Skip to main content

Recurrent Primary Sclerosing Cholangitis in Comparison with Native Primary Sclerosing Cholangitis

  • Chapter
  • First Online:
Pathology of the Bile Duct

Abstract

This chapter covers primary sclerosing cholangitis (PSC) and its overlap with autoimmune hepatitis (AIH) and liver transplantation (LT) for PSC. Histologic findings similar to those of PSC are known to occur in liver allografts transplanted for PSC; this is thought to be a recurrence of the original PSC. We mainly focus on characterizing the histologic features of recurrent PSC after LT with an emphasis on the overlap of active hepatitis and hilar xanthogranulomatous cholangitis with respect to outcome. These histologic findings may represent an important and unrecognized cause of progressive deterioration followed by cirrhotic transformation in recurrent PSC after LT.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 159.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Reference

  1. Nakanuma Y, Zen Y, Portmann B. Diseases of the bile ducts. In: Burt A, Portmann B, Ferrell L, editors. MacSween’s pathology of the liver. 6th ed. New York: Churchill Livingstone ELSEVIER; 2012. p. 491–562.

    Chapter  Google Scholar 

  2. Tischendorf JJ, Geier A, Trautwein C. Current diagnosis and management of primary sclerosing cholangitis. Liver Transpl. 2008;14:735–46.

    Article  PubMed  Google Scholar 

  3. Miyagawa-Hayashino A, Egawa H, Yoshizawa A, Ueda Y, Ichida T, Ueno Y, Uemoto S, Harada K, Nakanuma Y. Frequent overlap of active hepatitis in recurrent primary sclerosing cholangitis after living-donor liver transplantation relates to its rapidly progressive course. Hum Pathol. 2011;42:1329–36.

    Article  PubMed  Google Scholar 

  4. van Buuren HR, van Hoogstraten HJE, Terkivatan T, Schalm SW, Vleggaar FP. High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis. J Hepatol. 2000;33:543–8.

    Article  PubMed  Google Scholar 

  5. Gregorio GV, Portmann B, Karani J, Harrison P, Donaldson PT, Vergani D, Mieli-Vergani G. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology. 2001;33:544–53.

    Article  CAS  PubMed  Google Scholar 

  6. Abdo AA, Bain VG, Kichian K, Lee SS. Evolution of autoimmune hepatitis to primary sclerosing cholangitis: a sequential syndrome. Hepatology. 2002;36:1393–9.

    Article  PubMed  Google Scholar 

  7. Abdalian R, Dhar P, Jhaveri K, Haider M, Guindi M, Heathcote EJ. Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: evaluating the role of routine magnetic resonance imaging. Hepatology. 2008;47:949–57.

    Article  CAS  PubMed  Google Scholar 

  8. Mieli-Vergani G, Vergani D. Unique features of primary sclerosing cholangitis in children. Curr Opin Gastroenterol. 2010;26:265–8.

    Article  PubMed  Google Scholar 

  9. Boberg KM, Chapman RW, Hirschfield GM, Lohse AW, Manns MP, Schrumpf E. Overlap syndromes: the International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue. J Hepatol. 2011;54:374–85.

    Article  PubMed  Google Scholar 

  10. Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, Chapman RW, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. 1999;31:929–38.

    Article  CAS  PubMed  Google Scholar 

  11. Zenouzi R, Lohse AW. Long-term outcome in PSC/AIH “overlap syndrome”: does immunosuppression also treat the PSC component? J Hepatol. 2014;61:1189–91.

    Article  PubMed  Google Scholar 

  12. Hildebrand T, Pannicke N, Dechene A, Gotthardt DN, Kirchner G, Reiter FP, Sterneck M, Herzer K, Lenzen H, Rupp C, Barg-Hock H, de Leuw P, Teufel A, Zimmer V, Lammert F, Sarrazin C, Spengler U, Rust C, Manns MP, Strassburg CP, Schramm C. Weismüller TJ; German PSC Study Group. German PSC Study Group. Biliary strictures and recurrence after liver transplantation for primary sclerosing cholangitis: a retrospective multicenter analysis. Liver Transpl. 2016;22:42–52.

    Article  PubMed  Google Scholar 

  13. Demetris AJ, Minervini M, Nalesnik M, Ochoa E, Randhawa P, Sasatomi E, Wu T. Diseases of the bile ducts. In: Ruiz P, editor. Transplantation pathology. New York: Cambridge University Press; 2009. p. 111–84.

    Google Scholar 

  14. Graziadei IW. Recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl. 2002;8:575–81.

    Article  PubMed  Google Scholar 

  15. Harrison RF, Davies MH, Neuberger JM, Hubscher SG. Fibrous and obliterative cholangitis in liver allografts: evidence of recurrent primary sclerosing cholangitis? Hepatology. 1994;20:356–61.

    Article  CAS  PubMed  Google Scholar 

  16. Sheng R, Campbell WL, Zajko AB, Baron RL. Cholangiographic features of biliary strictures after liver transplantation for primary sclerosing cholangitis: evidence of recurrent disease. AJR Am J Roentgenol. 1996;166:1109–13.

    Article  CAS  PubMed  Google Scholar 

  17. Graziadei IW, Wiesner RH, Batts KP, Marotta PJ, LaRusso NF, Porayko MK, Hay JE, Gores GJ, Charlton MR, Ludwig J, Poterucha JJ, Steers JL, Krom RA. Recurrence of primary sclerosing cholangitis following liver transplantation. Hepatology. 1999;29:1050–6.

    Article  CAS  PubMed  Google Scholar 

  18. Graziadei IW, Wiesner RH, Marotta PJ, Porayko MK, Hay JE, Charlton MR, Poterucha JJ, Rosen CB, Gores GJ, LaRusso NF, Krom RA. Long-term results of patients undergoing liver transplantation for primary sclerosing cholangitis. Hepatology. 1999;30:1121–7.

    Article  CAS  PubMed  Google Scholar 

  19. Haga H, Miyagawa-Hayashino A, Taira K, Morioka D, Egawa H, Takada Y, Manabe T, Uemoto S. Histological recurrence of autoimmune liver diseases after living-donor liver transplantation. Hepatol Res. 2007;37(Suppl 3):S463–9.

    Article  PubMed  Google Scholar 

  20. Tamura S, Sugawara Y, Kaneko J, Matsui Y, Togashi J, Makuuchi M. Recurrence of primary sclerosing cholangitis after living donor liver transplantation. Liver Int. 2007;27:86–94.

    Article  PubMed  Google Scholar 

  21. Egawa H, Taira K, Teramukai S, Haga H, Ueda Y, Yonezawa A, Masuda S, Tsuji H, Ashihara E, Takada Y, Uemoto S. Risk factors for recurrence of primary sclerosing cholangitis after living donor liver transplantation: a single center experience. Dig Dis Sci. 2009;54:1347–54.

    Article  PubMed  Google Scholar 

  22. Egawa H, Ueda Y, Ichida T, Teramukai S, Nakanuma Y, Onishi S, Tsubouchi H. Risk factors for recurrence of primary sclerosing cholangitis after living donor liver transplantation in Japanese registry. Am J Transplant. 2011;11:518–27.

    Article  CAS  PubMed  Google Scholar 

  23. Alabraba E, Nightingale P, Gunson B, Hubscher S, Olliff S, Mirza D, Neuberger J. A re-evaluation of the risk factors for the recurrence of PSC in liver allografts. Liver Transpl. 2009;15:330–40.

    Article  PubMed  Google Scholar 

  24. Khettry U, Keaveny A, Goldar-Najafi A, Lewis WD, Pomfret EA, Pomposelli JJ, Jenkins RL, Gordon FD. Liver transplantation for primary sclerosing cholangitis: a long-term clinicopathologic study. Hum Pathol. 2003;34:1127–36.

    Article  PubMed  Google Scholar 

  25. Keaveny AP, Gordon FD, Goldar-Najafi A, Lewis WD, Pomfret EA, Pomposelli JJ, Jenkins RL, Khettry U. Native liver xanthogranulomatous cholangiopathy in primary sclerosing cholangitis: impact on posttransplant outcome. Liver Transpl. 2004;10:115–22.

    Article  PubMed  Google Scholar 

  26. Demetris AJ. Distinguishing between recurrent primary sclerosing cholangitis and chronic rejection. Liver Transpl. 2006;12:S68–72.

    Article  PubMed  Google Scholar 

  27. Kerkar N, Yanni G. De novo and ‘recurrent’ autoimmune hepatitis after liver transplantation: a comprehensive review. J Autoimmun. 2016;66:17–24.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Aya Miyagawa-Hayashino .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer Nature Singapore Pte Ltd.

About this chapter

Cite this chapter

Miyagawa-Hayashino, A., Haga, H. (2017). Recurrent Primary Sclerosing Cholangitis in Comparison with Native Primary Sclerosing Cholangitis. In: Nakanuma, Y. (eds) Pathology of the Bile Duct. Springer, Singapore. https://doi.org/10.1007/978-981-10-3500-5_7

Download citation

  • DOI: https://doi.org/10.1007/978-981-10-3500-5_7

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-10-3499-2

  • Online ISBN: 978-981-10-3500-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics