Update on primary sclerosing cholangitis

Journal of Hepatology

Volumn 59, Issue 3, 2013, Pages 571-582

  Karlsen, Tom H ^a,b   Boberg, Kirsten Muri ^a,c,d  

^a OSLO UNIVERSITY HOSPITAL   (Norway)

^b UNIVERSITY OF BERGEN   (Norway)

^c UNIVERSITY OF OSLO   (Norway)

^d OSLO UNIVERSITY HOSPITAL   (Norway)

Author keywords

Cholangiocarcinoma; Inflammatory bowel disease; Primary sclerosing cholangitis

Indexed keywords

ANTIFIBROTIC AGENT; AZATHIOPRINE; AZITHROMYCIN; BILE ACID; CYCLOSPORIN A; GS 6624; IMMUNOGLOBULIN G4; METRONIDAZOLE; MINOCYCLINE; MONOCLONAL ANTIBODY; MYCOPHENOLIC ACID; TACROLIMUS; UNCLASSIFIED DRUG; URSODEOXYCHOLIC ACID; VANCOMYCIN;

AUTOIMMUNE HEPATITIS; BILE DUCT CARCINOMA; CANCER EPIDEMIOLOGY; CANCER RISK; CHOLESTASIS; DRUG TARGETING; ENTERITIS; FOLLOW UP; GENETIC ASSOCIATION; HUMAN; IMMUNE MEDIATED INJURY; IMMUNOGLOBULIN BLOOD LEVEL; IMMUNOSUPPRESSIVE TREATMENT; LIVER TRANSPLANTATION; NONHUMAN; PATHOGENESIS; PRIMARY SCLEROSING CHOLANGITIS; PRIORITY JOURNAL; PRURITUS; REVIEW; TOXICITY;

CHOLANGIOCARCINOMA; INFLAMMATORY BOWEL DISEASE; PRIMARY SCLEROSING CHOLANGITIS;

ANIMALS; AUTOIMMUNITY; BILE ACIDS AND SALTS; BILE DUCT NEOPLASMS; BILE DUCTS, INTRAHEPATIC; CHOLANGIOCARCINOMA; CHOLANGITIS, SCLEROSING; CHOLESTASIS; DIGESTIVE SYSTEM; GENETIC ASSOCIATION STUDIES; GENETIC PREDISPOSITION TO DISEASE; HEPATITIS, AUTOIMMUNE; HUMANS; IMMUNOGLOBULIN A; INFLAMMATORY BOWEL DISEASES; LIVER TRANSPLANTATION; MICROBIOTA; MODELS, BIOLOGICAL; PRURITUS;

EID: 84882846687     PISSN: 01688278     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.jhep.2013.03.015     Document Type: Review

References (158)

1. C.E.E. Hoffman Verschluss der gallenwege durch verdickung der wandungen Arch Pathol Anat Physiol 39 1867 206 215
2. T.H. Karlsen, E. Schrumpf, and K.M. Boberg Update on primary sclerosing cholangitis Dig Liver Dis 42 2010 390 400
3. F.D. Mendes, R. Jorgensen, J. Keach, J.A. Katzmann, T. Smyrk, and J. Donlinger Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis Am J Gastroenterol 101 2006 2070 2075
4. T.H. Karlsen A lecture on the genetics of primary sclerosing cholangitis Dig Dis 30 2012 32 38
5. T.H. Karlsen, and A. Kaser Deciphering the genetic predisposition to primary sclerosing cholangitis Semin Liver Dis 31 2011 188 207
6. T. Folseraas, E. Melum, A. Franke, and T.H. Karlsen Genetics in primary sclerosing cholangitis Best Pract Res Clin Gastroenterol 25 2011 713 726
7. S. Naess, A. Shiryaev, J.R. Hov, A. Franke, and T.H. Karlsen Genetics in primary sclerosing cholangitis Clin Res Hepatol Gastroenterol 36 2012 325 333
8. T.A. Manolio, F.S. Collins, N.J. Cox, D.B. Goldstein, L.A. Hindorff, and D.J. Hunter Finding the missing heritability of complex diseases Nature 461 2009 747 753
9. J.Z. Liu, J.E. Hov, T. Folseraas, E. Ellinghaus, S.M. Rushbrook, and N.T. Doncheva Dense genotyping of immune-related disease regions identifies nine new risk loci for primary sclerosing cholangitis Nature Genetics 45 2013 670 675
10. A. Cortes, and M.A. Brown Promise and pitfalls of the Immunochip Arthritis Res Ther 13 2011 101
11. N. Mitro, C. Godio, E. De Fabiani, E. Scotti, A. Galmozzi, and F. Gilardi Insights in the regulation of cholesterol 7 alpha-hydroxylase gene reveal a target for modulating bile acid synthesis Hepatology 46 2007 885 897
12. K. Clark, K.F. MacKenzie, K. Petkevicius, Y. Kristariyanto, J. Zhang, and H.G. Choi Phosphorylation of CRTC3 by the salt-inducible kinases controls the interconversion of classically activated and regulatory macrophages Proc Natl Acad Sci U S A 109 2012 16986 16991
13. U. Beuers Drug insight: mechanisms and sites of action of ursodeoxycholic acid in cholestasis Nat Clin Pract Gastroenterol Hepatol 3 2006 318 328
14. U. Beuers, S. Hohenester, L.J. De Buy Wenniger, A.E. Kremer, P.L. Jansen, and R.P. Elferink The biliary HCO(3) (-) umbrella: a unifying hypothesis on pathogenetic and therapeutic aspects of fibrosing cholangiopathies Hepatology 52 2010 1489 1496
15. S. Hohenester, L.M. Wenniger, C.C. Paulusma, S.J. van Vliet, D.M. Jefferson, and R.P. Elferink A biliary HCO3- umbrella constitutes a protective mechanism against bile acid-induced injury in human cholangiocytes Hepatology 55 2012 173 183
16. - channel activated by extracellular nucleotides, in biliary epithelium J Biol Chem 286 2011 766 776
17. V. Keitel, C. Ullmer, and D. Haussinger The membrane-bound bile acid receptor TGR5 (Gpbar-1) is localized in the primary cilium of cholangiocytes Biol Chem 391 2010 785 789
18. V. Keitel, K. Cupisti, C. Ullmer, W.T. Knoefel, R. Kubitz, and D. Haussinger The membrane-bound bile acid receptor TGR5 is localized in the epithelium of human gallbladders Hepatology 50 2009 861 870
19. -/- mice results in bile duct injury Am J Physiol Gastrointest Liver Physiol 287 2004 G491 G496
20. R. Fiorotto, R. Scirpo, M. Trauner, L. Fabris, R. Hoque, and C. Spirli Loss of CFTR affects biliary epithelium innate immunity and causes TLR4-NF-kappaB-mediated inflammatory response in mice Gastroenterology 141 2011 1498 1508 e1491-1495
21. R.M. McMinn, and J.H. Kugler The glands of the bile and pancreatic ducts: autoradiographic and histochemical studies J Anat 95 1961 1 11
22. L. Spitz, and A. Petropoulos The development of the glands of the common bile duct J Pathol 128 1979 213 220
23. E. Halilbasic, T. Claudel, and M. Trauner Bile acid transporters and regulatory nuclear receptors in the liver and beyond J Hepatol 58 2013 155 168
24. M. Trauner, and E. Halilbasic Nuclear receptors as new perspective for the management of liver diseases Gastroenterology 140 2011 1120 1125 e1121-1112
25. S. Modica, M. Petruzzelli, E. Bellafante, S. Murzilli, L. Salvatore, and N. Celli Selective activation of nuclear bile acid receptor FXR in the intestine protects mice against cholestasis Gastroenterology 142 2012 355 365 e351-354
26. -/-) mouse cholangiopathy model by promoting biliary HCO output Hepatology 54 2011 1303 1312
27. F.G. Schaap, N.A. van der Gaag, D.J. Gouma, and P.L. Jansen High expression of the bile salt-homeostatic hormone fibroblast growth factor 19 in the liver of patients with extrahepatic cholestasis Hepatology 49 2009 1228 1235
28. V. Triantis, E. Saeland, N. Bijl, R.P. Oude-Elferink, and P.L. Jansen Glycosylation of fibroblast growth factor receptor 4 is a key regulator of fibroblast growth factor 19-mediated down-regulation of cytochrome P450 7A1 Hepatology 52 2010 656 666
29. S.J. Zweers, K.A. Booij, M. Komuta, T. Roskams, D.J. Gouma, and P.L. Jansen The human gallbladder secretes fibroblast growth factor 19 into bile: towards defining the role of fibroblast growth factor 19 in the enterobiliary tract Hepatology 55 2012 575 583
30. C.A. Anderson, G. Boucher, C.W. Lees, A. Franke, M. D'Amato, and K.D. Taylor Meta-analysis identifies 29 additional ulcerative colitis risk loci, increasing the number of confirmed associations to 47 Nat Genet 43 2011 246 252
31. J.R. Hov, V. Keitel, J.K. Laerdahl, L. Spomer, E. Ellinghaus, and A. Elsharawy Mutational characterization of the bile acid receptor TGR5 in primary sclerosing cholangitis PLoS One 5 2010 e12403
32. Y.D. Wang, W.D. Chen, D. Yu, B.M. Forman, and W. Huang The G-protein-coupled bile acid receptor, Gpbar1 (TGR5), negatively regulates hepatic inflammatory response through antagonizing nuclear factor kappa light-chain enhancer of activated B cells (NF-kappaB) in mice Hepatology 54 2011 1421 1432
33. S. Cipriani, A. Mencarelli, M.G. Chini, E. Distrutti, B. Renga, and G. Bifulco The bile acid receptor GPBAR-1 (TGR5) modulates integrity of intestinal barrier and immune response to experimental colitis PLoS One 6 2011 e25637
34. V. Keitel, M. Donner, S. Winandy, R. Kubitz, and D. Haussinger Expression and function of the bile acid receptor TGR5 in Kupffer cells Biochem Biophys Res Commun 372 2008 78 84
35. Y. Kawamata, R. Fujii, M. Hosoya, M. Harada, H. Yoshida, and M. Miwa A G protein-coupled receptor responsive to bile acids J Biol Chem 278 2003 9435 9440
36. W.D. Chen, D. Yu, B.M. Forman, W. Huang, and Y.D. Wang Deficiency of G-protein-coupled bile acid receptor Gpbar1 (TGR5) enhances chemically induced liver carcinogenesis Hepatology 57 2013 656 666
37. T. Li, S.R. Holmstrom, S. Kir, M. Umetani, D.R. Schmidt, and S.A. Kliewer The G protein-coupled bile acid receptor, TGR5, stimulates gallbladder filling Mol Endocrinol 25 2011 1066 1071
38. V. Keitel, and D. Haussinger TGR5 in the biliary tree Dig Dis 29 2011 45 47
39. K. Isse, K. Harada, and Y. Nakanuma IL-8 expression by biliary epithelial cells is associated with neutrophilic infiltration and reactive bile ductules Liver Int 27 2007 672 680
40. A.S. Bansal, A. Thomson, C. Steadman, G. Le Gros, P.G. Hogan, and P. Kerlin Serum levels of interleukins 8 and 10, interferon gamma, granulocyte-macrophage colony stimulating factor and soluble CD23 in patients with primary sclerosing cholangitis Autoimmunity 26 1997 223 229
41. -/-) mouse cholangiopathy model by promoting biliary HCO(-)(3) output Hepatology 54 2011 1303 1312
42. T.H. Karlsen, B.A. Lie, K. Frey Froslie, E. Thorsby, U. Broome, and E. Schrumpf Polymorphisms in the steroid and xenobiotic receptor gene influence survival in primary sclerosing cholangitis Gastroenterology 131 2006 781 787
43. D. Ellinghaus, T. Folseraas, K. Holm, E. Ellinghaus, E. Melum, and T. Balschun Genome-wide association analysis in primary sclerosing cholangitis and ulcerative colitis identifies risk loci at GPR35 and TCF4 Hepatology 2013 10.1002/hep.25977 in press
44. T. Moustafa, P. Fickert, C. Magnes, C. Guelly, A. Thueringer, and S. Frank Alterations in lipid metabolism mediate inflammation, fibrosis, and proliferation in a mouse model of chronic cholestatic liver injury Gastroenterology 142 2012 e112
45. G.M. Weinstock Genomic approaches to studying the human microbiota Nature 489 2012 250 256
46. The Human Microbiome Project Consortium Structure, function and diversity of the healthy human microbiome Nature 486 2012 207 214
47. J. Kuczynski, C.L. Lauber, W.A. Walters, L.W. Parfrey, J.C. Clemente, and D. Gevers Experimental and analytical tools for studying the human microbiome Nat Rev Genet 13 2012 47 58
48. J.G. Markle, D.N. Frank, S. Mortin-Toth, C.E. Robertson, L.M. Feazel, and U. Rolle-Kampczyk Sex differences in the gut microbiome drive hormone-dependent regulation of autoimmunity Science 339 2013 1084 1088
49. A. Spor, O. Koren, and R. Ley Unravelling the effects of the environment and host genotype on the gut microbiome Nat Rev Microbiol 9 2011 279 290
50. W.S. Garrett, G.M. Lord, S. Punit, G. Lugo-Villarino, S.K. Mazmanian, and S. Ito Communicable ulcerative colitis induced by T-bet deficiency in the innate immune system Cell 131 2007 33 45
51. C.F. Maurice, H.J. Haiser, and P.J. Turnbaugh Xenobiotics shape the physiology and gene expression of the active human gut microbiome Cell 15 2013 39 50
52. D.P. McGovern, M.R. Jones, K.D. Taylor, K. Marciante, X. Yan, and M. Dubinsky Fucosyltransferase 2 (FUT2) non-secretor status is associated with Crohn's disease Hum Mol Genet 19 2010 3468 3476
53. P. Rausch, A. Rehman, S. Kunzel, R. Hasler, S.J. Ott, and S. Schreiber Colonic mucosa-associated microbiota is influenced by an interaction of Crohn disease and FUT2 (secretor) genotype Proc Natl Acad Sci U S A 108 2011 19030 19035
54. P. Wacklin, H. Makivuokko, N. Alakulppi, J. Nikkila, H. Tenkanen, and J. Rabina Secretor genotype (FUT2 gene) is strongly associated with the composition of Bifidobacteria in the human intestine PLoS One 6 2011 e20113
55. A. Franke, T. Balschun, T.H. Karlsen, J. Sventoraityte, S. Nikolaus, and G. Mayr Sequence variants in IL10, ARPC2 and multiple other loci contribute to ulcerative colitis susceptibility Nat Genet 40 2008 1319 1323
56. -/- mice Nature 487 2012 104 108
57. D.H. Adams, and B. Eksteen Aberrant homing of mucosal T cells and extra-intestinal manifestations of inflammatory bowel disease Nat Rev Immunol 6 2006 244 251
58. M. Iwata, A. Hirakiyama, Y. Eshima, H. Kagechika, C. Kato, and S.Y. Song Retinoic acid imprints gut-homing specificity on T cells Immunity 21 2004 527 538
59. B. Eksteen, J.R. Mora, E.L. Haughton, N.C. Henderson, L. Lee-Turner, and E.J. Villablanca Gut homing receptors on CD8 T cells are retinoic acid dependent and not maintained by liver dendritic or stellate cells Gastroenterology 137 2009 320 329
60. K. Neumann, N. Kruse, B. Szilagyi, U. Erben, C. Rudolph, and A. Flach Connecting liver and gut: murine liver sinusoidal endothelium induces gut tropism of CD4+ T cells via retinoic acid Hepatology 55 2012 1976 1984
61. E. Liaskou, M. Karikoski, G.M. Reynolds, P.F. Lalor, C.J. Weston, and N. Pullen Regulation of mucosal addressin cell adhesion molecule 1 expression in human and mice by vascular adhesion protein 1 amine oxidase activity Hepatology 53 2011 661 672
62. P.F. Lalor, C. Tuncer, C. Weston, A. Martin-Santos, D.J. Smith, and D.H. Adams Vascular adhesion protein-1 as a potential therapeutic target in liver disease Ann N Y Acad Sci 1110 2007 485 496
63. S. Shetty, T. Bruns, C.J. Weston, Z. Stamataki, Y.H. Oo, and H.M. Long Recruitment mechanisms of primary and malignant B cells to the human liver Hepatology 56 2012 1521 1531
64. Y.H. Oo, C.J. Weston, P.F. Lalor, S.M. Curbishley, D.R. Withers, and G.M. Reynolds Distinct roles for CCR4 and CXCR3 in the recruitment and positioning of regulatory T cells in the inflamed human liver J Immunol 184 2010 2886 2898
65. A.I. Aspinall, S.M. Curbishley, P.F. Lalor, C.J. Weston, E. Liaskou, and R.M. Adams CX(3)CR1 and vascular adhesion protein-1-dependent recruitment of CD16(+) monocytes across human liver sinusoidal endothelium Hepatology 51 2010 2030 2039
66. Y.H. Oo, V. Banz, D. Kavanagh, E. Liaskou, D.R. Withers, and E. Humphreys CXCR3-dependent recruitment and CCR6-mediated positioning of Th-17 cells in the inflamed liver J Hepatol 57 2012 1044 1051
67. M.C. Barth, N. Ahluwalia, T.J. Anderson, G.J. Hardy, S. Sinha, and J.A. Alvarez-Cardona Kynurenic acid triggers firm arrest of leukocytes to vascular endothelium under flow conditions J Biol Chem 284 2009 19189 19195
68. C.Y. Ponsioen Novel developments in IBD-related sclerosing cholangitis Best Pract Res Clin Gastroenterol 25 2011 S15 S18
69. L.J. de Buy Wenniger, M.E. Doorenspleet, P.L. Klarenbeek, J. Verheij, F. Baas, and R.P. Elferink IgG4+ clones identified by next-generation sequencing dominate the b-cell receptor repertoire in IgG4-associated cholangitis Hepatology 2013 10.1002/hep.26232 in press
70. L. Maillette de Buy Wenniger, E.A. Rauws, and U. Beuers What an endoscopist should know about immunoglobulin-G4-associated disease of the pancreas and biliary tree Endoscopy 44 2012 66 73
71. P. Dite, I. Novotny, J. Trna, and A. Sevcikova Autoimmune pancreatitis Best Pract Res Clin Gastroenterol 22 2008 131 143
72. S.H. Moon, M.H. Kim, H. Park do, T.J. Song, J. Eum, and S.S. Lee IgG4 immunostaining of duodenal papillary biopsy specimens may be useful for supporting a diagnosis of autoimmune pancreatitis Gastrointest Endosc 71 2010 960 966
73. K. Vosskuhl, A.A. Negm, T. Framke, T. Weismuller, M.P. Manns, and H. Wedemeyer Measurement of IgG4 in bile: a new approach for the diagnosis of IgG4-associated cholangiopathy Endoscopy 44 2012 48 52
74. A.M. Oseini, R. Chaiteerakij, A.M. Shire, A. Ghazale, J. Kaiya, and C.D. Moser Utility of serum immunoglobulin G4 in distinguishing immunoglobulin G4-associated cholangitis from cholangiocarcinoma Hepatology 54 2011 940 948
75. E. Bjornsson, S. Chari, M. Silveira, A. Gossard, N. Takahashi, and T. Smyrk Primary sclerosing cholangitis associated with elevated immunoglobulinG4: clinical characteristics and response to therapy Am J Ther 18 2010 198 205
76. L.E. Lamberts, M. Janse, E.B. Haagsma, A.P. van den Berg, and R.K. Weersma Immune-mediated diseases in primary sclerosing cholangitis Dig Liver Dis 43 2011 802 806
77. J.S. Halliday, J. Djordjevic, M. Lust, E.L. Culver, B. Braden, and S.P. Travis A unique clinical phenotype of primary sclerosing cholangitis associated with Crohn's disease J Crohn's Colitis 6 2012 174 181
78. C. Rupp, A. Mummelthei, P. Sauer, K.H. Weiss, P. Schirmacher, and A. Stiehl Non-IBD immunological diseases are a risk factor for reduced survival in PSC Liver Int 33 2013 86 93
79. J.H. Ngu, R.B. Gearry, A.J. Wright, and C.A. Stedman Inflammatory bowel disease is associated with poor outcomes of patients with primary sclerosing cholangitis Clin Gastroenterol Hepatol 9 2011 1092 1097
80. G. Rudolph, D. Gotthardt, P. Kloeters-Plachky, D. Rost, H. Kulaksiz, and A. Stiehl In PSC with dominant bile duct stenosis, IBD is associated with an increase of carcinomas and reduced survival J Hepatol 53 2010 313 317
81. U. Broome, and A. Bergquist Primary sclerosing cholangitis, inflammatory bowel disease, and colon cancer Semin Liver Dis 26 2006 31 41
82. M.M. Claessen, M.W. Lutgens, H.R. van Buuren, B. Oldenburg, P.C. Stokkers, and C.J. van der Woude More right-sided IBD-associated colorectal cancer in patients with primary sclerosing cholangitis Inflamm Bowel Dis 15 2009 1331 1336
83. A. Bergquist, S.M. Montgomery, S. Bahmanyar, R. Olsson, A. Danielsson, and S. Lindgren Increased risk of primary sclerosing cholangitis and ulcerative colitis in first-degree relatives of patients with primary sclerosing cholangitis Clin Gastroenterol Hepatol 6 2008 939 943
84. L. Lindstrom, A. Lapidus, A. Ost, and A. Bergquist Increased risk of colorectal cancer and dysplasia in patients with Crohn's colitis and primary sclerosing cholangitis Dis Colon Rectum 54 2011 1392 1397
85. E.V. Loftus Jr., G.C. Harewood, C.G. Loftus, W.J. Tremaine, W.S. Harmsen, and A.R. Zinsmeister PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis Gut 54 2005 91 96
86. M.H. Chapman, G.J. Webster, S. Bannoo, G.J. Johnson, J. Wittmann, and S.P. Pereira Cholangiocarcinoma and dominant strictures in patients with primary sclerosing cholangitis: a 25-year single-centre experience Eur J Gastroenterol Hepatol 24 2012 1051 1058
87. J. Fevery, L. Henckaerts, R. Van Oirbeek, S. Vermeire, P. Rutgeerts, and F. Nevens Malignancies and mortality in 200 patients with primary sclerosering cholangitis: a long-term single-centre study Liver Int 32 2012 214 222
88. K.M. Boberg, A. Bergquist, S. Mitchell, A. Pares, F. Rosina, and U. Broome Cholangiocarcinoma in primary sclerosing cholangitis: risk factors and clinical presentation Scand J Gastroenterol 37 2002 1205 1211
89. K.M. Boberg, and G.E. Lind Primary sclerosing cholangitis and malignancy Best Pract Res Clin Gastroenterol 25 2011 753 764
90. N. Chalasani, A. Baluyut, A. Ismail, A. Zaman, G. Sood, and R. Ghalib Cholangiocarcinoma in patients with primary sclerosing cholangitis: a multicenter case-control study Hepatology 31 2000 7 11
91. K. Said, H. Glaumann, and A. Bergquist Gallbladder disease in patients with primary sclerosing cholangitis J Hepatol 48 2008 598 605
92. T.H. Karlsen, E. Schrumpf, and K.M. Boberg Gallbladder polyps in primary sclerosing cholangitis: not so benign Curr Opin Gastroenterol 24 2008 395 399
93. C. Schramm, and A.W. Lohse Gallbladder polyps in primary sclerosing cholangitis: indication for early intervention Hepatology 56 2012 396
94. J.E. Eaton, E.W. Thackeray, and K.D. Lindor Likelihood of malignancy in gallbladder polyps and outcomes following cholecystectomy in primary sclerosing cholangitis Am J Gastroenterol 107 2012 431 439
95. E. Sinakos, A.K. Saenger, J. Keach, W.R. Kim, and K.D. Lindor Many patients with primary sclerosing cholangitis and increased serum levels of carbohydrate antigen 19-9 do not have cholangiocarcinoma Clin Gastroenterol Hepatol 9 2011 e431
96. N. Razumilava, and G.J. Gores Classification, diagnosis, and management of cholangiocarcinoma Clin Gastroenterol Hepatol 11 2013 e11
97. H. Narimatsu, H. Iwasaki, F. Nakayama, Y. Ikehara, T. Kudo, and S. Nishihara Lewis and secretor gene dosages affect CA19-9 and DU-PAN-2 serum levels in normal individuals and colorectal cancer patients Cancer Res 58 1998 512 518
98. L. Halme, J. Arola, K. Numminen, L. Krogerus, H. Makisalo, and M. Farkkila Biliary dysplasia in patients with primary sclerosing cholangitis: additional value of DNA ploidity Liver Int 32 2012 783 789
99. E.G. Barr Fritcher, B.R. Kipp, J.S. Voss, A.C. Clayton, K.D. Lindor, and K.C. Halling Primary sclerosing cholangitis patients with serial polysomy fluorescence in situ hybridization results are at increased risk of cholangiocarcinoma Am J Gastroenterol 106 2011 2023 2028
100. E.G. Barr Fritcher, J.L. Caudill, J.E. Blue, K. Djuric, L. Feipel, and B.K. Maritim Identification of malignant cytologic criteria in pancreatobiliary brushings with corresponding positive fluorescence in situ hybridization results Am J Clin Pathol 136 2011 442 449
101. B.R. Kipp, L.M. Stadheim, S.A. Halling, N.L. Pochron, S. Harmsen, and D.M. Nagorney A comparison of routine cytology and fluorescence in situ hybridization for the detection of malignant bile duct strictures Am J Gastroenterol 99 2004 1675 1681
102. P. Charatcharoenwitthaya, F.B. Enders, K.C. Halling, and K.D. Lindor Utility of serum tumor markers, imaging, and biliary cytology for detecting cholangiocarcinoma in primary sclerosing cholangitis Hepatology 48 2008 1106 1117
103. K.M. Boberg, P. Jebsen, O.P. Clausen, A. Foss, L. Aabakken, and E. Schrumpf Diagnostic benefit of biliary brush cytology in cholangiocarcinoma in primary sclerosing cholangitis J Hepatol 45 2006 568 574
104. S.Y. Bangarulingam, E. Bjornsson, F. Enders, E.G. Barr Fritcher, G. Gores, and K.C. Halling Long-term outcomes of positive fluorescence in situ hybridization tests in primary sclerosing cholangitis Hepatology 51 2010 174 180
105. J.T. Lewis, J.A. Talwalkar, C.B. Rosen, T.C. Smyrk, and S.C. Abraham Precancerous bile duct pathology in end-stage primary sclerosing cholangitis, with and without cholangiocarcinoma Am J Surg Pathol 34 2010 27 34
106. M.H. Imam, A.A. Gossard, E. Sinakos, and K.D. Lindor Pathogenesis and management of pruritus in cholestatic liver disease J Gastroenterol Hepatol 27 2012 1150 1158
107. A. Pares, M. Herrera, J. Aviles, M. Sanz, and A. Mas Treatment of resistant pruritus from cholestasis with albumin dialysis: combined analysis of patients from three centers J Hepatol 53 2010 307 312
108. C. Pauli-Magnus, P.J. Meier, and B. Stieger Genetic determinants of drug-induced cholestasis and intrahepatic cholestasis of pregnancy Semin Liver Dis 30 2010 147 159
109. M. Gay, A. Pares, M. Carrascal, P. Bosch-i-Crespo, M. Gorga, and A. Mas Proteomic analysis of polypeptides captured from blood during extracorporeal albumin dialysis in patients with cholestasis and resistant pruritus PLoS One 6 2011 e21850
110. A.E. Kremer, J.J. Martens, W. Kulik, F. Rueff, E.M. Kuiper, and H.R. van Buuren Lysophosphatidic acid is a potential mediator of cholestatic pruritus Gastroenterology 139 2010 1008 1018 e1001
111. F. Alemi, E. Kwon, D.P. Poole, T. Lieu, V. Lyo, and F. Cattaruzza The TGR5 receptor mediates bile acid-induced itch and analgesia J Clin Invest 123 2013 1513 1530
112. K.M. Boberg, R.W. Chapman, G.M. Hirschfield, A.W. Lohse, M.P. Manns, and E. Schrumpf Overlap syndromes: The International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue J Hepatol 54 2010 374 385
113. E.M. Hennes, M. Zeniya, A.J. Czaja, A. Pares, G.N. Dalekos, and E.L. Krawitt Simplified criteria for the diagnosis of autoimmune hepatitis Hepatology 48 2008 169 176
114. European Association for the Study of the Liver EASL clinical practice guidelines: management of cholestatic liver diseases J Hepatol 51 2009 237 267
115. S. Luth, S. Kanzler, C. Frenzel, H.U. Kasper, H.P. Dienes, and C. Schramm Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome J Clin Gastroenterol 43 2008 75 80
116. T. Al-Chalabi, B.C. Portmann, W. Bernal, I.G. McFarlane, and M.A. Heneghan Autoimmune hepatitis overlap syndromes: an evaluation of treatment response, long-term outcome and survival Aliment Pharmacol Ther 28 2008 209 220
117. R. Chapman, J. Fevery, A. Kalloo, D.M. Nagorney, K.M. Boberg, and B. Shneider Diagnosis and management of primary sclerosing cholangitis Hepatology 51 2010 660 678
118. J.M. Farrant, K.M. Hayllar, M.L. Wilkinson, J. Karani, B.C. Portmann, and D. Westaby Natural history and prognostic variables in primary sclerosing cholangitis Gastroenterology 100 1991 1710 1717
119. J.C. Chambers, W. Zhang, J. Sehmi, X. Li, M.N. Wass, and P. Van der Harst Genome-wide association study identifies loci influencing concentrations of liver enzymes in plasma Nat Genet 43 2011 1131 1138
120. T. Folseraas, E. Melum, P. Rausch, B.D. Juran, E. Ellinghaus, and A. Shiryaev Extended analysis of a genome-wide association study in primary sclerosing cholangitis detects multiple novel risk loci J Hepatol 57 2012 366 375
121. S. Al Mamari, J. Djordjevic, J.S. Halliday, and R.W. Chapman 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 58 2013 329 334
122. L. Lindstrom, R. Hultcrantz, K.M. Boberg, I. Friis-Liby, and A. Bergquist Association between reduced levels of alkaline phosphatase and survival times of patients with primary sclerosing cholangitis Clin Gastroenterol Hepatol 2013 10.1016/j.cgh.2012.12.032 in press
123. P.P. Stanich, E. Bjornsson, A.A. Gossard, F. Enders, R. Jorgensen, and K.D. Lindor Alkaline phosphatase normalization is associated with better prognosis in primary sclerosing cholangitis Dig Liver Dis 43 2011 309 313
124. E. Sinakos, H.U. Marschall, K.V. Kowdley, A. Befeler, J. Keach, and K. Lindor Bile acid changes after high-dose ursodeoxycholic acid treatment in primary sclerosing cholangitis: Relation to disease progression Hepatology 52 2010 197 203
125. D. Rost, G. Rudolph, P. Kloeters-Plachky, and A. Stiehl Effect of high-dose ursodeoxycholic acid on its biliary enrichment in primary sclerosing cholangitis Hepatology 40 2004 693 698
126. P.J. Turnbaugh Microbiology: fat, bile and gut microbes Nature 487 2012 47 48
127. V. Tremaroli, and F. Backhed Functional interactions between the gut microbiota and host metabolism Nature 489 2012 242 249
128. H. Duboc, S. Rajca, D. Rainteau, D. Benarous, M.A. Maubert, and E. Quervain Connecting dysbiosis, bile-acid dysmetabolism and gut inflammation in inflammatory bowel diseases Gut 62 2013 531 539
129. G. Kakiyama, W.M. Pandak, P.M. Gillevet, P.B. Hylemon, D.M. Heuman, and K. Daita Modulation of the Fecal Bile Acid Profile by Gut Microbiota in Cirrhosis J Hepatol 58 2013 949 955
130. J. Henao-Mejia, E. Elinav, C. Jin, L. Hao, W.Z. Mehal, and T. Strowig Inflammasome-mediated dysbiosis regulates progression of NAFLD and obesity Nature 482 2012 179 185
131. D.N. Abarbanel, S.M. Seki, Y. Davies, N. Marlen, J.A. Benavides, and K. Cox Immunomodulatory effect of vancomycin on treg in pediatric inflammatory bowel disease and primary sclerosing cholangitis J Clin Immunol 33 2013 397 406
132. M. Farkkila, A.L. Karvonen, H. Nurmi, H. Nuutinen, M. Taavitsainen, and P. Pikkarainen Metronidazole and ursodeoxycholic acid for primary sclerosing cholangitis: a randomized placebo-controlled trial Hepatology 40 2004 1379 1386
133. A.L. Boner, D. Peroni, A. Bodini, G. Delaini, and G. Piacentini Azithromycin may reduce cholestasis in primary sclerosing cholangitis: a case report and serendipitous observation Int J Immunopathol Pharm 20 2007 847 849
134. M.G. Silveira, N.J. Torok, A.A. Gossard, J.C. Keach, R.A. Jorgensen, and J.L. Petz Minocycline in the treatment of patients with primary sclerosing cholangitis: results of a pilot study Am J Gastroenterol 104 2009 83 88
135. W.J. Sandborn, R.H. Wiesner, W.J. Tremaine, and N.F. Larusso Ulcerative colitis disease activity following treatment of associated primary sclerosing cholangitis with cyclosporin Gut 34 1993 242 246
136. J.A. Talwalkar, A.A. Gossard, J.C. Keach, R.A. Jorgensen, J.L. Petz, and R.N. Lindor Tacrolimus for the treatment of primary sclerosing cholangitis Liver Int 27 2007 451 453
137. K.K. Jorgensen, L. Lindstrom, M. Cvancarova, T.H. Karlsen, M. Castedal, and S. Friman Immunosuppression after liver transplantation for primary sclerosing cholangitis influences activity of inflammatory bowel disease Clin Gastroenterol Hepatol 11 2013 517 523
138. -/- mice by inhibition of cholangiocyte inflammatory response and portal myofibroblast proliferation Gut 59 2010 521 530
139. M. Trauner, A. Baghdasaryan, T. Claudel, P. Fickert, E. Halilbasic, and T. Moustafa Targeting nuclear bile acid receptors for liver disease Dig Dis 29 2011 98 102
140. K.E. Nakken, S. Nygard, T. Haaland, K.E. Berge, K. Arnkvaern, and A. Odegaard Multiple inflammatory-, tissue remodelling- and fibrosis genes are differentially transcribed in the livers of Abcb4 (-/-) mice harbouring chronic cholangitis Scand J Gastroenterol 42 2007 1245 1255
141. N.S. Sandanayake, J. Sinclair, F. Andreola, M.H. Chapman, A. Xue, and G.J. Webster A combination of serum leucine-rich alpha-2-glycoprotein 1, CA19-9 and interleukin-6 differentiate biliary tract cancer from benign biliary strictures Br J Cancer 105 2011 1370 1378
142. T.O. Lankisch, J. Metzger, A.A. Negm, K. Vosskuhl, E. Schiffer, and J. Siwy Bile proteomic profiles differentiate cholangiocarcinoma from primary sclerosing cholangitis and choledocholithiasis Hepatology 53 2011 875 884
143. J. Metzger, A.A. Negm, R.R. Plentz, T.J. Weismuller, J. Wedemeyer, and T.H. Karlsen Urine proteomic analysis differentiates cholangiocarcinoma from primary sclerosing cholangitis and other benign biliary disorders Gut 62 2013 122 130
144. B.H. Kim, N.Y. Cho, M. Choi, S. Lee, J.J. Jang, and G.H. Kang Methylation profiles of multiple CpG island loci in extrahepatic cholangiocarcinoma versus those of intrahepatic cholangiocarcinomas Arch Pathol Lab Med 131 2007 923 930
145. J.B. Rawson, and B. Bapat Epigenetic biomarkers in colorectal cancer diagnostics Expert Rev Mol Diagn 12 2012 499 509
146. K. Andresen, K.M. Boberg, H.M. Vedeld, H. Honne, M. Hektoen, and C.A. Wadsworth Novel target genes and a valid biomarker panel identified for cholangiocarcinoma Epigenetics 7 2012 1249 1257
147. B. Fosby, T.H. Karlsen, and E. Melum Recurrence and rejection in liver transplantation for primary sclerosing cholangitis World J Gastroenterol 18 2012 1 15
148. E. Alabraba, P. Nightingale, B. Gunson, S. Hubscher, S. Olliff, and D. Mirza A re-evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts Liver Transpl 15 2009 330 340
149. S. Darwish Murad, W.R. Kim, D.M. Harnois, D.D. Douglas, J. Burton, and L.M. Kulik Efficacy of neoadjuvant chemoradiation, followed by liver transplantation, for perihilar cholangiocarcinoma at 12 US centers Gastroenterology 143 2012 88 98 e83; quiz e14
150. C.B. Rosen, S. Darwish Murad, J.K. Heimbach, S.L. Nyberg, D.M. Nagorney, and G.J. Gores Neoadjuvant therapy and liver transplantation for hilar cholangiocarcinoma: is pretreatment pathological confirmation of diagnosis necessary? J Am Coll Surg 215 2012 31 38
151. S. Darwish Murad, W.R. Kim, T. Therneau, G.J. Gores, C.B. Rosen, and J.A. Martenson Predictors of pretransplant dropout and posttransplant recurrence in patients with perihilar cholangiocarcinoma Hepatology 56 2012 972 981
152. S. Friman, A. Foss, H. Isoniemi, M. Olausson, K. Hockerstedt, and S. Yamamoto Liver transplantation for cholangiocarcinoma: selection is essential for acceptable results Scand J Gastroenterol 46 2011 370 375
153. D.S. Goldberg, A. Camp, A. Martinez-Camacho, L. Forman, B. Fortune, and K.R. Reddy Risk of waitlist mortality in patients with primary sclerosing cholangitis and bacterial cholangitis Liver transplantation: official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society 19 2013 250 258
154. W.A. Faubion Jr., E.V. Loftus, W.J. Sandborn, D.K. Freese, and J. Perrault Pediatric "PSC-IBD": a descriptive report of associated inflammatory bowel disease among pediatric patients with PSC J Pediatr Gastroenterol Nutr 33 2001 296 300
155. M. Joo, P. Abreu-e-Lima, F. Farraye, T. Smith, P. Swaroop, and L. Gardner Pathologic features of ulcerative colitis in patients with primary sclerosing cholangitis: a case-control study Am J Surg Pathol 33 2009 854 862
156. K.K. Jorgensen, K. Grzyb, K.E. Lundin, O.P. Clausen, G. Aamodt, and E. Schrumpf Inflammatory bowel disease in patients with primary sclerosing cholangitis: Clinical characterization in liver transplanted and nontransplanted patients Inflamm Bowel Dis 18 2012 536 545
157. B.D. Ye, S.K. Yang, S.J. Boo, Y.K. Cho, D.H. Yang, and S.M. Yoon Clinical characteristics of ulcerative colitis associated with primary sclerosing cholangitis in Korea Inflamm Bowel Dis 17 2011 1901 1906
158. L. Jostins, S. Ripke, R.K. Weersma, R.H. Duerr, D.P. McGovern, and K.Y. Hui Host-microbe interactions have shaped the genetic architecture of inflammatory bowel disease Nature 491 2012 119 124.