Lippincott, Williams & Wilkins; 2003:673–684. This textbook chapter presents a broad review of PSC and discusses recent data regarding testing and treatment of PSC patients.

    Google Scholar 

  17. Huibregtse K, Tytgat G: Palliative treatment of obstructive jaundice by transpapillary introduction of large bore bile duct endoprosthesis. Gut 1982, 23:371–375. This paper was the first to report a substantial number of patients improved with invasive therapeutic ERCP as applied to PSC. Other important papers were published later by this group.

    PubMed  CAS  Google Scholar 

  18. Johnson G, Geenen J, Venu R, et al.: Endoscopic treatment of biliry strictures in sclerosing cholangitis: A larger series and recommendations for treatment. Gastrointest Endosc 1991, 37:38–43. The first report to measure improvement in terms of hospital admissions, laboratory values, and anatomic changes in the biliary tract in a substantial number of patients.

    PubMed  CAS  Google Scholar 

  19. Lombard M, Farrant M, Karani J, et al.: Improving biliary-enteric drainage in primary sclerosing cholangitis: experience with endoscopic methods. Gut 1991, 32:1364–1368.

    PubMed  CAS  Google Scholar 

  20. Cotton P, Nickl N: Endoscopic and radiologic approaches to therapy in primary sclerosing cholangitis. Semin Liver Dis 1991, 11:40–48.

    PubMed  CAS  Google Scholar 

  21. Gaing A, Geders K, Cohen S, et al.: Endoscopic management of primary sclerosing cholangitis: review and report of an open series. Am J Gastroenterol 1993, 12:2000–2008.

    Google Scholar 

  22. VanLaethem J, Deviere J, Bourgeois N, et al.: Cholangiographic findings in deteriorating primary sclerosing cholangitis. Endoscopy 1995, 27:223–228.

    CAS  Google Scholar 

  23. Lee J, Schutz S, England R, et al.: Endoscopic therapy of sclerosing Cholangitis. Hepatology 1995, 21:661–667.

    PubMed  CAS  Google Scholar 

  24. Van Milligen de Wit A, van Bracht J, Rauws E, et al.: Endoscopic stent therapy for dominant extrahepatic bile duct strictures in primary sclerosing cholangitis. Gastrointest Endosc 1996, 44:293–299.

    Article  PubMed  Google Scholar 

  25. Wagner S, Gebel M, Meier P, et al.: Endoscopic management of biliary tract strictures in primary sclerosing cholangitis. Endoscopy 1996, 28:546–551.

    PubMed  CAS  Google Scholar 

  26. Ahrendt S, Pitt H, Kalloo A, et al.: Primary sclerosing cholangitis: resect, dilate or transplant? Ann Surg 1998, 227:412–423.

    Article  PubMed  CAS  Google Scholar 

  27. Ponsioen L, Lam K, van Milligen de Wit A, et al.: Four years experience with short term stenting in primary sclerosing cholangitis. Am J Gastroenterol 1999, 94:2403–2407.

    Article  PubMed  CAS  Google Scholar 

  28. Kaya M, Petersen B, Angulo P, et al.: Balloon dilation compared to stenting of dominant strictures in primary sclerosing cholangitis. Am J Gastroenterol 2001, 96:1069–1066.

    Google Scholar 

  29. Baluyut A, Sherman S, Lehman G, et al.: Impact of endoscopic therapy on the survival of patients with primary sclerosing cholangitis. Gastrointest Endosc 2001, 53:308–312. This paper compares a Kaplan-Meier survival plot of patients treated endoscopically in accordance with the Mayo multicenter model. Computations of expected survival in PSC patients and actual survival showed a significant survival improvement after dilation of biliary strictures.

    PubMed  CAS  Google Scholar 

  30. Stiehl A, Rudolph G, Kloters-Plachky R, et al.: Development of dominant bile duct stenoses in patients with primary sclerosing cholangitis treated with ursodeoxycholic acid: outcome after endoscopic treatment. J Hepatol 2002, 36:151–156. This paper compares expected survival using the Mayo multicenter model with actual survival and reports significant improvement in patients who were treated with EBD and ursodeoxycholic acid. It also reports significant numbers of patients who developed CCA during the time of observation.

    Article  PubMed  Google Scholar 

  31. Reznick R, Guest C: Survival analysis: a practical approach. Dis Colon Rectum 1989, 32:898–902.

    Article  PubMed  CAS  Google Scholar 

  32. Dickson E, Murtaugh P, Wiesner R, et al.: Primary sclerosing cholangitis: Refinement and validation of survival models. Gastroenterology 1992, 103:1893–1901.

    PubMed  CAS  Google Scholar 

  33. Kobayashi H, Hosotani R, Imai Y, et al.: Primary sclerosing cholangitis in which differential diagnosis from gallbladder carcinoma was difficult. J Hepatobiliary Pancreat Surg 2005, 12:151–154.

    Article  PubMed  Google Scholar 

  34. Alifieri C, Tanner T, Carpentier et al.: Epstein-Barr virus transmission from a blood donor to an organ transplant recipient with recovery of the same virus strain from the recipient’s blood and oropharynx. Blood 1996, 87:812–817.

    Google Scholar 

  35. Kenagy D, Schlesinger Y, Weck K, et al.: Epstein-Barr virus DNA in peripheral blood leukocytes of patients with post-transplant lymphoproliferative disease. Transplantation 1995, 60:547–554.

    Article  PubMed  CAS  Google Scholar 

  36. Weisner R, La Russo N, Dozois R, et al.: Peristomal varices after proctocolectomy in patients with primary sclerosing cholangitis. Gastroenterology 1986, 90:316–322.

    Google Scholar 

  37. Nashan B, Schlitt H, Tusch G: Biliary malignancies in primary sclerosing cholangitis: timing for liver transplantation. Hepatology 1996, 23:1105–1111.

    Article  PubMed  CAS  Google Scholar 

  38. Bergquist A, Ekbom A, Olsson R, et al.: Hepatic and extrahepatic malignancies in primary sclerosing cholangitis. J Hepatol 2002, 36:321–327.

    Article  PubMed  Google Scholar 

  39. Chalasani N, Baluyut A, Ismail A, et al.: Cholangiocarcinoma in patients with primary sclerosing cholangitis: A multi center case control study. Hepatology 2000, 31:7–11.

    Article  PubMed  CAS  Google Scholar 

  40. Jailwala J, Fogel E, Sherman S, et al.: Triple tissue sampling at ERCP in malignant biliary obstruction. Gastrointest Endosc 2000, 51:383–390.

    Article  PubMed  CAS  Google Scholar 

  41. Konstantinos N, Gores L, Gores G: Cholangiocarcinoma. Gastroenterology 2005, 128:1656–1667.

    Google Scholar 

  42. Ramage J, Donaghy A, Farrant J, et al.: Serum tumor markers for the diagnosis of cholangiocarcinoma in primary sclerosing cholangitis. Gastroenterology 1995, 108:865–869.

    Article  PubMed  CAS  Google Scholar 

  43. Itoi T, Shinohara Y, Takeda K, et al.: Detection of telomerase activity in biopsy specimens for diagnosis of biliary tract cancers. Gastrointest Endosc 2000, 52:380–386.

    Article  PubMed  CAS  Google Scholar 

  44. Knechtle S, D’Alessandro A, Harms B, et al.: Relationships between sclerosing cholangitis, inflammatory bowel disease and cancer in patients undergoing liver transplantation. Surgery 1995, 118:615–620.

    Article  PubMed  CAS  Google Scholar 

  45. Heimbach J, Madock N, Alberts S, et al.: Transplantation for hilar cholangiocarcinoma. Liver Transplantation 2004, 10:S.65-S.68. Pilot study showing the improvement of CCA/PSC patients transplanted after treatment with radiation and chemotherapy. These patients were previously thought not to be candidates for transplant.

    Article  Google Scholar 

  46. Kim W, Poterucha J, Wiesner R, et al.: The relative role of the Child-Pugh classification and the Mayo natural history model in the assessment of survival in patients with primary sclerosing cholangitis. Hepatology 1999, 29:1643–1648.

    Article  PubMed  CAS  Google Scholar 

  47. Bjoro K, Friman S, Hockerstedt K, et al.: Liver transplantation in the Nordic countries, 1982–1998: changes of indications and improving results. Scand J Gastroenterol 1999, 34:714–722.

    Article  PubMed  CAS  Google Scholar 

  48. Kim W, Therneau T, Wiesner R, et al.: A revised natural history model for primary sclerosing cholangitis. Mayo Clin Proc 2000, 75:688–694.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Kia Saeian MD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Johnson, G.K., Saeian, K. & Geenen, J.E. Primary sclerosing cholangitis treated by endoscopic biliary dilation: Review and long-term follow-up evaluation. Curr Gastroenterol Rep 8, 147–155 (2006). https://doi.org/10.1007/s11894-006-0011-y

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11894-006-0011-y

Keywords

Navigation