Advertisement

Liver Diseases pp 153-159 | Cite as

Primary Sclerosing Cholangitis

  • Andreas L. Koutsoumpas
  • George Efthymiou
  • Maria G. Mytilinaiou
  • Dimitrios P. BogdanosEmail author
Chapter

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by immune-mediated inflammation and multifocal biliary strictures, leading to cirrhosis, portal hypertension and hepatic decompensation, the only curative option being that of liver transplantation [1, 2].

Keywords

Autoimmunity Autoimmune liver disease Biliary epithelial cells Cholestasis Liver Transplantation Ulcerative colitis Ursodeoxycholic acid 

Abbreviations

AASLD

American Association for the Study of Liver Diseases

AIH

Autoimmune hepatitis

ALP

Alkaline phosphatase

ASC

Autoimmune sclerosing cholangitis

CCA

Cholangiocarcinoma

ERC

Endoscopic retrograde cholangiography

GP2

Zymogen granule glycoprotein 2

GWAS

Genome wide association studies

HLA

Human leukocyte antigen

IBD

Inflammatory bowel disease

IgG

Immunoglobulin G

MRC

Magnetic resonance cholangiography

MRI

Magnetic resonance imaging

OLT

Orthotopic liver transplantation

PBC

Primary biliary cholangitis

PSC

Primary sclerosing cholangitis

UC

Ulcerative colitis

UDCA

Ursodeoxycholic acid

References

  1. 1.
    Aadland E, Schrumpf E, Fausa O, Elgjo K, Heilo A, Aakhus T, et al. Primary sclerosing cholangitis: a long-term follow-up study. Scand J Gastroenterol. 1987;22(6):655–64.CrossRefGoogle Scholar
  2. 2.
    Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010;51(2):660–78.CrossRefGoogle Scholar
  3. 3.
    Lerut J, Demetris AJ, Stieber AC, Marsh JW, Gordon RD, Esquivel CO, et al. Intrahepatic bile duct strictures after human orthotopic liver transplantation. Recurrence of primary sclerosing cholangitis or unusual presentation of allograft rejection? Transpl Int. 1988;1(3):127–30.CrossRefGoogle Scholar
  4. 4.
    Thorpe ME, Scheuer PJ, Sherlock S. Primary sclerosing cholangitis, the biliary tree, and ulcerative colitis. Gut. 1967;8(5):435–48.CrossRefGoogle Scholar
  5. 5.
    Wee A, Ludwig J, Coffey RJ Jr, LaRusso NF, Wiesner RH. Hepatobiliary carcinoma associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hum Pathol. 1985;16(7):719–26.CrossRefGoogle Scholar
  6. 6.
    Lazaridis KN, LaRusso NF. Primary sclerosing cholangitis. N Engl J Med. 2016;375(25):2501–2.PubMedGoogle Scholar
  7. 7.
    Al Mamari S, Djordjevic J, Halliday JS, Chapman RW. Improvement of serum alkaline phosphatase to <1.5 upper limit of normal predicts better outcome and reduced risk of cholangiocarcinoma in primary sclerosing cholangitis. J Hepatol. 2013;58(2):329–34.CrossRefGoogle Scholar
  8. 8.
    Burak KW, Angulo P, Lindor KD. Is there a role for liver biopsy in primary sclerosing cholangitis? Am J Gastroenterol. 2003;98(5):1155–8.CrossRefGoogle Scholar
  9. 9.
    Molodecky NA, Kareemi H, Parab R, Barkema HW, Quan H, Myers RP, et al. Incidence of primary sclerosing cholangitis: a systematic review and meta-analysis. Hepatology. 2011;53(5):1590–9.CrossRefGoogle Scholar
  10. 10.
    Tischendorf JJ, Hecker H, Kruger M, Manns MP, Meier PN. Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: a single center study. Am J Gastroenterol. 2007;102(1):107–14.CrossRefGoogle Scholar
  11. 11.
    Boonstra K, Weersma RK, van Erpecum KJ, Rauws EA, Spanier BW, Poen AC, et al. Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis. Hepatology. 2013;58(6):2045–55.CrossRefGoogle Scholar
  12. 12.
    Angulo P, Peter JB, Gershwin ME, DeSotel CK, Shoenfeld Y, Ahmed AE, et al. Serum autoantibodies in patients with primary sclerosing cholangitis. J Hepatol. 2000;32(2):182–7.CrossRefGoogle Scholar
  13. 13.
    Pavlidis P, Forbes A, Bogdanos DP. Anti-GP2 antibodies in inflammatory bowel disease patients with ileal pouch. J Crohns Colitis. 2013;7(11):e602–3.CrossRefGoogle Scholar
  14. 14.
    Pavlidis P, Komorowski L, Teegen B, Liaskos C, Koutsoumpas AL, Smyk DS, et al. Diagnostic and clinical significance of Crohn’s disease-specific pancreatic anti-GP2 and anti-CUZD1 antibodies. Clin Chem Lab Med. 2016;54(2):249–56.CrossRefGoogle Scholar
  15. 15.
    Sowa M, Kolenda R, Baumgart DC, Pratschke J, Papp M, Tornai T, et al. Mucosal autoimmunity to cell-bound GP2 isoforms is a sensitive marker in PSC and associated with the clinical phenotype. Front Immunol. 2018;9:1959.CrossRefGoogle Scholar
  16. 16.
    Ludwig J, Barham SS, LaRusso NF, Elveback LR, Wiesner RH, McCall JT. Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hepatology. 1981;1(6):632–40.CrossRefGoogle Scholar
  17. 17.
    Gohlke F, Lohse AW, Dienes HP, Lohr H, Marker-Hermann E, Gerken G, et al. Evidence for an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis. J Hepatol. 1996;24(6):699–705.CrossRefGoogle Scholar
  18. 18.
    Loftus EV Jr, Sandborn WJ, Tremaine WJ, Mahoney DW, Zinsmeister AR, Offord KP, et al. Risk of colorectal neoplasia in patients with primary sclerosing cholangitis. Gastroenterology. 1996;110(2):432–40.CrossRefGoogle Scholar
  19. 19.
    Boonstra K, van Erpecum KJ, van Nieuwkerk KM, Drenth JP, Poen AC, Witteman BJ, et al. Primary sclerosing cholangitis is associated with a distinct phenotype of inflammatory bowel disease. Inflamm Bowel Dis. 2012;18(12):2270–6.CrossRefGoogle Scholar
  20. 20.
    Razumilava N, Gores GJ. Classification, diagnosis, and management of cholangiocarcinoma. Clin Gastroenterol Hepatol. 2013;11(1):13–21 e1. quiz e3-4CrossRefGoogle Scholar
  21. 21.
    Lewis JT, Talwalkar JA, Rosen CB, Smyrk TC, Abraham SC. Prevalence and risk factors for gallbladder neoplasia in patients with primary sclerosing cholangitis: evidence for a metaplasia-dysplasia-carcinoma sequence. Am J Surg Pathol. 2007;31(6):907–13.CrossRefGoogle Scholar
  22. 22.
    Jiang X, Karlsen TH. Genetics of primary sclerosing cholangitis and pathophysiological implications. Nat Rev Gastroenterol Hepatol. 2017;14(5):279–95.CrossRefGoogle Scholar
  23. 23.
    Lichtman SN, Keku J, Clark RL, Schwab JH, Sartor RB. Biliary tract disease in rats with experimental small bowel bacterial overgrowth. Hepatology. 1991;13(4):766–72.CrossRefGoogle Scholar
  24. 24.
    Rudolph G, Gotthardt D, Kloters-Plachky P, Kulaksiz H, Rost D, Stiehl A. Influence of dominant bile duct stenoses and biliary infections on outcome in primary sclerosing cholangitis. J Hepatol. 2009;51(1):149–55.CrossRefGoogle Scholar
  25. 25.
    Lindor KD. Ursodiol for primary sclerosing cholangitis. Mayo Primary Sclerosing Cholangitis-Ursodeoxycholic Acid Study Group. N Engl J Med. 1997;336(10):691–5.CrossRefGoogle Scholar
  26. 26.
    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. 2009;50(3):808–14.CrossRefGoogle Scholar
  27. 27.
    Olsson R, Boberg KM, de Muckadell OS, Lindgren S, Hultcrantz R, Folvik G, et al. High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5-year multicenter, randomized, controlled study. Gastroenterology. 2005;129(5):1464–72.CrossRefGoogle Scholar
  28. 28.
    Dyson JK, Beuers U, Jones DEJ, Lohse AW, Hudson M. Primary sclerosing cholangitis. Lancet. 2018;391(10139):2547–59.CrossRefGoogle Scholar
  29. 29.
    Hansen JD, Kumar S, Lo WK, Poulsen DM, Halai UA, Tater KC. Ursodiol and colorectal cancer or dysplasia risk in primary sclerosing cholangitis and inflammatory bowel disease: a meta-analysis. Dig Dis Sci. 2013;58(11):3079–87.CrossRefGoogle Scholar
  30. 30.
    Allegretti JR, Kassam Z, Carrellas M, Mullish BH, Marchesi JR, Pechlivanis A, et al. Fecal microbiota transplantation in patients with primary sclerosing cholangitis: a pilot clinical trial. Am J Gastroenterol. 2019;114(7):1071–9.CrossRefGoogle Scholar
  31. 31.
    European Association for the Study of the L. EASL Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol. 2009;51(2):237–67.CrossRefGoogle Scholar
  32. 32.
    Kamath PS, Wiesner RH, Malinchoc M, Kremers W, Therneau TM, Kosberg CL, et al. A model to predict survival in patients with end-stage liver disease. Hepatology. 2001;33(2):464–70.CrossRefGoogle Scholar
  33. 33.
    Steenstraten IC, Sebib Korkmaz K, Trivedi PJ, Inderson A, van Hoek B, Rodriguez Girondo MDM, et al. Systematic review with meta-analysis: risk factors for recurrent primary sclerosing cholangitis after liver transplantation. Aliment Pharmacol Ther. 2019;49(6):636–43.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Andreas L. Koutsoumpas
    • 1
  • George Efthymiou
    • 1
  • Maria G. Mytilinaiou
    • 1
  • Dimitrios P. Bogdanos
    • 1
    Email author
  1. 1.Division of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health SciencesUniversity of ThessalyLarissaGreece

Personalised recommendations