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Primary Sclerosing Cholangitis

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Inflammation and Gastrointestinal Cancers

Part of the book series: Recent Results in Cancer Research ((RECENTCANCER,volume 185))

Abstract

Primary sclerosing cholangitis (PSC) is a chronic liver condition which may affect both intra and extrahepatic biliary tree. Etiology of PSC remains to be fully elucidated but genetic, autoimmune, inflammatory and possibly infective factors could all contribute to its development. More than two-thirds of patients are males and the most commonly associated condition is an inflammatory bowel disease which occurs in up to 70% of affected subjects. Endoscopic cholangiopancreatography (ERCP) and magnetic resonanse cholangiopancreatography (MRCP) remain a gold standard in the diagnosis of this condition. No curative treatment of PSC exists and a proportion of patients who develop liver failure or suffer from recurrent episodes of cholangitis requires liver transplantation. PSC is associated with increased risk of malignancies, in particular cholangiocarcinoma which may arise in 12% of patients. The main aim of this chapter is to review the current knowledge on pathogenesis and clinical aspects of PSC as well as its associated malignancies.

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References

  • Abdalian R, Dhar P, Jhaveri K, Haider M, Guindi M, Heathcote EJ (2008) Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: evaluating the role of routine magnetic resonance imaging. Hepatology 47(3):949–957

    Article  PubMed  CAS  Google Scholar 

  • Alabraba E, Nightingale P, Gunson B et al (2009) A re-evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts. Liver Transpl 15(3):330–340

    Article  PubMed  Google Scholar 

  • Al-Harthy N, Kumagi T, Parasar S, Coltescu C, Heathcote EJ, Hirschfield GM (2009) High prevalence of fatigue in patients with sclerosing cholangitis. J Hepatol 50:S241

    Google Scholar 

  • Angulo P, Peter JB, Gershwin ME et al (2000) Serum autoantibodies in patients with primary sclerosing cholangitis. J Hepatol 32(2):182–187

    Article  PubMed  CAS  Google Scholar 

  • Baluyut AR, Sherman S, Lehman GA, Hoen H, Chalasani N (2001) Impact of endoscopic therapy on the survival of patients with primary sclerosing cholangitis. Gastrointest Endosc 53(3):308–312

    PubMed  CAS  Google Scholar 

  • Bambha K, Kim WR, Talwalkar J et al (2003) Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community. Gastroenterology 125(5):1364–1369

    Article  PubMed  Google Scholar 

  • Bangarulingam SY, Gossard AA, Petersen BT, Ott BJ, Lindor KD (2009) Complications of endoscopic retrograde cholangiopancreatography in primary sclerosing cholangitis. Am J Gastroenterol 104(4):855–860

    Article  PubMed  Google Scholar 

  • Bergquist A, Ekbom A, Olsson R et al (2002) Hepatic and extrahepatic malignancies in primary sclerosing cholangitis. J Hepatol 36(3):321–327

    Article  PubMed  Google Scholar 

  • Bergquist A, Lindberg G, Saarinen S, Broome U (2005) Increased prevalence of primary sclerosing cholangitis among first-degree relatives. J Hepatol 42(2):252–256

    Article  PubMed  Google Scholar 

  • Bergquist A, Montgomery SM, Bahmanyar S et al (2008) Increased risk of primary sclerosing cholangitis and ulcerative colitis in first-degree relatives of patients with primary sclerosing cholangitis. Clin Gastroenterol Hepatol 6(8):939–943

    Article  PubMed  Google Scholar 

  • Bernstein H, Bernstein C, Payne CM, Dvorakova K, Garewal H (2005) Bile acids as carcinogens in human gastrointestinal cancers. Mutat Res 589(1):47–65

    Article  PubMed  CAS  Google Scholar 

  • Beuers U, Bilzer M, Chittattu A et al (2001) Tauroursodeoxycholic acid inserts the apical conjugate export pump, Mrp2, into canalicular membranes and stimulates organic anion secretion by protein kinase C-dependent mechanisms in cholestatic rat liver. Hepatology 33(5):1206–1216

    Article  PubMed  CAS  Google Scholar 

  • Beuers U, Kullak-Ublick GA, Pusl T, Rauws ER, Rust C (2009) Medical treatment of primary sclerosing cholangitis: a role for novel bile acids and other (post-)transcriptional modulators? Clin Rev Allergy Immunol 36(1):52–61

    Article  PubMed  CAS  Google Scholar 

  • Bjornsson E, Olsson R (2004) Dominant strictures in patients with primary sclerosing cholangitis-revisited. Am J Gastroenterol 99(11):2281

    Article  PubMed  Google Scholar 

  • Bjornsson E, Boberg KM, Cullen S et al (2002) Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis. Gut 51(5):731–735

    Article  PubMed  CAS  Google Scholar 

  • Bjornsson E, Simren M, Olsson R, Chapman RW (2004a) Fatigue in patients with primary sclerosing cholangitis. Scand J Gastroenterol 39(10):961–968

    Article  PubMed  CAS  Google Scholar 

  • Bjornsson E, Lindqvist-Ottosson J, Asztely M, Olsson R (2004b) Dominant strictures in patients with primary sclerosing cholangitis. Am J Gastroenterol 99(3):502–508

    Article  PubMed  Google Scholar 

  • Bjornsson E, Olsson R, Bergquist A et al (2008) The natural history of small-duct primary sclerosing cholangitis. Gastroenterology 134(4):975–980

    Article  PubMed  Google Scholar 

  • Blechacz B, Gores GJ (2008) Cholangiocarcinoma: advances in pathogenesis, diagnosis, and treatment. Hepatology 48(1):308–321

    Article  PubMed  CAS  Google Scholar 

  • Boberg KM, Aadland E, Jahnsen J, Raknerud N, Stiris M, Bell H (1998) Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol 33(1):99–103

    Article  PubMed  CAS  Google Scholar 

  • Boberg KM, Bergquist A, Mitchell S et al (2002) Cholangiocarcinoma in primary sclerosing cholangitis: risk factors and clinical presentation. Scand J Gastroenterol 37(10):1205–1211

    Article  PubMed  CAS  Google Scholar 

  • Boberg KM, Egeland T, Schrumpf E (2003) Long-term effect of corticosteroid treatment in primary sclerosing cholangitis patients. Scand J Gastroenterol 38(9):991–995

    Article  PubMed  CAS  Google Scholar 

  • Brandsaeter B, Isoniemi H, Broome U et al (2004) Liver transplantation for primary sclerosing cholangitis; predictors and consequences of hepatobiliary malignancy. J Hepatol 40(5):815–822

    Article  PubMed  CAS  Google Scholar 

  • Broome U, Lofberg R, Veress B, Eriksson LS (1995) Primary sclerosing cholangitis and ulcerative colitis: evidence for increased neoplastic potential. Hepatology 22(5):1404–1408

    PubMed  CAS  Google Scholar 

  • Buckles DC, Lindor KD, LaRusso NF, Petrovic LM, Gores GJ (2002) In primary sclerosing cholangitis, gallbladder polyps are frequently malignant. Am J Gastroenterol 97(5):1138–1142

    Article  PubMed  Google Scholar 

  • Burak KW, Angulo P, Lindor KD (2003) Is there a role for liver biopsy in primary sclerosing cholangitis? Am J Gastroenterol 98(5):1155–1158

    Article  PubMed  Google Scholar 

  • Card TR, Solaymani-Dodaran M, West J (2008) Incidence and mortality of primary sclerosing cholangitis in the UK: a population-based cohort study. J Hepatol 48(6):939–944

    Article  PubMed  Google Scholar 

  • Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ (2010) Diagnosis and management of primary sclerosing cholangitis. Hepatology 51:660–678

    PubMed  CAS  Google Scholar 

  • Chapman RW (2009) High-dose ursodeoxycholic acid in the treatment of primary sclerosing cholangitis: throwing the urso out with the bathwater? Hepatology 50:671–673

    Article  PubMed  CAS  Google Scholar 

  • Chen L, Borozan I, Milkiewicz P et al (2008) Gene expression profiling of early primary biliary cirrhosis: possible insights into the mechanism of action of ursodeoxycholic acid. Liver Int 28(7):997–1010

    Article  PubMed  CAS  Google Scholar 

  • Claessen MM, Vleggaar FP, Tytgat KM, Siersema PD, van Buuren HR (2009a) High lifetime risk of cancer in primary sclerosing cholangitis. J Hepatol 50(1):158–164

    Article  PubMed  Google Scholar 

  • Claessen MM, Lutgens MW, van Buuren HR et al. (2009b) More right-sided IBD-associated colorectal cancer in patients with primary sclerosing cholangitis. Inflamm Bowel Dis 15(9):1331–1336.

    Google Scholar 

  • Cullen SN, Chapman RW (2006) The medical management of primary sclerosing cholangitis. Semin Liver Dis 26(1):52–61

    Article  PubMed  CAS  Google Scholar 

  • Cullen SN, Rust C, Fleming K, Edwards C, Beuers U, Chapman RW (2008) High dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis is safe and effective. J Hepatol 48(5):792–800

    Article  PubMed  CAS  Google Scholar 

  • EASL (2009) Clinical Practice Guidelines: Management of cholistatic liver diseases. J Hepatol 51:2009 237–267

    Google Scholar 

  • Endo K, Yoon BI, Pairojkul C, Demetris AJ, Sirica AE (2002) ERBB-2 overexpression and cyclooxygenase-2 up-regulation in human cholangiocarcinoma and risk conditions. Hepatology 36(2):439–450

    Article  PubMed  CAS  Google Scholar 

  • European liver transplant registry 2009. http://www.eltr.org

  • Farkkila M, Karvonen AL, Nurmi H et al (2004) Metronidazole and ursodeoxycholic acid for primary sclerosing cholangitis: a randomized placebo-controlled trial. Hepatology 40(6):1379–1386

    Article  PubMed  Google Scholar 

  • Fava G, Marucci L, Glaser S et al (2005) Gamma-aminobutyric acid inhibits cholangiocarcinoma growth by cyclic AMP-dependent regulation of the protein kinase A/extracellular signal-regulated kinase 1/2 pathway. Cancer Res 65(24):11437–11446

    Article  PubMed  CAS  Google Scholar 

  • Fox JG, Dewhirst FE, Shen Z et al (1998) Hepatic Helicobacter species identified in bile and gallbladder tissue from Chileans with chronic cholecystitis. Gastroenterology 114(4):755–763

    Article  PubMed  CAS  Google Scholar 

  • Gluck M, Cantone NR, Brandabur JJ, Patterson DJ, Bredfeldt JE, Kozarek RA (2008) A twenty-year experience with endoscopic therapy for symptomatic primary sclerosing cholangitis. J Clin Gastroenterol 42(9):1032–1039

    Article  PubMed  Google Scholar 

  • Grant AJ, Lalor PF, Hubscher SG, Briskin M, Adams DH (2001) MAdCAM-1 expressed in chronic inflammatory liver disease supports mucosal lymphocyte adhesion to hepatic endothelium (MAdCAM-1 in chronic inflammatory liver disease). Hepatology 33(5):1065–1072

    Article  PubMed  CAS  Google Scholar 

  • Grant AJ, Lalor PF, Salmi M, Jalkanen S, Adams DH (2002) Homing of mucosal lymphocytes to the liver in the pathogenesis of hepatic complications of inflammatory bowel disease. Lancet 359(9301):150–157

    Article  PubMed  Google Scholar 

  • Gregorio GV, Portmann B, Karani J et al (2001) Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16 year prospective study. Hepatology 33(3):544–553

    Article  PubMed  CAS  Google Scholar 

  • Han C, Leng J, Demetris AJ, Wu T (2004) Cyclooxygenase-2 promotes human cholangiocarcinoma growth: evidence for cyclooxygenase-2-independent mechanism in celecoxib-mediated induction of p21waf1/cip1 and p27kip1 and cell cycle arrest. Cancer Res 64(4):1369–1376

    Article  PubMed  CAS  Google Scholar 

  • Harnois DM, Angulo P, Jorgensen RA, LaRusso NF, Lindor KD (2001) High-dose ursodeoxycholic acid as a therapy for patients with primary sclerosing cholangitis. Am J Gastroenterol 96(5):1558–1562

    Article  PubMed  CAS  Google Scholar 

  • Isomoto H, Mott JL, Kobayashi S et al (2007) Sustained IL-6/STAT-3 signaling in cholangiocarcinoma cells due to SOCS-3 epigenetic silencing. Gastroenterology 132(1):384–396

    Article  PubMed  CAS  Google Scholar 

  • Joo M, Abreu-e-Lima P, Farraye F et al (2009) Pathologic features of ulcerative colitis in patients with primary sclerosing cholangitis: a case-control study. Am J Surg Pathol 33(6):854–862

    Article  PubMed  Google Scholar 

  • Kahana DD, Cass O, Jessurun J, Schwarzenberg SJ, Sharp H, Khan K (2003) Sclerosing cholangitis associated with trichosporon infection and natural killer cell deficiency in an 8-year-old girl. J Pediatr Gastroenterol Nutr 37(5):620–623

    Article  PubMed  Google Scholar 

  • Kanno N, Lesage G, Phinizy JL, Glaser S, Francis H, Alpini G (2002) Stimulation of alpha 2-adrenergic receptor inhibits cholangiocarcinoma growth through modulation of Raf-1 and B-Raf activities. Hepatology 35(6):1329–1340

    Article  PubMed  CAS  Google Scholar 

  • Kaplan GG, Laupland KB, Butzner D, Urbanski SJ, Lee SS (2007) The burden of large and small duct primary sclerosing cholangitis in adults and children: a population-based analysis. Am J Gastroenterol 102(5):1042–1049

    Article  PubMed  Google Scholar 

  • Khan SA, Toledano MB, Taylor-Robinson SD (2008) Epidemiology, risk factors, and pathogenesis of cholangiocarcinoma. HPB (Oxford) 10(2):77–82

    CAS  Google Scholar 

  • Kitiyakara T, Chapman RW (2008) Chemoprevention and screening in primary sclerosing cholangitis. Postgrad Med J 84(991):228–237

    Article  PubMed  CAS  Google Scholar 

  • Kobayashi S, Werneburg NW, Bronk SF, Kaufmann SH, Gores GJ (2005) Interleukin-6 contributes to Mcl-1 up-regulation and TRAIL resistance via an Akt-signaling pathway in cholangiocarcinoma cells. Gastroenterology 128(7):2054–2065

    Article  PubMed  CAS  Google Scholar 

  • Kono K, Ohnishi K, Omata M et al (1988) Experimental portal fibrosis produced by intraportal injection of killed nonpathogenic Escherichia coli in rabbits. Gastroenterology 94(3):787–796

    PubMed  CAS  Google Scholar 

  • Kulaksiz H, Rudolph G, Kloeters-Plachky P, Sauer P, Geiss H, Stiehl A (2006) Biliary candida infections in primary sclerosing cholangitis. J Hepatol 45(5):711–716

    Article  PubMed  Google Scholar 

  • Lee J, Belanger A, Doucette JT, Stanca C, Friedman S, Bach N (2007) Transplantation trends in primary biliary cirrhosis. Clin Gastroenterol Hepatol 5(11):1313–1315

    Article  PubMed  Google Scholar 

  • Lewis JT, Talwalkar JA, Rosen CB, Smyrk TC, Abraham SC (2007) Prevalence and risk factors for gallbladder neoplasia in patients with primary sclerosing cholangitis: evidence for a metaplasia-dysplasia-carcinoma sequence. Am J Surg Pathol 31(6):907–913

    Article  PubMed  Google Scholar 

  • Lichtman SN, Sartor RB, Keku J, Schwab JH (1990) Hepatic inflammation in rats with experimental small intestinal bacterial overgrowth. Gastroenterology 98(2):414–423

    PubMed  CAS  Google Scholar 

  • Lindor KD, Kowdley KV, Luketic VA, Harrison ME, McCashland T, Befeler AS, Harnois D, Jorgensen R, Petz J, Keach J, Mooney J, Sargeant C, Braaten J, Bernard T, King D, Miceli E, Schmoll J, Hoskin T, Thapa P, Enders F (2009) High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology 50:808–814

    Article  PubMed  CAS  Google Scholar 

  • Milkiewicz P, Heathcote EJ (2004) Fatigue in chronic cholestasis. Gut 53(4):475–477

    Article  PubMed  CAS  Google Scholar 

  • Milkiewicz P, Mills CO, Roma MG, Ahmed-Choudhury J, Elias E, Coleman R (1999) Tauroursodeoxycholate and S-adenosyl-l-methionine exert an additive ameliorating effect on taurolithocholate-induced cholestasis: a study in isolated rat hepatocyte couplets. Hepatology 29(2):471–476

    Article  PubMed  CAS  Google Scholar 

  • Milkiewicz P, Roma MG, Cardenas R, Mills CO, Elias E, Coleman R (2001) Effect of tauroursodeoxycholate and S-adenosyl-l-methionine on 17 beta-estradiol glucuronide-induced cholestasis. J Hepatol 34(2):184–191

    Article  PubMed  CAS  Google Scholar 

  • Milkiewicz P, Roma MG, Elias E, Coleman R (2002a) Pathobiology and experimental therapeutics in hepatocellular cholestasis: lessons from the hepatocyte couplet model. Clin Sci (Lond) 102(6):603–614

    Article  CAS  Google Scholar 

  • Milkiewicz P, Roma MG, Elias E, Coleman R (2002b) Hepatoprotection with tauroursodeoxycholate and beta muricholate against taurolithocholate induced cholestasis: involvement of signal transduction pathways. Gut 51(1):113–119

    Article  PubMed  CAS  Google Scholar 

  • Norris S, Kondeatis E, Collins R et al (2001) Mapping MHC-encoded susceptibility and resistance in primary sclerosing cholangitis: the role of MICA polymorphism. Gastroenterology 120(6):1475–1482

    Article  PubMed  CAS  Google Scholar 

  • O’Mahony CA, Vierling JM (2006) Etiopathogenesis of primary sclerosing cholangitis. Semin Liver Dis 26(1):3–21

    Article  PubMed  Google Scholar 

  • Okuda K, Nakanuma Y, Miyazaki M (2002) Cholangiocarcinoma: recent progress. Part 1: epidemiology and etiology. J Gastroenterol Hepatol 17(10):1049–1055

    Article  PubMed  Google Scholar 

  • Olsson R, Hagerstrand I, Broome U et al (1995) Sampling variability of percutaneous liver biopsy in primary sclerosing cholangitis. J Clin Pathol 48(10):933–935

    Article  PubMed  CAS  Google Scholar 

  • Olsson R, Bjornsson E, Backman L et al (1998) Bile duct bacterial isolates in primary sclerosing cholangitis: a study of explanted livers. J Hepatol 28(3):426–432

    Article  PubMed  CAS  Google Scholar 

  • Olsson R, Boberg KM, de Muckadell OS et al (2005) High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5 year multicenter, randomized, controlled study. Gastroenterology 129(5):1464–1472

    Article  PubMed  CAS  Google Scholar 

  • Pardi DS, Loftus EV Jr, Kremers WK, Keach J, Lindor KD (2003) Ursodeoxycholic acid as a chemopreventive agent in patients with ulcerative colitis and primary sclerosing cholangitis. Gastroenterology 124(4):889–893

    Article  PubMed  CAS  Google Scholar 

  • Park J, Tadlock L, Gores GJ, Patel T (1999) Inhibition of interleukin 6-mediated mitogen-activated protein kinase activation attenuates growth of a cholangiocarcinoma cell line. Hepatology 30(5):1128–1133

    Article  PubMed  CAS  Google Scholar 

  • Paumgartner G, Beuers U (2004) Mechanisms of action and therapeutic efficacy of ursodeoxycholic acid in cholestatic liver disease. Clin Liver Dis 8(1):67–81 vi.

    Article  PubMed  Google Scholar 

  • Rea DJ, Heimbach JK, Rosen CB et al (2005) Liver transplantation with neoadjuvant chemoradiation is more effective than resection for hilar cholangiocarcinoma. Ann Surg 242(3):451–458

    PubMed  Google Scholar 

  • Rosignoli P, Fabiani R, De BA, Fuccelli R, Pelli MA, Morozzi G (2008) Genotoxic effect of bile acids on human normal and tumour colon cells and protection by dietary antioxidants and butyrate. Eur J Nutr 47(6):301–309

    Article  PubMed  CAS  Google Scholar 

  • Rudolph G, Kloeters-Plachky P, Rost D, Stiehl A (2007) The incidence of cholangiocarcinoma in primary sclerosing cholangitis after long-time treatment with ursodeoxycholic acid. Eur J Gastroenterol Hepatol 19(6):487–491

    Article  PubMed  CAS  Google Scholar 

  • Rudolph G, Gotthardt D, Kloters-Plachky P, Kulaksiz H, Rost D, Stiehl A (2009) Influence of dominant bile duct stenoses and biliary infections on outcome in primary sclerosing cholangitis. J Hepatol 51(1):149–155

    Article  PubMed  Google Scholar 

  • Said K, Glaumann H, Bergquist A (2008) Gallbladder disease in patients with primary sclerosing cholangitis. J Hepatol 48(4):598–605

    PubMed  Google Scholar 

  • Sampson LK, Vickers SM, Ying W, Phillips JO (1997) Tamoxifen-mediated growth inhibition of human cholangiocarcinoma. Cancer Res 57(9):1743–1749

    PubMed  CAS  Google Scholar 

  • Selmi C, Balkwill DL, Invernizzi P et al (2003) Patients with primary biliary cirrhosis react against a ubiquitous xenobiotic-metabolizing bacterium. Hepatology 38(5):1250–1257

    Article  PubMed  CAS  Google Scholar 

  • Serfaty L, De LA, Rosmorduc O et al (2003) Ursodeoxycholic acid therapy and the risk of colorectal adenoma in patients with primary biliary cirrhosis: an observational study. Hepatology 38(1):203–209

    Article  PubMed  CAS  Google Scholar 

  • Shaib Y, El-Serag HB (2004) The epidemiology of cholangiocarcinoma. Semin Liver Dis 24(2):115–125

    Article  PubMed  Google Scholar 

  • Silveira MG, Torok NJ, Gossard AA et al (2009) Minocycline in the treatment of patients with primary sclerosing cholangitis: results of a pilot study. Am J Gastroenterol 104(1):83–88

    Article  PubMed  CAS  Google Scholar 

  • Sokol H, Cosnes J, Chazouilleres O et al (2008) Disease activity and cancer risk in inflammatory bowel disease associated with primary sclerosing cholangitis. World J Gastroenterol 14(22):3497–3503

    Article  PubMed  Google Scholar 

  • Spurkland A, Saarinen S, Boberg KM et al (1999) HLA class II haplotypes in primary sclerosing cholangitis patients from five European populations. Tissue Antigens 53(5):459–469

    Article  PubMed  CAS  Google Scholar 

  • Terjung B, Spengler U (2005) Role of auto-antibodies for the diagnosis of chronic cholestatic liver diseases. Clin Rev Allergy Immunol 28(2):115–133

    Article  PubMed  CAS  Google Scholar 

  • Terjung B, Spengler U (2009) Atypical p-ANCA in PSC and AIH: a hint toward a “leaky gut”? Clin Rev Allergy Immunol 36(1):40–51

    Article  PubMed  CAS  Google Scholar 

  • Tung BY, Emond MJ, Haggitt RC et al (2001) Ursodiol use is associated with lower prevalence of colonic neoplasia in patients with ulcerative colitis and primary sclerosing cholangitis. Ann Intern Med 134(2):89–95

    PubMed  CAS  Google Scholar 

  • Vera A, Moledina S, Gunson B et al (2002) Risk factors for recurrence of primary sclerosing cholangitis of liver allograft. Lancet 360(9349):1943–1944

    Article  PubMed  Google Scholar 

  • Vera A, Gunson BK, Ussatoff V et al (2003) Colorectal cancer in patients with inflammatory bowel disease after liver transplantation for primary sclerosing cholangitis. Transplantation 75(12):1983–1988

    Article  PubMed  Google Scholar 

  • Watanapa P (1996) Cholangiocarcinoma in patients with opisthorchiasis. Br J Surg 83(8):1062–1064

    Article  PubMed  CAS  Google Scholar 

  • Weismuller TJ, Wedemeyer J, Kubicka S, Strassburg CP, Manns MP (2008) The challenges in primary sclerosing cholangitis–aetiopathogenesis, autoimmunity, management and malignancy. J Hepatol 48(Suppl 1):S38–S57

    PubMed  Google Scholar 

  • Werneburg NW, Yoon JH, Higuchi H, Gores GJ (2003) Bile acids activate EGF receptor via a TGF-alpha-dependent mechanism in human cholangiocyte cell lines. Am J Physiol Gastrointest Liver Physiol 285(1):G31–G36

    PubMed  CAS  Google Scholar 

  • Wimmer R, Hohenester S, Pusl T, Denk GU, Rust C, Beuers U (2008) Tauroursodeoxycholic acid exerts anticholestatic effects by a cooperative cPKC alpha-/PKA-dependent mechanism in rat liver. Gut 57(10):1448–1454

    Article  PubMed  CAS  Google Scholar 

  • Wise C, Pilanthananond M, Perry BF, Alpini G, McNeal M, Glaser SS (2008) Mechanisms of biliary carcinogenesis and growth. World J Gastroenterol 14(19):2986–2989

    Article  PubMed  CAS  Google Scholar 

  • Wolf JM, Rybicki LA, Lashner BA (2005) The impact of ursodeoxycholic acid on cancer, dysplasia and mortality in ulcerative colitis patients with primary sclerosing cholangitis. Aliment Pharmacol Ther 22(9):783–788

    Article  PubMed  CAS  Google Scholar 

  • Yokomuro S, Lunz JG III, Sakamoto T, Ezure T, Murase N, Demetris AJ (2000) The effect of interleukin-6 (IL-6)/gp 130 signalling on biliary epithelial cell growth, in vitro. Cytokine 12(6):727–730

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Hepatology and Liver Transplantation, M.Curie Hospital, Arkonska 4, 71-455, Szczecin, Poland

    Piotr Milkiewicz & Ewa Wunsch

  2. Liver Research Laboratories, Pomeranian Medical School, Powstancow Wlkp. 72, 70-111, Szczecin, Poland

    Piotr Milkiewicz

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Correspondence to Piotr Milkiewicz .

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  1. Leicester Royal Infirmary, Dept. Cancer Studies and, University of Leicester, Windsor Building, Level 4, Leicester, LE1 5WW, United Kingdom

    Janusz A. Z. Jankowski

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Milkiewicz, P., Wunsch, E. (2011). Primary Sclerosing Cholangitis. In: Jankowski, J. (eds) Inflammation and Gastrointestinal Cancers. Recent Results in Cancer Research, vol 185. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-03503-6_7

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