Primary sclerosing cholangitis

The Lancet

Volumn 382, Issue 9904, 2013, Pages 1587-1599

  Hirschfield, Gideon M ^a   Karlsen, Tom H ^b,c   Lindor, Keith D ^d   Adams, David H ^a  

^a UNIVERSITY OF BIRMINGHAM   (United Kingdom)

^b OSLO UNIVERSITY HOSPITAL   (Norway)

^c UNIVERSITY OF BERGEN   (Norway)

^d ARIZONA STATE UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

24 NORURSODEOXYCHOLIC ACID; AMOXICILLIN PLUS CLAVULANIC ACID; ANTIBIOTIC AGENT; ANTIINFLAMMATORY AGENT; AZATHIOPRINE; AZITHROMYCIN; BILE ACID; BUDESONIDE; CALCINEURIN INHIBITOR; CEFALEXIN; CEFPODOXIME; CIPROFLOXACIN; COLESTYRAMINE; COTRIMOXAZOLE; DOCOSAHEXAENOIC ACID; IMMUNOSUPPRESSIVE AGENT; LITHOCHOLIC ACID; METRONIDAZOLE; MINOCYCLINE; MONOCLONAL ANTIBODY; NALTREXONE; OBETICHOLIC ACID; PLACEBO; PROBIOTIC AGENT; RIFAMPICIN; SERTRALINE; UNCLASSIFIED DRUG; URSODEOXYCHOLIC ACID; VANCOMYCIN; VEDOLIZUMAB;

ABSENCE OF SIDE EFFECTS; ALBUMIN DIALYSIS; AUTOIMMUNE PANCREATITIS; B LYMPHOCYTE; BILE DUCT CARCINOMA; BILIARY CIRRHOSIS; BIOCHEMISTRY; BIOLOGY; CANCER RISK; CHOLANGIOGRAPHY; CHOLANGITIS; CHOLESTASIS; CLINICAL FEATURE; COLON RESECTION; COLORECTAL CANCER; CONFERENCE PAPER; DISEASE ASSOCIATION; DISEASE COURSE; DRUG MEGADOSE; END STAGE LIVER DISEASE; ENTERITIS; ENVIRONMENTAL FACTOR; FATIGUE; FIBROSIS; GENETICS; HEPATOBILIARY SYSTEM CANCER; HUMAN; IMMUNOSUPPRESSIVE TREATMENT; INCIDENCE; INFECTION; LIVER CELL CARCINOMA; LIVER TRANSPLANTATION; LYMPHOCYTE; LYMPHOID TISSUE; MACROPHAGE; MEMORY T LYMPHOCYTE; NATURAL KILLER CELL; NATURAL KILLER T CELL; NONHUMAN; NUCLEAR MAGNETIC RESONANCE IMAGING; OSTEOLYSIS; PATHOGENESIS; PATHOPHYSIOLOGY; PLASMAPHERESIS; PRIMARY SCLEROSING CHOLANGITIS; PRIORITY JOURNAL; PROGNOSIS; PRURITUS; RECURRENT DISEASE; RISK; SEPSIS; SIDE EFFECT; T LYMPHOCYTE; ULCERATIVE COLITIS; ULTRAVIOLET RADIATION;

EID: 84887254519     PISSN: 01406736     EISSN: 1474547X     Source Type: Journal    
DOI: 10.1016/S0140-6736(13)60096-3     Document Type: Conference Paper

References (141)

1. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol 2009; 51: 237-67.
2. Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology 2010; 51: 660-78.
3. Ngu JH, Gearry RB, Frampton CM, Stedman CA. Mortality and the risk of malignancy in autoimmune liver diseases: a population-based study in Canterbury, New Zealand. Hepatology 2012; 55: 522-29.
4. Lindkvist B, Benito de Valle M, Gullberg B, Bjornsson E. Incidence and prevalence of primary sclerosing cholangitis in a defined adult population in Sweden. Hepatology 2010; 52: 571-77.
5. Molodecky NA, Kareemi H, Parab R, et al. Incidence of primary sclerosing cholangitis: a systematic review and meta-analysis. Hepatology 2011; 53: 1590-99.
6. Boonstra K, Beuers U, Ponsioen CY. Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review. J Hepatol 2012; 56: 1181-88.
7. Boonstra K, van Erpecum KJ, van Nieuwkerk KM, et al. Primary sclerosing cholangitis is associated with a distinct phenotype of inflammatory bowel disease. Inflamm Bowel Dis 2012; 18: 2270-76.
8. Broome U, Olsson R, Loof L, et al. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis. Gut 1996; 38: 610-15.
9. 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: 107-14.
10. Claessen MM, Vleggaar FP, Tytgat KM, Siersema PD, van Buuren HR. High lifetime risk of cancer in primary sclerosing cholangitis. J Hepatol 2009; 50: 158-64.
11. Portmann B, Zen Y. Inflammatory disease of the bile ducts- cholangiopathies: liver biopsy challenge and clinicopathological correlation. Histopathology 2012; 60: 236-48.
12. Syal G, Fausther M, Dranoff JA. Advances in cholangiocyte immunobiology. Am J Physiol Gastrointest Liver Physiol 2012; 303: G1077-86.
13. Burak K, Angulo P, Pasha TM, Egan K, Petz J, Lindor KD. Incidence and risk factors for cholangiocarcinoma in primary sclerosing cholangitis. Am J Gastroenterol 2004; 99: 523-26.
14. Karlsen TH, Franke A, Melum E, et al. Genome-wide association analysis in primary sclerosing cholangitis. Gastroenterology 2010; 138: 1102-11.
15. Melum E, Franke A, Schramm C, et al. Genome-wide association analysis in primary sclerosing cholangitis identifies two non-HLA susceptibility loci. Nat Genet 2011; 43: 17-19.
16. Janse M, Lamberts LE, Franke L, et al. Three ulcerative colitis susceptibility loci are associated with primary sclerosing cholangitis and indicate a role for IL2, REL, and CARD9. Hepatology 2011; 53: 1977-85.
17. Folseraas T, Melum E, Rausch P, et al. Extended analysis of a genome-wide association study in primary sclerosing cholangitis detects multiple novel risk loci. J Hepatol 2012; 57: 366-75.
18. Ellinghaus D, Folseraas T, Holm K, et al. Genome-wide association analysis in sclerosing cholangitis and ulcerative colitis identifies risk loci at GPR35 and TCF4. Hepatology 2012; published online July 23. DOI:10.1002/hep.25977.
19. Liu JZ, Hov JE, Folseraas T, et al. Dense genotyping of immune-related disease regions identifies nine new risk loci for primary sclerosing cholangitis. Nat Genet 2013; published online April 21. DOI:10.1038/ng.2616.
20. Hov JR, Kosmoliaptsis V, Traherne JA, et al. Electrostatic modifications of the human leukocyte antigen-DR P9 peptide-binding pocket and susceptibility to primary sclerosing cholangitis. Hepatology 2011; 53: 1967-76.
21. Hov JR, Keitel V, Laerdahl JK, et al. Mutational characterization of the bile acid receptor TGR5 in primary sclerosing cholangitis. PLoS One 2010; 5: e12403.
22. Fickert P, Fuchsbichler A, Wagner M, et al. Regurgitation of bile acids from leaky bile ducts causes sclerosing cholangitis in Mdr2 (Abcb4) knockout mice. Gastroenterology 2004; 127: 261-74.
23. Fickert P, Wagner M, Marschall HU, et al. 24-norUrsodeoxycholic acid is superior to ursodeoxycholic acid in the treatment of sclerosing cholangitis in Mdr2 (Abcb4) knockout mice. Gastroenterology 2006; 130: 465-81.
24. Halilbasic E, Fiorotto R, Fickert P, et al. Side chain structure determines unique physiologic and therapeutic properties of norursodeoxycholic acid in Mdr2-/- mice. Hepatology 2009; 49: 1972-81.
25. Baghdasaryan A, Claudel T, Gumhold J, et al. Dual farnesoid X receptor/TGR5 agonist INT-767 reduces liver injury in the Mdr2-/- (Abcb4-/-) mouse cholangiopathy model by promoting biliary HCO(-)(3) output. Hepatology 2011; 54: 1303-12.
26. Martin CR, Zaman MM, Ketwaroo GA, et al. CFTR dysfunction predisposes to fibrotic liver disease in a murine model. Am J Physiol Gastrointest Liver Physiol 2012; 303: G474-81.
27. Borchers AT, Shimoda S, Bowlus C, Keen CL, Gershwin ME. Lymphocyte recruitment and homing to the liver in primary biliary cirrhosis and primary sclerosing cholangitis. Semin Immunopathol 2009; 31: 309-22.
28. Grant AJ, Goddard S, Ahmed-Choudhury J, et al. Hepatic expression of secondary lymphoid chemokine (CCL21) promotes the development of portal-associated lymphoid tissue in chronic inflammatory liver disease. Am J Pathol 2002; 160: 1445-55.
29. Grant AJ, Lalor PF, Salmi M, Jalkanen S, Adams DH. Homing of mucosal lymphocytes to the liver in the pathogenesis of hepatic complications of inflammatory bowel disease. Lancet 2002; 359: 150-57.
30. Grant AJ, Lalor PF, Hubscher SG, Briskin M, Adams DH. MAdCAM-1 expressed in chronic inflammatory liver disease supports mucosal lymphocyte adhesion to hepatic endothelium (MAdCAM-1 in chronic inflammatory liver disease). Hepatology 2001; 33: 1065-72.
31. Eksteen B, Grant AJ, Miles A, et al. Hepatic endothelial CCL25 mediates the recruitment of CCR9+ gut-homing lymphocytes to the liver in primary sclerosing cholangitis. J Exp Med 2004; 200: 1511-17.
32. Eksteen B, Miles A, Curbishley SM, et al. Epithelial inflammation is associated with CCL28 production and the recruitment of regulatory T cells expressing CCR10. J Immunol 2006; 177: 593-603.
33. Miles A, Liaskou E, Eksteen B, Lalor PF, Adams DH. CCL25 and CCL28 promote alpha4 beta7-integrin-dependent adhesion of lymphocytes to MAdCAM-1 under shear flow. Am J Physiol Gastrointest Liver Physiol 2008; 294: G1257-67.
34. Aspinall AI, Curbishley SM, Lalor PF, et al. CX(3)CR1 and vascular adhesion protein-1-dependent recruitment of CD16(+) monocytes across human liver sinusoidal endothelium. Hepatology 2010; 51: 2030-39.
35. Liaskou E, Karikoski M, Reynolds GM, et al. Regulation of mucosal addressin cell adhesion molecule 1 expression in human and mice by vascular adhesion protein 1 amine oxidase activity. Hepatology 2011; 53: 661-72.
36. Hohenester S, Wenniger LM, Paulusma CC, et al. A biliary HCO3-umbrella constitutes a protective mechanism against bile acid-induced injury in human cholangiocytes. Hepatology 2012; 55: 173-83.
37. Jonker JW, Liddle C, Downes M. FXR and PXR: potential therapeutic targets in cholestasis. J Steroid Biochem Mol Biol 2012; 130: 147-58.
38. Jostins L, Ripke S, Weersma RK, et al. Host-microbe interactions have shaped the genetic architecture of inflammatory bowel disease. Nature 2012; 491: 119-24.
39. Arthur JC, Perez-Chanona E, Muhlbauer M, et al. Intestinal inflammation targets cancer-inducing activity of the microbiota. Science 2012; 338: 120-23.
40. Devkota S, Wang Y, Musch MW, et al. Dietary-fat-induced taurocholic acid promotes pathobiont expansion and colitis in Il10-/- mice. Nature 2012; 487: 104-08.
41. Rausch P, Rehman A, Kunzel S, et al. Colonic mucosa-associated microbiota is influenced by an interaction of Crohn disease and FUT2 (Secretor) genotype. Proc Natl Acad Sci USA 2011; 108: 19030-35.
42. Fan X, Upadhyaya B, Wu L, et al. CD40 agonist antibody mediated improvement of chronic Cryptosporidium infection in patients with X-linked hyper IgM syndrome. Clin Immunol 2012; 143: 152-61.
43. Frulloni L, Lunardi C, Simone R, et al. Identification of a novel antibody associated with autoimmune pancreatitis. N Engl J Med 2009; 361: 2135-42.
44. Terjung B, Sohne J, Lechtenberg B, et al. p-ANCAs in autoimmune liver disorders recognise human beta-tubulin isotype 5 and cross-react with microbial protein FtsZ. Gut 2010; 59: 808-16.
45. Strazzabosco M, Fabris L. Functional anatomy of normal bile ducts. Anat Rec (Hoboken) 2008; 291: 653-60.
46. Benito de Valle M, Rahman M, Lindkvist B, Bjornsson E, Chapman R, Kalaitzakis E. Factors that reduce health-related quality of life in patients with primary sclerosing cholangitis. Clin Gastroenterol Hepatol 2012; 10: 769-75.
47. 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: 48-54.
48. Schrumpf E, Elgjo K, Fausa O, Gjone E, Kolmannskog F, Ritland S. Sclerosing cholangitis in ulcerative colitis. Scand J Gastroenterol 1980; 15: 689-97.
49. Terg R, Sambuelli A, Coronel E, et al. Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis and the risk of developing malignancies. A large prospective study. Acta Gastroenterol Latinoam 2008; 38: 26-33.
50. O'Toole A, Alakkari A, Keegan D, Doherty G, Mulcahy H, O'Donoghue D. Primary sclerosing cholangitis and disease distribution in inflammatory bowel disease. Clin Gastroenterol Hepatol 2012; 10: 439-41.
51. Olsson R, Danielsson A, Jarnerot G, et al. Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis. Gastroenterology 1991; 100: 1319-23.
52. Halliday JS, Djordjevic J, Lust M, et al. A unique clinical phenotype of primary sclerosing cholangitis associated with Crohn's disease. J Crohns Colitis 2012; 6: 174-81.
53. Marelli L, Xirouchakis E, Kalambokis G, Cholongitas E, Hamilton MI, Burroughs AK. Does the severity of primary sclerosing cholangitis influence the clinical course of associated ulcerative colitis? Gut 2011; 60: 1224-28.
54. Navaneethan U, Venkatesh PG, Mukewar S, et al. Progressive primary sclerosing cholangitis requiring liver transplantation is associated with reduced need for colectomy in patients with ulcerative colitis. Clin Gastroenterol Hepatol 2012; 10: 540-46.
55. Aljiffry M, Renfrew PD, Walsh MJ, Laryea M, Molinari M. Analytical review of diagnosis and treatment strategies for dominant bile duct strictures in patients with primary sclerosing cholangitis. HPB (Oxford) 2011; 13: 79-90.
56. Chapman MH, Webster GJ, Bannoo S, Johnson GJ, Wittmann J, Pereira SP. Cholangiocarcinoma and dominant strictures in patients with primary sclerosing cholangitis: a 25-year single-centre experience. Eur J Gastroenterol Hepatol 2012; 24: 1051-58.
57. Jess T, Simonsen J, Jorgensen KT, Pedersen BV, Nielsen NM, Frisch M. Decreasing risk of colorectal cancer in patients with inflammatory bowel disease over 30 years. Gastroenterology 2012; 143: 375-81.
58. Broome U, Lofberg R, Veress B, Eriksson LS. Primary sclerosing cholangitis and ulcerative colitis: evidence for increased neoplastic potential. Hepatology 1995; 22: 1404-08.
59. Dave M, Elmunzer BJ, Dwamena BA, Higgins PD. Primary sclerosing cholangitis: meta-analysis of diagnostic performance of MR cholangiopancreatography. Radiology 2010; 256: 387-96.
60. MacCarty RL, LaRusso NF, Wiesner RH, Ludwig J. Primary sclerosing cholangitis: findings on cholangiography and pancreatography. Radiology 1983; 149: 39-44.
61. Engler S, Elsing C, Flechtenmacher C, Theilmann L, Stremmel W, Stiehl A. Progressive sclerosing cholangitis after septic shock: a new variant of vanishing bile duct disorders. Gut 2003; 52: 688-93.
62. Sah RP, Chari ST, Pannala R, et al. Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis. Gastroenterology 2010; 139: 140-48.
63. Mendes FD, Jorgensen R, Keach J, et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 2006; 101: 2070-75.
64. Alswat K, Al-Harthy N, Mazrani W, Alshumrani G, Jhaveri K, Hirschfield GM. The spectrum of sclerosing cholangitis and the relevance of IgG4 elevations in routine practice. Am J Gastroenterol 2012; 107: 56-63.
65. Bjornsson E, Chari S, Silveira M, et al. Primary sclerosing cholangitis associated with elevated Immunoglobulin G4: clinical characteristics and response to therapy. Am J Ther 2011; 18: 198-205.
66. Bergquist A, Glaumann H, Persson B, Broome U. Risk factors and clinical presentation of hepatobiliary carcinoma in patients with primary sclerosing cholangitis: a case-control study. Hepatology 1998; 27: 311-16.
67. Razumilava N, Gores GJ, Lindor KD. Cancer surveillance in patients with primary sclerosing cholangitis. Hepatology 2011; 54: 1842-52.
68. Boberg KM, Bergquist A, Mitchell S, et al. Cholangiocarcinoma in primary sclerosing cholangitis: risk factors and clinical presentation. Scand J Gastroenterol 2002; 37: 1205-11.
69. de Valle MB, Bjornsson E, Lindkvist B. Mortality and cancer risk related to primary sclerosing cholangitis in a Swedish population-based cohort. Liver Int 2012; 32: 441-48.
70. 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: 1106-17.
71. Sinakos E, Saenger AK, Keach J, Kim WR, Lindor KD. Many patients with primary sclerosing cholangitis and increased serum levels of carbohydrate antigen 19-9 do not have cholangiocarcinoma. Clin Gastroenterol Hepatol 2011; 9: 434-39.
72. Rabinovitz M, Zajko AB, Hassanein T, et al. Diagnostic value of brush cytology in the diagnosis of bile duct carcinoma: a study in 65 patients with bile duct strictures. Hepatology 1990; 12: 747-52.
73. Boberg KM, Jebsen P, Clausen OP, Foss A, Aabakken L, Schrumpf E. Diagnostic benefit of biliary brush cytology in cholangiocarcinoma in primary sclerosing cholangitis. J Hepatol 2006; 45: 568-74.
74. Marsh Rde W, Alonzo M, Bajaj S, et al. Comprehensive review of the diagnosis and treatment of biliary tract cancer 2012. Part I: diagnosis-clinical staging and pathology. J Surg Oncol 2012; 106: 332-38.
75. Marsh Rde W, Alonzo M, Bajaj S, et al. Comprehensive review of the diagnosis and treatment of biliary tract cancer 2012. Part II: multidisciplinary management. J Surg Oncol 2012; 106: 339-45.
76. Bangarulingam S Y, Gossard AA, Petersen BT, Ott BJ, Lindor KD. Complications of endoscopic retrograde cholangiopancreatography in primary sclerosing cholangitis. Am J Gastroenterol 2009; 104: 855-60.
77. Mohamadnejad M, DeWitt JM, Sherman S, et al. Role of EUS for preoperative evaluation of cholangiocarcinoma: a large single-center experience. Gastrointest Endosc 2011; 73: 71-78.
78. Moreno Luna LE, Kipp B, Halling KC, et al. Advanced cytologic techniques for the detection of malignant pancreatobiliary strictures. Gastroenterology 2006; 131: 1064-72.
79. Levy MJ, Baron TH, Clayton AC, et al. Prospective evaluation of advanced molecular markers and imaging techniques in patients with indeterminate bile duct strictures. Am J Gastroenterol 2008; 103: 1263-73.
80. Fritcher EG, Kipp BR, Halling KC, et al. A multivariable model using advanced cytologic methods for the evaluation of indeterminate pancreatobiliary strictures. Gastroenterology 2009; 136: 2180-86.
81. Kalaitzakis E, Webster GJ, Oppong KW, et al. Diagnostic and therapeutic utility of single-operator peroral cholangioscopy for indeterminate biliary lesions and bile duct stones. Eur J Gastroenterol Hepatol 2012; 24: 656-64.
82. Choi ER, Chung YH, Lee JK, et al. Preoperative evaluation of the longitudinal extent of borderline resectable hilar cholangiocarcinoma by intraductal ultrasonography. J Gastroenterol Hepatol 2011; 26: 1804-10.
83. Metzger J, Negm AA, Plentz RR, et al. Urine proteomic analysis differentiates cholangiocarcinoma from primary sclerosing cholangitis and other benign biliary disorders. Gut 2013; 62: 122-30.
84. Lankisch TO, Metzger J, Negm AA, et al. Bile proteomic profiles differentiate cholangiocarcinoma from primary sclerosing cholangitis and choledocholithiasis. Hepatology 2011; 53: 875-84.
85. Buckles DC, Lindor KD, Larusso NF, Petrovic LM, Gores GJ. In primary sclerosing cholangitis, gallbladder polyps are frequently malignant. Am J Gastroenterol 2002; 97: 1138-42.
86. Said K, Glaumann H, Bergquist A. Gallbladder disease in patients with primary sclerosing cholangitis. J Hepatol 2008; 48: 598-605.
87. Eaton JE, Thackeray EW, Lindor KD. Likelihood of malignancy in gallbladder polyps and outcomes following cholecystectomy in primary sclerosing cholangitis. Am J Gastroenterol 2012; 107: 431-39.
88. Bjornsson E, Boberg KM, Cullen S, et al. Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis. Gut 2002; 51: 731-35.
89. Bjornsson E, Olsson R, Bergquist A, et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology 2008; 134: 975-80.
90. 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.
91. Lewin M, Vilgrain V, Ozenne V, et al. Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: a prospective magnetic resonance imaging and histological study. Hepatology 2009; 50: 528-37.
92. 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.
93. Gregorio GV, Portmann B, Karani J, et al. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology 2001; 33: 544-53.
94. Al Mamari S, Djordjevic J, Halliday JS, Chapman R W. 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: 329-34.
95. Treeprasertsuk S, Kowdley KV, Luketic VA, et al. The predictors of the presence of varices in patients with primary sclerosing cholangitis. Hepatology 2010; 51: 1302-10.
96. Alabraba E, Nightingale P, Gunson B, et al. A re-evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts. Liver Transpl 2009; 15: 330-40.
97. Jørgensen KK, Lindström L, Cvancarova M, et al. Immunosuppression after liver transplantation for primary sclerosing cholangitis influences activity of inflammatory bowel disease. Clin Gastroenterol Hepatol 2013; published online Jan 16. DOI:10.1016/j.cgh.2012.12.027.
98. Chapman RW. High-dose ursodeoxycholic acid in the treatment of primary sclerosing cholangitis: throwing the urso out with the bathwater? Hepatology 2009; 50: 671-73.
99. Beuers U, Spengler U, Kruis W, et al. Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial. Hepatology 1992; 16: 707-14.
100. Chazouilleres O, Poupon R, Capron JP, et al. Ursodeoxycholic acid for primary sclerosing cholangitis. J Hepatol 1990; 11: 120-23.
101. O'Brien CB, Senior JR, Arora-Mirchandani R, Batta AK, Salen G. Ursodeoxycholic acid for the treatment of primary sclerosing cholangitis: a 30-month pilot study. Hepatology 1991; 14: 838-47.
102. Lindor KD. Ursodiol for primary sclerosing cholangitis. Mayo Primary Sclerosing Cholangitis-Ursodeoxycholic Acid Study Group. N Engl J Med 1997; 336: 691-95.
103. Mitchell SA, Bansi DS, Hunt N, Von Bergmann K, Fleming KA, Chapman RW. A preliminary trial of high-dose ursodeoxycholic acid in primary sclerosing cholangitis. Gastroenterology 2001; 121: 900-07.
104. Olsson R, Boberg KM, de Muckadell OS, et al. High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5-year multicenter, randomized, controlled study. Gastroenterology 2005; 129: 1464-72.
105. Lindor KD, Kowdley KV, Luketic VA, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology 2009; 50: 808-14.
106. Imam MH, Sinakos E, Gossard AA, et al. High-dose ursodeoxycholic acid increases risk of adverse outcomes in patients with early stage primary sclerosing cholangitis. Aliment Pharmacol Ther 2011; 34: 1185-92.
107. Eaton JE, Silveira MG, Pardi DS, et al. High-dose ursodeoxycholic acid is associated with the development of colorectal neoplasia in patients with ulcerative colitis and primary sclerosing cholangitis. Am J Gastroenterol 2011; 106: 1638-45.
108. Lindstrom L, Boberg KM, Wikman O, et al. High dose ursodeoxycholic acid in primary sclerosing cholangitis does not prevent colorectal neoplasia. Aliment Pharmacol Ther 2012; 35: 451-57.
109. Pardi DS, Loftus EV Jr, Kremers WK, Keach J, Lindor KD. Ursodeoxycholic acid as a chemopreventive agent in patients with ulcerative colitis and primary sclerosing cholangitis. Gastroenterology 2003; 124: 889-93.
110. Rudolph G, Kloeters-Plachky P, Rost D, Stiehl A. The incidence of cholangiocarcinoma in primary sclerosing cholangitis after long-time treatment with ursodeoxycholic acid. Eur J Gastroenterol Hepatol 2007; 19: 487-91.
111. Triantos CK, Koukias NM, Nikolopoulou VN, Burroughs AK. Meta-analysis: ursodeoxycholic acid for primary sclerosing cholangitis. Aliment Pharmacol Ther 2011; 34: 901-10.
112. Sinakos E, Marschall HU, Kowdley KV, Befeler A, Keach J, Lindor K. Bile acid changes after high-dose ursodeoxycholic acid treatment in primary sclerosing cholangitis: relation to disease progression. Hepatology 2010; 52: 197-203.
113. Denk GU, Maitz S, Wimmer R, et al. Conjugation is essential for the anticholestatic effect of NorUrsodeoxycholic acid in taurolithocholic acid-induced cholestasis in rat liver. Hepatology 2010; 52: 1758-68.
114. Woo K, Sathe M, Kresge C, et al. Adenosine triphosphate release and purinergic (P2) receptor-mediated secretion in small and large mouse cholangiocytes. Hepatology 2010; 52: 1819-28.
115. Martin CR, Blanco PG, Keach JC, et al. The safety and efficacy of oral docosahexaenoic acid supplementation for the treatment of primary sclerosing cholangitis - a pilot study. Aliment Pharmacol Ther 2012; 35: 255-65.
116. Modica S, Petruzzelli M, Bellafante E, et al. Selective activation of nuclear bile acid receptor FXR in the intestine protects mice against cholestasis. Gastroenterology 2012; 142: 355-65.
117. Angulo P, Batts KP, Jorgensen RA, LaRusso NA, Lindor KD. Oral budesonide in the treatment of primary sclerosing cholangitis. Am J Gastroenterol 2000; 95: 2333-37.
118. van Hoogstraten HJ, Vleggaar FP, Boland GJ, et al. Budesonide or prednisone in combination with ursodeoxycholic acid in primary sclerosing cholangitis: a randomized double-blind pilot study. Belgian-Dutch PSC Study Group. Am J Gastroenterol 2000; 95: 2015-22.
119. Schramm C, Schirmacher P, Helmreich-Becker I, Gerken G, zum Buschenfelde KH, Lohse AW. Combined therapy with azathioprine, prednisolone, and ursodiol in patients with primary sclerosing cholangitis. A case series. Ann Intern Med 1999; 131: 943-46.
120. Talwalkar JA, Gossard AA, Keach JC, Jorgensen RA, Petz JL, Lindor RN. Tacrolimus for the treatment of primary sclerosing cholangitis. Liver Int 2007; 27: 451-53.
121. Karaahmet F, Kilincalp S, Altinbas A, Coban S, Yuksel O. Intrabiliary rapamycin may slow progression of primary sclerosing cholangitis. Aliment Pharmacol Ther 2012; 35: 490-91.
122. Farkkila M, Karvonen AL, Nurmi H, et al. Metronidazole and ursodeoxycholic acid for primary sclerosing cholangitis: a randomized placebo-controlled trial. Hepatology 2004; 40: 1379-86.
123. Davies YK, Cox KM, Abdullah BA, Safta A, Terry AB, Cox KL. Long-term treatment of primary sclerosing cholangitis in children with oral vancomycin: an immunomodulating antibiotic. J Pediatr Gastroenterol Nutr 2008; 47: 61-67.
124. Boner AL, Peroni D, Bodini A, Delaini G, Piacentini G. Azithromycin may reduce cholestasis in primary sclerosing cholangitis: a case report and serendipitous observation. Int J Immunopathol Pharmacol 2007; 20: 847-49.
125. Silveira MG, Torok NJ, Gossard AA, et al. Minocycline in the treatment of patients with primary sclerosing cholangitis: results of a pilot study. Am J Gastroenterol 2009; 104: 83-88.
126. Hommes DW, Erkelens W, Ponsioen C, et al. A double-blind, placebo-controlled, randomized study of infliximab in primary sclerosing cholangitis. J Clin Gastroenterol 2008; 42: 522-26.
127. Pohl J, Ring A, Stremmel W, Stiehl A. The role of dominant stenoses in bacterial infections of bile ducts in primary sclerosing cholangitis. Eur J Gastroenterol Hepatol 2006; 18: 69-74.
128. Stiehl A, Rudolph G, Kloters-Plachky P, Sauer P, Walker S. Development of dominant bile duct stenoses in patients with primary sclerosing cholangitis treated with ursodeoxycholic acid: outcome after endoscopic treatment. J Hepatol 2002; 36: 151-56.
129. 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: 149-55.
130. Kaya M, Petersen BT, Angulo P, et al. Balloon dilation compared to stenting of dominant strictures in primary sclerosing cholangitis. Am J Gastroenterol 2001; 96: 1059-66.
131. Etzel JP, Eng SC, Ko CW, et al. Complications after ERCP in patients with primary sclerosing cholangitis. Gastrointest Endosc 2008; 67: 643-48.
132. Neuberger J, Gimson A, Davies M, et al. Selection of patients for liver transplantation and allocation of donated livers in the UK. Gut 2008; 57: 252-57.
133. Kamath PS, Wiesner RH, Malinchoc M, et al. A model to predict survival in patients with end-stage liver disease. Hepatology 2001; 33: 464-70.
134. Darwish Murad S, Kim WR, Harnois DM, et al. Efficacy of neoadjuvant chemoradiation, followed by liver transplantation, for perihilar cholangiocarcinoma at 12 US centers. Gastroenterology 2012; 143: 88-98.
135. Torres J, Pineton de Chambrun G, Itzkowitz S, Sachar DB, Colombel JF. Review article: colorectal neoplasia in patients with primary sclerosing cholangitis and inflammatory bowel disease. Aliment Pharmacol Ther 2011; 34: 497-508.
136. Jørgensen KK, Lindstrom L, Cvancarova M, et al. Colorectal neoplasia in patients with primary sclerosing cholangitis undergoing liver transplantation: a Nordic multicenter study. Scand J Gastroenterol 2012; 8-9: 1021-29.
137. Penna C, Dozois R, Tremaine W, et al. Pouchitis after ileal pouch-anal anastomosis for ulcerative colitis occurs with increased frequency in patients with associated primary sclerosing cholangitis. Gut 1996; 38: 234-39.
138. Kremer AE, van Dijk R, Leckie P, et al. Serum autotaxin is increased in pruritus of cholestasis, but not of other origin, and responds to therapeutic interventions. Hepatology 2012; 56: 1391-400.
139. Alemi F, Kwon E, Poole DP, et al. The TGR5 receptor mediates bile acid-induced itch and analgesia. J Clin Invest 2013; 123: 1513-30.
140. Al-Harthy N, Kumagi T, Coltescu C, Hirschfield GM. The specificity of fatigue in primary biliary cirrhosis: evaluation of a large clinic practice. Hepatology 2010; 52: 562-70.
141. Angulo P, Grandison GA, Fong DG, et al. Bone disease in patients with primary sclerosing cholangitis. Gastroenterology 2011; 140: 180-88.