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Primary Sclerosing Cholangitis

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Diseases of the Liver and Biliary Tree

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Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by progressive biliary inflammation and fibrosis. PSC has a strong association with inflammatory bowel disease (IBD), which is diagnosed in 60–80% of patients. The coexistence of the PSC and IBD increases the risk for colorectal cancer (CRC). Furthermore, PSC predisposes to the occurrence of other neoplastic conditions, such as cholangiocarcinoma (CCA), gallbladder, and hepatocellular carcinoma.

The pathogenesis of PSC is still unclear. Current evidence supports a multifactorial etiology, where genetic predisposition, altered gut microbiota, and immune-mediated phenomena play a crucial role.

The highly variable natural history of PSC, with possible intercurrent clinical events (e.g., cholangitis, biliary lithiasis) that could be dissociated from the severity of underlying liver disease with consequent fluctuant clinical symptoms and serum cholestasis marker, makes the prognostic assessment of these patients challenging. To date, no effective medical therapy has been registered, and liver transplantation is the only therapeutic option in patients developing end-stage liver disease.

In this chapter, we describe epidemiological and clinical features of PSC, current understanding of its pathogenesis, and address challenges and opportunities associated with its management while focusing on risk stratification.

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References

  1. Hirschfield GM, Karlsen TH, Lindor KD, Adams DH. Primary sclerosing cholangitis. Lancet. 2013;382(9904):1587–99.

    Article  PubMed  Google Scholar 

  2. Boonstra K, Beuers U, Ponsioen CY. Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review. J Hepatol. 2012;56(5):1181–8.

    Article  PubMed  Google Scholar 

  3. Boonstra K, Weersma RK, van Erpecum KJ, Rauws EA, Spanier BWM, Poen AC, et al. Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis. Hepatology. 2013;58(6):2045–55.

    Article  CAS  PubMed  Google Scholar 

  4. 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.

    Article  PubMed  Google Scholar 

  5. Bakhshi Z, Hilscher MB, Gores GJ, Harmsen WS, Viehman JK, LaRusso NF, et al. An update on primary sclerosing cholangitis epidemiology, outcomes and quantification of alkaline phosphatase variability in a population-based cohort. J Gastroenterol. 2020;55(5):523–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Boonstra K, Van Erpecum KJ, Van Nieuwkerk KMJ, Drenth JPH, Poen AC, Witteman BJM, et al. Primary sclerosing cholangitis is associated with a distinct phenotype of inflammatory bowel disease. Inflamm Bowel Dis. 2012;18(12):2270–6.

    Article  PubMed  Google Scholar 

  7. Tanaka A, Tazuma S, Okazaki K, Tsubouchi H, Inui K, Takikawa H. Nationwide survey for primary sclerosing cholangitis and IgG4-related sclerosing cholangitis in Japan. J Hepatobiliary Pancreat Sci. 2014;21(1):43–50.

    Article  PubMed  Google Scholar 

  8. Wörns MA, Lohse AW, Neurath MF, Croxford A, Otto G, Kreft A, et al. Five cases of de novo inflammatory bowel disease after orthotopic liver transplantation. Am J Gastroenterol. 2006;101(8):1931–7.

    Article  PubMed  Google Scholar 

  9. Joo M, Abreu-E-Lima P, Farraye F, Smith T, Swaroop P, Gardner L, et al. Pathologic features of ulcerative colitis in patients with primary sclerosing cholangitis: a case-control study. Am J Surg Pathol. 2009;33(6):854–62.

    Article  PubMed  Google Scholar 

  10. Karlsen T, Franke A, Melum E, Kaser A, et al. Genome-wide association analysis in primary sclerosing cholangitis. Gastroenterology. 2010;138:1102–11.

    Article  PubMed  Google Scholar 

  11. Liu J, Hov J, Folseraas T, et al. Dense genotyping of immune-related disease regions identifies nine new risk loci for primary sclerosing cholangitis. Nat Genet. 2013;45:670–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Ellinghaus D, Jostins L, Spain SL, Cortes A, Bethune J, Han B, et al. Analysis of five chronic inflammatory diseases identifies 27 new associations and highlights disease-specific patterns at shared loci. Nat Genet. 2016;48(5):510–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Dean G, Hanauer S, Levitsky J. The role of the intestine in the pathogenesis of primary sclerosing cholangitis: evidence and therapeutic implications. Hepatology. 2020;72(3):1127–38.

    Google Scholar 

  14. Steck N, Hoffmann M, Sava IG, Kim SC, Hahne H, Tonkonogy SL, et al. Enterococcus faecalis metalloprotease compromises epithelial barrier and contributes to intestinal inflammation. Gastroenterology. 2011;141:959.

    Article  CAS  PubMed  Google Scholar 

  15. Sabino J, Vieira-Silva S, Machiels K, Joossens M, Falony G, Ballet V, et al. Primary sclerosing cholangitis is characterised by intestinal dysbiosis independent from IBD. Gut. 2016;65(10):1681–9.

    Article  CAS  PubMed  Google Scholar 

  16. Nakamoto N, Sasaki N, Aoki R, Miyamoto K, Suda W, Teratani T, et al. Gut pathobionts underlie intestinal barrier dysfunction and liver T helper 17 cell immune response in primary sclerosing cholangitis. Nat Microbiol. 2019;4(3):492–503.

    Article  CAS  PubMed  Google Scholar 

  17. Hobson CH, Butt TJ, Ferry DM, Hunter J, Chadwick VS, Broom MF. Enterohepatic circulation of bacterial chemotactic peptide in rats with experimental colitis. Gastroenterology. 1988;94(4):1006–13.

    Article  CAS  PubMed  Google Scholar 

  18. Lichtman SN, Keku J, Schwab JH, Sartor RB. Hepatic injury associated with small bowel bacterial overgrowth in rats is prevented by metronidazole and tetracycline. Gastroenterology. 1991;100(2):513–9.

    Article  CAS  PubMed  Google Scholar 

  19. European Association for the Study of the Liver. EASL clinical practice guidelines: management of cholestatic liver diseases. J Hepatol. 2009;51(2):237–67.

    Article  Google Scholar 

  20. Björnsson E, Olsson R, Bergquist A, Lindgren S, Braden B, Chapman RW, et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology. 2008;134(4):975–80.

    Article  PubMed  Google Scholar 

  21. Angulo P, Maor-Kendler Y, Lindor KD. Small-duct primary sclerosing cholangitis: a long-term follow-up study. Hepatology. 2002;35(6):1494–500.

    Article  PubMed  Google Scholar 

  22. Karlsen TH, Folseraas T, Thorburn D, Vesterhus M. Primary sclerosing cholangitis—a comprehensive review. J Hepatol. 2017;67:1298–323.

    Article  PubMed  Google Scholar 

  23. Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344(10):732–8.

    Article  CAS  PubMed  Google Scholar 

  24. Löhr JM, Beuers U, Vujasinovic M, Alvaro D, Frøkjær JB, Buttgereit F, et al. European guideline on IgG4-related digestive disease—UEG and SGF evidence-based recommendations. United Eur Gastroenterol J. 2020;8(6):637–66.

    Article  Google Scholar 

  25. Stone J, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539–51.

    Article  CAS  PubMed  Google Scholar 

  26. Alderlieste YA, van den Elzen BDJ, Rauws EAJ, Beuers U. Immunoglobulin G4-associated cholangitis: one variant of immunoglobulin G4-related systemic disease. Digestion. 2009;79(4):220–8.

    Article  CAS  PubMed  Google Scholar 

  27. Ravi K, Chari ST, Vege SS, Sandborn WJ, Smyrk TC, Loftus EV. Inflammatory bowel disease in the setting of autoimmune pancreatitis. Inflamm Bowel Dis. 2009;15(9):1326–30.

    Article  PubMed  Google Scholar 

  28. Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, et al. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol. 2006;4(8):1010–6.

    Article  PubMed  Google Scholar 

  29. Boonstra K, Culver EL, de Buy Wenniger LM, van Heerde MJ, van Erpecum KJ, Poen AC, et al. Serum immunoglobulin G4 and immunoglobulin G1 for distinguishing immunoglobulin G4-associated cholangitis from primary sclerosing cholangitis. Hepatology. 2014;59(5):1954–63.

    Article  CAS  PubMed  Google Scholar 

  30. Doorenspleet ME, Hubers LM, Culver EL, Maillette de Buy Wenniger LJ, Klarenbeek PL, Chapman RW, et al. Immunoglobulin G4+ B-cell receptor clones distinguish immunoglobulin G4-related disease from primary sclerosing cholangitis and biliary/pancreatic malignancies. Hepatology. 2016;64(2):501–7.

    Article  CAS  PubMed  Google Scholar 

  31. Talwalkar JA, Angulo P, Johnson CD, Petersen BT, Lindor KD. Cost-minimization analysis of MRC versus ERCP for the diagnosis of primary sclerosing cholangitis. Hepatology. 2004;40(1):39–45.

    Article  PubMed  Google Scholar 

  32. Dave M, Elmunzer BJ, Dwamena BA, Higgins PDR. Primary sclerosing cholangitis: meta-analysis of diagnostic performance of MR cholangiopancreatography. Radiology. 2010;256(2):387–96.

    Article  PubMed  Google Scholar 

  33. Burak K, Angulo P, et al. Is there a role for liver biopsy in primary sclerosing cholangitis? Am J Gastroenterol. 2013;98(5):1155–8.

    Article  Google Scholar 

  34. Newsome PN, Cramb R, Davison SM, Dillon JF, Foulerton M, Godfrey EM, et al. Guidelines on the management of abnormal liver blood tests. Gut. 2018;67:6–19.

    Article  PubMed  Google Scholar 

  35. Albert J, Arvanitakis M, Chazouilleres O, Dumonceau J-M, Färkkilä M, Fickert P, et al. Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) clinical guideline. J Hepatol. 2017;66(6):1265–81.

    Article  Google Scholar 

  36. Broomé U, Olsson R, Lööf L, Bodemar G, Hultcrantz R, Danielsson Å, et al. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis. Gut. 1996;38(4):610–5.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Tischendorf JJW, Hecker H, Krüger 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.

    Article  PubMed  Google Scholar 

  38. Bergquist A, Ekbom A, Olsson R, et al. Hepatic and extrahepatic malignancies in primary sclerosing cholangitis. J Hepatol. 2002;36(3):321–7.

    Article  PubMed  Google Scholar 

  39. Ali AH, Tabibian JH, Nasser-Ghodsi N, Lennon RJ, DeLeon T, Borad MJ, et al. Surveillance for hepatobiliary cancers in patients with primary sclerosing cholangitis. Hepatology. 2018;67(6):2338–51.

    Article  CAS  PubMed  Google Scholar 

  40. Charatcharoenwitthaya P, Enders FB, Halling KC, Lindor KD. Utility of serum tumor markers, imaging, and biliary cytology for detecting cholangiocarcinoma in primary sclerosing cholangitis. Hepatology. 2008;48(4):1106–17.

    Article  CAS  PubMed  Google Scholar 

  41. Razumilava N, Gores GJ, Lindor KD. Cancer surveillance in patients with primary sclerosing cholangitis. Hepatology. 2011;54:1842–52.

    Article  PubMed  Google Scholar 

  42. Arnelo U, Von Seth E, Bergquist A. Prospective evaluation of the clinical utility of single-operator peroral cholangioscopy in patients with primary sclerosing cholangitis. Endoscopy. 2015;47(8):696–702.

    Article  PubMed  Google Scholar 

  43. Mendes F, Lindor KD. Primary sclerosing cholangitis: overview and update. Nat Rev Gastroenterol Hepatol. 2010;7:611–9.

    Article  PubMed  Google Scholar 

  44. Lindor KD, Kowdley KV, Harrison ME, American College of Gastroenterology. ACG clinical guideline: primary sclerosing cholangitis. Am J Gastroenterol. 2015;110(5):646–59.

    Article  CAS  PubMed  Google Scholar 

  45. Soetikno RM, Lin OS, Heidenreich PA, Young HS, Blackstone MO. Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis: a meta-analysis. Gastrointest Endosc. 2002;56(1):48–54.

    Article  PubMed  Google Scholar 

  46. Kamiński MF, Hassan C, Bisschops R, Pohl J, Pellisé M, Dekker E, et al. Advanced imaging for detection and differentiation of colorectal neoplasia: European Society of Gastrointestinal Endoscopy (ESGE) guideline. Endoscopy. 2014;46:435–49.

    Article  PubMed  Google Scholar 

  47. Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010;51:660–78.

    Article  CAS  PubMed  Google Scholar 

  48. Yahagi M, Okabayashi K, Hasegawa H, Tsuruta M, Kitagawa Y. The worse prognosis of right-sided compared with left-sided colon cancers: a systematic review and meta-analysis. J Gastrointest Surg. 2015;20(3):648–55.

    Article  PubMed  Google Scholar 

  49. Kim WR, Therneau TM, Wiesner RH, Poterucha JJ, Benson JT, Malinchoc M, et al. A revised natural history model for primary sclerosing cholangitis. Mayo Clin Proc. 2000;75(7):688–94.

    Article  CAS  PubMed  Google Scholar 

  50. De Vries EM, Wang J, Williamson KD, Leeflang MM, Boonstra K, Weersma RK, et al. A novel prognostic model for transplant-free survival in primary sclerosing cholangitis. Gut. 2018;67(10):1864–9.

    Article  PubMed  CAS  Google Scholar 

  51. Goet JC, Floreani A, Verhelst X, Cazzagon N, Perini L, Lammers WJ, et al. Validation, clinical utility and limitations of the Amsterdam-Oxford model for primary sclerosing cholangitis. J Hepatol. 2019;71(5):992–9.

    Article  PubMed  Google Scholar 

  52. Goode EC, Clark AB, Mells GF, Srivastava B, Spiess K, Gelson WTH, et al. Factors associated with outcomes of patients with primary sclerosing cholangitis and development and validation of a risk scoring system. Hepatology. 2019;69(5):2120–35.

    Article  CAS  PubMed  Google Scholar 

  53. Rupp C, Rössler A, Halibasic E, Sauer P, Weiss KH, Friedrich K, et al. Reduction in alkaline phosphatase is associated with longer survival in primary sclerosing cholangitis, independent of dominant stenosis. Aliment Pharmacol Ther. 2014;40(11–12):1292–301.

    Article  CAS  PubMed  Google Scholar 

  54. Lindström L, Hultcrantz R, Boberg KM, Friis-Liby I, Bergquist A. Association between reduced levels of alkaline phosphatase and survival times of patients with primary sclerosing cholangitis. Clin Gastroenterol Hepatol. 2013;11(7):841–6.

    Article  PubMed  CAS  Google Scholar 

  55. de Vries EMG, Wang J, Leeflang MMG, Boonstra K, Weersma RK, Beuers UH, et al. Alkaline phosphatase at diagnosis of primary sclerosing cholangitis and 1 year later: evaluation of prognostic value. Liver Int. 2016;36(12):1867–75.

    Article  PubMed  CAS  Google Scholar 

  56. Trivedi PJ, Muir AJ, Levy C, Bowlus CL, Manns M, Lu X, et al. Inter- and intra-individual variation and limited prognostic utility of serum alkaline phosphatase in a trial of patients with primary sclerosing cholangitis. Clin Gastroenterol Hepatol. 2020;22:S1542–3565(20)30994–0.

    Google Scholar 

  57. Vesterhus M, Hov JR, Holm A, Schrumpf E, Nygård S, Godang K, et al. Enhanced liver fibrosis score predicts transplant-free survival in primary sclerosing cholangitis. Hepatology. 2015;62(1):188–97.

    Article  PubMed  Google Scholar 

  58. Zenouzi R, Liwinski T, Yamamura J, Weiler-Normann C, Sebode M, Keller S, et al. Follow-up magnetic resonance imaging/3D-magnetic resonance cholangiopancreatography in patients with primary sclerosing cholangitis: challenging for experts to interpret. Aliment Pharmacol Ther. 2018;48(2):169–78.

    Article  CAS  PubMed  Google Scholar 

  59. Corpechot C, Gaouar F, El Naggar A, Kemgang A, Wendum D, Poupon R, et al. Baseline values and changes in liver stiffness measured by transient elastography are associated with severity of fibrosis and outcomes of patients with primary sclerosing cholangitis. Gastroenterology. 2014;146(4):970–979.e6.

    Article  PubMed  Google Scholar 

  60. Cazzagon N, Lemoinne S, El Mouhadi S, Trivedi PJ, Gaouar F, Kemgang A, et al. The complementary value of magnetic resonance imaging and vibration-controlled transient elastography for risk stratification in primary sclerosing cholangitis. Am J Gastroenterol. 2019;114(12):1878–85.

    Article  PubMed  Google Scholar 

  61. Bolier R, de Vries ES, Parés A, Helder J, Kemper EM, Zwinderman K, et al. Fibrates for the treatment of cholestatic itch (FITCH): study protocol for a randomized controlled trial. Trials. 2017;18(1):230.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  62. Poropat G, Giljaca V, Stimac D, Gluud C. Bile acids for primary sclerosing cholangitis. Cochrane Database Syst Rev. 2011;2011(1):CD003626.

    PubMed Central  Google Scholar 

  63. Wolf JM, Rybicki LA, Lashner BA. The impact of ursodeoxycholic acid on cancer, dysplasia and mortality in ulcerative colitis patients with primary sclerosing cholangitis. Aliment Pharmacol Ther. 2005;22(9):783–8.

    Article  CAS  PubMed  Google Scholar 

  64. Lindor KD, Kowdley KV, Luketic VAC, 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.

    Article  CAS  PubMed  Google Scholar 

  65. Boberg KM, Egeland T, Schrumpf E. Long-term effect of corticosteroid treatment in primary sclerosing cholangitis patients. Scand J Gastroenterol. 2003;38(9):991–5.

    Article  CAS  PubMed  Google Scholar 

  66. Angulo P, Jorgensen RA, Keach JC, Dickson ER, Smith C, Lindor KD. Oral budesonide in the treatment of patients with primary biliary cirrhosis with a suboptimal response to ursodeoxycholic acid. Hepatology. 2000;31(2):318–23.

    Article  CAS  PubMed  Google Scholar 

  67. Schramm C, Schirmacher P, Helmreich-Becker I, Gerken G, Zum Büschenfelde KHM, Lohse AW. Combined therapy with azathioprine, prednisolone, and ursodiol in patients with primary sclerosing cholangitis: a case series. Ann Intern Med. 1999;131(12):943–6.

    Article  CAS  PubMed  Google Scholar 

  68. Talwalkar JA, Gossard AA, Keach JC, Jorgensen RA, Petz JL, Lindor RNKD. Tacrolimus for the treatment of primary sclerosing cholangitis. Liver Int. 2007;27(4):451–3.

    Article  CAS  PubMed  Google Scholar 

  69. Knox TA, Kaplan MM. A double-blind controlled trial of oral-pulse methotrexate therapy in the treatment of primary sclerosing cholangitis. Gastroenterology. 1994;106(2):494–9.

    Article  CAS  PubMed  Google Scholar 

  70. Lankarani KB. Mycophenolate mofetil for the treatment of primary sclerosing cholangitis. Aliment Pharmacol Ther. 2005;21(10):1279–80.

    Article  CAS  PubMed  Google Scholar 

  71. Olsson R, Broomé U, Danielsson Å, Hägerstrand I, Järnerot G, Lööf L, et al. Colchicine treatment of primary sclerosing cholangitis. Gastroenterology. 1995;108(4):1199–203.

    Article  CAS  PubMed  Google Scholar 

  72. LaRusso NF, Wiesner RH, Ludwig J, MacCarty RL, Beaver SJ, Zinsmeister AR. Prospective trial of penicillamine in primary sclerosing cholangitis. Gastroenterology. 1988;95(4):1036–42.

    Article  CAS  PubMed  Google Scholar 

  73. Hommes DW, Erkelens W, Ponsioen C, Stokkers P, Rauws E, Van Der Spek M, et al. A double-blind, placebo-controlled, randomized study of infliximab in primary sclerosing cholangitis. J Clin Gastroenterol. 2008;42(5):522–6, [cited 2020 Aug 31].

    Article  CAS  PubMed  Google Scholar 

  74. Floreani A, Rizzotto ER, Ferrara F, Carderi I, Caroli D, Blasone L, et al. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. Am J Gastroenterol. 2005;100(7):1516–22.

    Article  PubMed  Google Scholar 

  75. Gregorio GV, Portmann B, Karani J, Harrison P, Donaldson PT, Vergani D, et al. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology. 2001;33(3):544–53.

    Article  CAS  PubMed  Google Scholar 

  76. Tabibian JH, Weeding E, Jorgensen RA, Petz JL, Keach JC, Talwalkar JA, et al. Randomised clinical trial: vancomycin or metronidazole in patients with primary sclerosing cholangitis—a pilot study. Aliment Pharmacol Ther. 2013;37(6):604–12.

    Article  CAS  PubMed  Google Scholar 

  77. Silveira M, Torok N, et al. Minocycline in the treatment of patients with primary sclerosing cholangitis: results of a pilot study. Am J Gastrol. 2009;104(1):83–8.

    Article  CAS  Google Scholar 

  78. Tabibian JH, Gossard A, El-Youssef M, Eaton JE, Petz J, Jorgensen R, et al. Prospective clinical trial of rifaximin therapy for patients with primary sclerosing cholangitis. Am J Ther. 2017;24(1):e56–63.

    Article  PubMed  PubMed Central  Google Scholar 

  79. Färkkilä M, Karvonen AL, Nurmi H, Nuutinen H, Taavitsainen M, Pikkarainen P, et al. Metronidazole and ursodeoxycholic acid for primary sclerosing cholangitis: a randomized placebo-controlled trial. Hepatology. 2004;40(6):1379–86.

    Article  PubMed  CAS  Google Scholar 

  80. Bjøro K, Brandsærter B, Foss A, Schrumpf E. Liver transplantation in primary sclerosing cholangitis, Seminars in liver disease, vol. 26. New York: Thieme Medical Publishers; 2006. p. 69–79.

    Google Scholar 

  81. Khungar V, Goldberg DS. Liver transplantation for cholestatic liver diseases in adults, Clinics in liver disease, vol. 20. Philadelphia: W.B. Saunders; 2016. p. 191–203.

    Google Scholar 

  82. Rea DJ, Heimbach JK, Rosen CB, Haddock MG, Alberts SR, Kremers WK, et al. Liver transplantation with neoadjuvant chemoradiation is more effective than resection for hilar cholangiocarcinoma. In: Annals of surgery. Philadelphia: Lippincott, Williams, and Wilkins; 2005. p. 451–61.

    Google Scholar 

  83. Darwish Murad S, Kim WR, Harnois DM, Douglas DD, Burton J, Kulik LM, et al. Efficacy of neoadjuvant chemoradiation, followed by liver transplantation, for perihilar cholangiocarcinoma at 12 US centers. Gastroenterology. 2012;143(1):88.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Gastroenterology, Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy

    Laura Cristoferi, Alessio Gerussi, Marco Carbone & Pietro Invernizzi

  2. European Reference Network on Hepatological Diseases (ERN RARE-LIVER), San Gerardo Hospital, Monza, Italy

    Laura Cristoferi, Alessio Gerussi, Marco Carbone & Pietro Invernizzi

  3. Bicocca Bioinformatics Biostatistics and Bioimaging Centre-B4, School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy

    Laura Cristoferi

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Correspondence to Laura Cristoferi .

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    Annarosa Floreani

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Cristoferi, L., Gerussi, A., Carbone, M., Invernizzi, P. (2021). Primary Sclerosing Cholangitis. In: Floreani, A. (eds) Diseases of the Liver and Biliary Tree. Springer, Cham. https://doi.org/10.1007/978-3-030-65908-0_9

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