Update on primary sclerosing cholangitis

Digestive and Liver Disease

Volumn 42, Issue 6, 2010, Pages 390-400

  Karlsen, Tom H ^a   Schrumpf, Erik ^a   Boberg, Kirsten M ^a  

^a OSLO UNIVERSITY HOSPITAL   (Norway)

Author keywords

Cholangiocarcinoma; HLA genes; Inflammatory bowel disease; Primary sclerosing cholangitis

Indexed keywords

BILE ACID; CORTICOSTEROID; IMMUNOGLOBULIN G4; PLACEBO; TUMOR NECROSIS FACTOR ALPHA ANTIBODY; URSODEOXYCHOLIC ACID; CHOLAGOGUE; IMMUNOSUPPRESSIVE AGENT;

AUTOIMMUNITY; BALLOON DILATATION; BILE DUCT CARCINOMA; CHOLANGIOGRAPHY; CLINICAL TRIAL; DRUG MEGADOSE; ENDOSCOPIC THERAPY; GENETIC ASSOCIATION; GENETIC RISK; GENETIC SUSCEPTIBILITY; HUMAN; IMMUNOHISTOCHEMISTRY; LIVER BIOPSY; LIVER TRANSPLANTATION; LYMPHOCYTE HOMING; NONHUMAN; NUCLEAR MAGNETIC RESONANCE IMAGING; PATHOGENESIS; PRIMARY SCLEROSING CHOLANGITIS; PRIORITY JOURNAL; RISK FACTOR; SHORT SURVEY; CHOLANGITIS, SCLEROSING; COMPUTER ASSISTED TOMOGRAPHY; DIAGNOSTIC IMAGING; DIFFERENTIAL DIAGNOSIS; DISEASE COURSE; ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY; GENETIC PREDISPOSITION; PRACTICE GUIDELINE; PROCEDURES; PROGNOSIS;

AUTOIMMUNITY; CHOLAGOGUES AND CHOLERETICS; CHOLANGIOPANCREATOGRAPHY, ENDOSCOPIC RETROGRADE; CHOLANGITIS, SCLEROSING; DIAGNOSIS, DIFFERENTIAL; DIAGNOSTIC IMAGING; DISEASE PROGRESSION; GENETIC PREDISPOSITION TO DISEASE; HUMANS; IMMUNOSUPPRESSIVE AGENTS; LIVER TRANSPLANTATION; PRACTICE GUIDELINES AS TOPIC; PROGNOSIS; TOMOGRAPHY, X-RAY COMPUTED; URSODEOXYCHOLIC ACID;

EID: 77952742582     PISSN: 15908658     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.dld.2010.01.011     Document Type: Short Survey

References (223)

1. Ludwig J., MacCarty R.L., LaRusso N.F., et al. Intrahepatic cholangiectases and large-duct obliteration in primary sclerosing cholangitis. Hepatology 1986, 6:560-568.
2. Wiesner R.H., LaRusso N.F. Clinicopathologic features of the syndrome of primary sclerosing cholangitis. Gastroenterology 1980, 79:200-206.
3. Olsson R., Hagerstrand I., Broome U., et al. Sampling variability of percutaneous liver biopsy in primary sclerosing cholangitis. J Clin Pathol 1995, 48:933-935.
4. Tischendorf J.J., Hecker H., Kruger M., et al. Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: a single center study. Am J Gastroenterol 2007, 102:107-114.
5. Bjornsson E., Lindqvist-Ottosson J., Asztely M., et al. Dominant strictures in patients with primary sclerosing cholangitis. Am J Gastroenterol 2004, 99:502-508.
6. Rudolph G., Gotthardt D., Kloters-Plachky P., et al. Influence of dominant bile duct stenoses and biliary infections on outcome in primary sclerosing cholangitis. J Hepatol 2009, 51:149-155.
7. Porayko M.K., LaRusso N.F. Wiesner RH Primary sclerosing cholangitis: a progressive disease?. Semin Liver Dis 1991, 11:18-25.
8. Wiesner R.H. Liver transplantation for primary sclerosing cholangitis: timing, outcome, impact of inflammatory bowel disease and recurrence of disease. Best Pract Res Clin Gastroenterol 2001, 15:667-680.
9. Farrant J.M., Hayllar K.M., Wilkinson M.L., et al. Natural history and prognostic variables in primary sclerosing cholangitis. Gastroenterology 1991, 100:1710-1717.
10. Broome U., Olsson R., Loof L., et al. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis. Gut 1996, 38:610-615.
11. Aadland E., Schrumpf E., Fausa O., et al. Primary sclerosing cholangitis: a long-term follow-up study. Scand J Gastroenterol 1987, 22:655-664.
12. Boberg K.M., Aadland E., Jahnsen J., et al. Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol 1998, 33:99-103.
13. Kingham J.G., Kochar N., Gravenor M.B. Incidence, clinical patterns, and outcomes of primary sclerosing cholangitis in South Wales, United Kingdom. Gastroenterology 2004, 126:1929-1930.
14. Bambha K., Kim W.R., Talwalkar J., et al. Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community. Gastroenterology 2003, 125:1364-1369.
15. Escorsell A., Pares A., Rodes J., et al. Epidemiology of primary sclerosing cholangitis in Spain. J Hepatol 1994, 21:787-791.
16. Ang T.L., Fock K.M., Ng T.M., et al. Clinical profile of primary sclerosing cholangitis in Singapore. J Gastroenterol Hepatol 2002, 17:908-913.
17. Okolicsanyi L., Fabris L., Viaggi S., et al. Primary sclerosing cholangitis: clinical presentation, natural history and prognostic variables: an Italian multicentre study. The Italian PSC Study Group. Eur J Gastroenterol Hepatol 1996, 8:685-691.
18. Kochhar R., Goenka M.K., Das K., et al. Primary sclerosing cholangitis: an experience from India. J Gastroenterol Hepatol 1996, 11:429-433.
19. Takikawa H., Takamori Y., Tanaka A., et al. Analysis of 388 cases of primary sclerosing cholangitis in Japan; Presence of a subgroup without pancreatic involvement in older patients. Hepatol Res 2004, 29:153-159.
20. Takikawa H., Manabe T. Primary sclerosing cholangitis in Japan-analysis of 192 cases. J Gastroenterol 1997, 32:134-137.
21. Fausa O., Schrumpf E., Elgjo K. Relationship of inflammatory bowel disease and primary sclerosing cholangitis. Semin Liver Dis 1991, 11:31-39.
22. Saarinen S., Olerup O., Broome U. Increased frequency of autoimmune diseases in patients with primary sclerosing cholangitis. Am J Gastroenterol 2000, 95:3195-3199.
23. Kaya M., Angulo P., Lindor K.D. Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system. J Hepatol 2000, 33:537-542.
24. Alvarez F., Berg P.A., Bianchi F.B., et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 1999, 31:929-938.
25. van Buuren H.R., van Hoogstraten H.J.E., Terkivatan T., et al. High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis. J Hepatol 2000, 33:543-548.
26. Miros M., Kerlin P., Walker N., et al. Predicting cholangiocarcinoma in patients with primary sclerosing cholangitis before transplantation. Gut 1991, 32:1369-1373.
27. Helzberg J.H., Petersen J.M., Boyer J.L. Improved survival with primary sclerosing cholangitis. A review of clinicopathologic features and comparison of symptomatic and asymptomatic patients. Gastroenterology 1987, 92:1869-1875.
28. Ataseven H., Parlak E., Yuksel I., et al. Primary sclerosing cholangitis in Turkish patients: characteristic features and prognosis. Hepatobiliary Pancreat Dis Int 2009, 8:312-315.
29. Karlsen T.H., Schrumpf E., Boberg K.M. Gallbladder polyps in primary sclerosing cholangitis: not so benign. Curr Opin Gastroenterol 2008, 24:395-399.
30. Soetikno R.M., Lin O.S., Heidenreich P.A., et al. Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis: a meta-analysis. Gastrointest Endosc 2002, 56:48-54.
31. Guicciardi M.E., Gores G.J. Cholestatic hepatocellular injury: what do we know and how should we proceed. J Hepatol 2005, 42:297-300.
32. Abdalian R., Heathcote E.J. Sclerosing cholangitis: a focus on secondary causes. Hepatology 2006, 44:1063-1074.
33. Bergquist A., Montgomery S.M., Bahmanyar S., et al. Increased risk of primary sclerosing cholangitis and ulcerative colitis in first-degree relatives of patients with primary sclerosing cholangitis. Clin Gastroenterol Hepatol 2008, 6:939-943.
34. Karlsen TH, Franke A, Melum E, et al. Genome-wide association analysis in primary sclerosing cholangitis. Gastroenterology, doi:10.1053/j.gastro.2009.11.046.
35. Schrumpf E., Fausa O., Forre O., et al. HLA antigens and immunoregulatory T cells in ulcerative colitis associated with hepatobiliary disease. Scand J Gastroenterol 1982, 17:187-191.
36. Karlsen T.H., Boberg K.M., Vatn M., et al. Different HLA class II associations in ulcerative colitis patients with and without primary sclerosing cholangitis. Genes Immun 2007, 8:275-278.
37. Karlsen T.H., Boberg K.M., Olsson M., et al. Particular genetic variants of ligands for natural killer cell receptors may contribute to the HLA associated risk of primary sclerosing cholangitis. J Hepatol 2007, 46:899-906.
38. Hov JR, Lleo A, Selmi C, et al. Genetic associations in Italian primary sclerosing cholangitis: heterogeneity across Europe defines a critical role for HLA-C. J Hepatol; in press.
39. Guinan K.J., Cunningham R.T., Meenagh A., et al. Receptor systems controlling natural killer cell function are genetically stratified in Europe. Genes Immun 2010, 11:67-78.
40. Donaldson P.T., Norris S. Evaluation of the role of MHC class II alleles, haplotypes and selected amino acid sequences in primary sclerosing cholangitis. Autoimmunity 2002, 35:555-564.
41. Wiencke K., Karlsen T.H., Boberg K.M., et al. Primary sclerosing cholangitis is associated with extended HLA-DR3 and HLA-DR6 haplotypes. Tissue Antigens 2007, 69:161-169.
42. Spurkland A., Saarinen S., Boberg K.M., et al. HLA class II haplotypes in primary sclerosing cholangitis patients from five European populations. Tissue Antigens 1999, 53:459-469.
43. Loftus E.V., Harewood G.C., Loftus C.G., et al. PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis. Gut 2005, 54:91-96.
44. Oshitani N., Jinno Y., Sawa Y., et al. Does colitis associated with primary sclerosing cholangitis represent an actual subset of ulcerative colitis?. Hepatogastroenterology 2003, 50:1830-1835.
45. Joo M., Abreu-e-Lima P., Farraye F., et al. Pathologic features of ulcerative colitis in patients with primary sclerosing cholangitis: a case-control study. Am J Surg Pathol 2009, 33:854-862.
46. Abdelrazeq A.S., Wilson T.R., Leitch D.L., et al. Ileitis in ulcerative colitis: is it a backwash?. Dis Colon Rectum 2005, 48:2038-2046.
47. Zhou Y.Q., Chen Y.Q., Fisher J.H., et al. Activation of the RON receptor tyrosine kinase by macrophage-stimulating protein inhibits inducible cyclooxygenase-2 expression in murine macrophages. J Biol Chem 2002, 277:38104-38110.
48. Goyette P., Lefebvre C., Ng A., et al. Gene-centric association mapping of chromosome 3p implicates MST1 in IBD pathogenesis. Mucosal Immunol 2008, 1:131-138.
49. Keitel V., Donner M., Winandy S., et al. Expression and function of the bile acid receptor TGR5 in Kupffer cells. Biochem Biophys Res Commun 2008, 372:78-84.
50. Keitel V., Cupisti K., Ullmer C., et al. The membrane-bound bile acid receptor TGR5 is localized in the epithelium of human gallbladders. Hepatology 2009, 50:861-870.
51. Beuers U. Drug insight: mechanisms and sites of action of ursodeoxycholic acid in cholestasis. Nat Clin Pract Gastroenterol Hepatol 2006, 3:318-328.
52. Blanco P.G., Zaman M.M., Junaidi O., et al. Induction of colitis in cftr-/- mice results in bile duct injury. Am J Physiol Gastrointest Liver Physiol 2004, 287:G491-G496.
53. Sheth S., Shea J.C., Bishop M.D., et al. Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis. Hum Genet 2003, 113:286-292.
54. Girodon E., Sternberg D., Chazouilleres O., et al. Cystic fibrosis transmembrane conductance regulator (CFTR) gene defects in patients with primary sclerosing cholangitis. J Hepatol 2002, 37:192-197.
55. Henckaerts L., Jaspers M., Van Steenbergen W., et al. Cystic fibrosis transmembrane conductance regulator gene polymorphisms in patients with primary sclerosing cholangitis. J Hepatol 2009, 50:150-157.
56. Pall H., Zielenski J., Jonas M.M., et al. Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function. J Pediatr 2007, 151:255-259.
57. Gallegos-Orozco J.F., EY C., Wang N., et al. Lack of association of common cystic fibrosis transmembrane conductance regulator gene mutations with primary sclerosing cholangitis. Am J Gastroenterol 2005, 100:874-878.
58. Hashimoto E., Lindor K.D., Homburger H.A., et al. Immunohistochemical characterization of hepatic lymphocytes in primary biliary cirrhosis in comparison with primary sclerosing cholangitis and autoimmune chronic active hepatitis. Mayo Clin Proc 1993, 68:1049-1055.
59. Hata K., Van Thiel D.H., Herberman R.B., et al. Phenotypic and functional characteristics of lymphocytes isolated from liver biopsy specimens from patients with active liver disease. Hepatology 1992, 15:816-823.
60. Cameron R.G., Blendis L.M., Neuman M.G. Accumulation of macrophages in primary sclerosing cholangitis. Clin Biochem 2001, 34:195-201.
61. Whiteside T.L., Lasky S., Si L., et al. Immunologic analysis of mononuclear cells in liver tissues and blood of patients with primary sclerosing cholangitis. Hepatology 1985, 5:468-474.
62. Ponsioen C.Y., Kuiper H., Ten Kate F.J., et al. Immunohistochemical analysis of inflammation in primary sclerosing cholangitis. Eur J Gastroenterol Hepatol 1999, 11:769-774.
63. Adams D.H., Eksteen B. Aberrant homing of mucosal T cells and extra-intestinal manifestations of inflammatory bowel disease. Nat Rev Immunol 2006, 6:244-251.
64. Grant A.J., Lalor P.F., Salmi M., et al. Homing of mucosal lymphocytes to the liver in the pathogenesis of hepatic complications of inflammatory bowel disease. Lancet 2002, 359:150-157.
65. Hillan K.J., Hagler K.E., MacSween R.N., et al. Expression of the mucosal vascular addressin, MAdCAM-1, in inflammatory liver disease. Liver 1999, 19:509-518.
66. Grant A.J., Lalor P.F., Hubscher S.G., et al. MAdCAM-1 expressed in chronic inflammatory liver disease supports mucosal lymphocyte adhesion to hepatic endothelium (MAdCAM-1 in chronic inflammatory liver disease). Hepatology 2001, 33:1065-1072.
67. Eksteen B., Grant A.J., 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, 200:1511-1517.
68. Chapman R.W., Cottone M., Selby W.S., et al. Serum autoantibodies, ulcerative colitis and primary sclerosing cholangitis. Gut 1986, 27:86-91.
69. Mandal A., Dasgupta A., Jeffers L., et al. Autoantibodies in sclerosing cholangitis against a shared peptide in biliary and colon epithelium. Gastroenterology 1994, 106:185-192.
70. Xu B., Broome U., Ericzon B.G., et al. High frequency of autoantibodies in patients with primary sclerosing cholangitis that bind biliary epithelial cells and induce expression of CD44 and production of interleukin 6. Gut 2002, 51:120-127.
71. Broome U., Grunewald J., Scheynius A., et al. Preferential V beta3 usage by hepatic T lymphocytes in patients with primary sclerosing cholangitis. J Hepatol 1997, 26:527-534.
72. Terjung B., Herzog V., Worman H.J., et al. Atypical antineutrophil cytoplasmic antibodies with perinuclear fluorescence in chronic inflammatory bowel diseases and hepatobiliary disorders colocalize with nuclear lamina proteins. Hepatology 1998, 28:332-340.
73. Terjung B., Spengler U., Sauerbruch T., et al. " Atypical p-ANCA" in IBD and hepatobiliary disorders react with a 50-kilodalton nuclear envelope protein of neutrophils and myeloid cell lines. Gastroenterology 2000, 119:310-322.
74. Billing P., Tahir S., Calfin B., et al. Nuclear localization of the antigen detected by ulcerative colitis-associated perinuclear antineutrophil cytoplasmic antibodies. Am J Pathol 1995, 147:979-987.
75. Klein R., Eisenburg J., Weber P., et al. Significance and specificity of antibodies to neutrophils detected by western blotting for the serological diagnosis of primary sclerosing cholangitis. Hepatology 1991, 14:1147-1152.
76. Terjung B, Soehne J, Lechtenberg B, et al. p-ANCA in autoimmune liver disorders recognize human beta-tubulin isotype 5 and cross-react with microbial protein FtsZ. Gut, doi:10.1136/gut.2008.157818.
77. Smit J.J., Schinkel A.H., Oude Elferink R.P., et al. Homozygous disruption of the murine mdr2 P-glycoprotein gene leads to a complete absence of phospholipid from bile and to liver disease. Cell 1993, 75:451-462.
78. Trauner M., Fickert P., Wagner M. MDR3 (ABCB4) defects: a paradigm for the genetics of adult cholestatic syndromes. Semin Liver Dis 2007, 27:77-98.
79. Popov Y., Patsenker E., Fickert P., et al. Mdr2 (Abcb4)-/- mice spontaneously develop severe biliary fibrosis via massive dysregulation of pro- and antifibrogenic genes. J Hepatol 2005, 43:1045-1054.
80. 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, 127:261-274.
81. Fickert P., Zollner G., Fuchsbichler A., et al. Ursodeoxycholic acid aggravates bile infarcts in bile duct-ligated and Mdr2 knockout mice via disruption of cholangioles. Gastroenterology 2002, 123:1238-1251.
82. Nakken K.E., Nygard S., Haaland T., et al. Multiple inflammatory-, tissue remodelling- and fibrosis genes are differentially transcribed in the livers of Abcb4 (-/-) mice harbouring chronic cholangitis. Scand J Gastroenterol 2007, 42:1245-1255.
83. Jacquemin E. Role of multidrug resistance 3 deficiency in pediatric and adult liver disease: one gene for three diseases. Semin Liver Dis 2001, 21:551-562.
84. Ziol M., Barbu V., Rosmorduc O., et al. ABCB4 heterozygous gene mutations associated with fibrosing cholestatic liver disease in adults. Gastroenterology 2008, 135:131-141.
85. Gotthardt D., Runz H., Keitel V., et al. A mutation in the canalicular phospholipid transporter gene, ABCB4, is associated with cholestasis, ductopenia, and cirrhosis in adults. Hepatology 2008, 48:1157-1166.
86. Melum E., Boberg K.M., Franke A., et al. Variation in the MDR3 gene influences disease progression in PSC patients and disease susceptibility in epistatic interaction with polymorphism in OST-alpha gene. Hepatology 2007, 46:265A.
87. Ohishi Y., Nakamura M., Iio N., et al. Single-nucleotide polymorphism analysis of the multidrug resistance protein 3 gene for the detection of clinical progression in Japanese patients with primary biliary cirrhosis. Hepatology 2008, 48:853-862.
88. Palmer R.H. Bile acids, liver injury, and liver disease. Arch Intern Med 1972, 130:606-617.
89. Wagner M., Halilbasic E., Marschall H.U., et al. CAR and PXR agonists stimulate hepatic bile acid and bilirubin detoxification and elimination pathways in mice. Hepatology 2005, 42:420-430.
90. Parks D.J., Blanchard S.G., Bledsoe R.K., et al. Bile acids: natural ligands for an orphan nuclear receptor. Science 1999, 284:1365-1368.
91. Guo G.L., Lambert G., Negishi M., et al. Complementary roles of farnesoid X receptor, pregnane X receptor, and constitutive androstane receptor in protection against bile acid toxicity. J Biol Chem 2003, 278:45062-45071.
92. Staudinger J.L., Goodwin B., Jones S.A., et al. The nuclear receptor PXR is a lithocholic acid sensor that protects against liver toxicity. Proc Natl Acad Sci USA 2001, 98:3369-3374.
93. Stedman C.A., Liddle C., Coulter S.A., et al. Nuclear receptors constitutive androstane receptor and pregnane X receptor ameliorate cholestatic liver injury. Proc Natl Acad Sci USA 2005, 102:2063-2068.
94. Saini S.P., Mu Y., Gong H., et al. Dual role of orphan nuclear receptor pregnane X receptor in bilirubin detoxification in mice. Hepatology 2005, 41:497-505.
95. Xie W., Radominska-Pandya A., Shi Y., et al. An essential role for nuclear receptors SXR/PXR in detoxification of cholestatic bile acids. Proc Natl Acad Sci USA 2001, 98:3375-3380.
96. Karlsen T.H., Lie B.A., Frey Froslie K., et al. Polymorphisms in the steroid and xenobiotic receptor gene influence survival in primary sclerosing cholangitis. Gastroenterology 2006, 131:781-787.
97. Sookoian S., Castano G.O., Burgueno A.L., et al. The nuclear receptor PXR gene variants are associated with liver injury in nonalcoholic fatty liver disease. Pharmacogenet Genomics 2010, 20:1-8.
98. Poupon R., Ping C., Chretien Y., et al. Genetic factors of susceptibility and of severity in primary biliary cirrhosis. J Hepatol 2008, 49:1038-1045.
99. Lichtman S.N., Okoruwa E.E., Keku J., et al. Degradation of endogenous bacterial cell wall polymers by the muralytic enzyme mutanolysin prevents hepatobiliary injury in genetically susceptible rats with experimental intestinal bacterial overgrowth. J Clin Invest 1992, 90:1313-1322.
100. Lichtman S.N., Keku J., Schwab J.H., et al. Evidence for peptidoglycan absorption in rats with experimental small bowel bacterial overgrowth. Infect Immun 1991, 59:555-562.
101. Lichtman S.N., Keku J., Schwab J.H., et al. Hepatic injury associated with small bowel bacterial overgrowth in rats is prevented by metronidazole and tetracycline. Gastroenterology 1991, 100:513-519.
102. Lichtman S.N., Keku J., Clark R.L., et al. Biliary tract disease in rats with experimental small bowel bacterial overgrowth. Hepatology 1991, 13:766-772.
103. Lichtman S.N., Sartor R.B., Keku J., et al. Hepatic inflammation in rats with experimental small intestinal bacterial overgrowth. Gastroenterology 1990, 98:414-423.
104. Bo X., Broome U., Remberger M., et al. Tumour necrosis factor alpha impairs function of liver derived T lymphocytes and natural killer cells in patients with primary sclerosing cholangitis. Gut 2001, 49:131-141.
105. Berg P.A., Klein R., Rocken M. Cytokines in primary biliary cirrhosis. Semin Liver Dis 1997, 17:115-123.
106. Aoki C.A., Dawson K., Kenny T.P., et al. Gene expression by PBMC in primary sclerosing cholangitis: evidence for dysregulation of immune mediated genes. Clin Dev Immunol 2006, 13:265-271.
107. Karrar A., Broome U., Södergren T., et al. Biliary epithelial cell antibodies link adaptive and innate immune responses in primary sclerosing cholangitis. Gastroenterology 2007, 4:1504-1514.
108. Farkkila M., Karvonen A.L., Nurmi H., et al. Metronidazole and ursodeoxycholic acid for primary sclerosing cholangitis: a randomized placebo-controlled trial. Hepatology 2004, 40:1379-1386.
109. Blechacz B., Gores G.J. Cholangiocarcinoma: advances in pathogenesis, diagnosis, and treatment. Hepatology 2008, 48:308-321.
110. Komichi D., Tazuma S., Nishioka T., et al. Glycochenodeoxycholate plays a carcinogenic role in immortalized mouse cholangiocytes via oxidative DNA damage. Free Radic Biol Med 2005, 39:1418-1427.
111. Forsbring M., Vik E.S., Dalhus B., et al. Catalytically impaired hMYH and NEIL1 mutant proteins identified in patients with primary sclerosing cholangitis and cholangiocarcinoma. Carcinogenesis 2009, 30:1147-1154.
112. Kobayashi S., Werneburg N.W., Bronk S.F., et al. Interleukin-6 contributes to Mcl-1 up-regulation and TRAIL resistance via an Akt-signaling pathway in cholangiocarcinoma cells. Gastroenterology 2005, 128:2054-2065.
113. Bromberg J., Wang T.C. Inflammation and cancer: IL-6 and STAT3 complete the link. Cancer Cell 2009, 15:79-80.
114. Isomoto H., Mott J.L., Kobayashi S., et al. Sustained IL-6/STAT-3 signaling in cholangiocarcinoma cells due to SOCS-3 epigenetic silencing. Gastroenterology 2007, 132:384-396.
115. Isomoto H. Epigenetic alterations associated with cholangiocarcinoma (review). Oncol Rep 2009, 22:227-232.
116. Condeelis J., Pollard J.W. Macrophages: obligate partners for tumor cell migration, invasion, and metastasis. Cell 2006, 124:263-266.
117. Guerra N., Tan Y.X., Joncker N.T., et al. NKG2D-deficient mice are defective in tumor surveillance in models of spontaneous malignancy. Immunity 2008, 28:571-580.
118. Melum E., Karlsen T.H., Schrumpf E., et al. Cholangiocarcinoma in primary sclerosing cholangitis is associated with NKG2D polymorphisms. Hepatology 2008, 47:90-96.
119. Hayashi T., Imai K., Morishita Y., et al. Identification of the NKG2D haplotypes associated with natural cytotoxic activity of peripheral blood lymphocytes and cancer immunosurveillance. Cancer Res 2006, 66:563-570.
120. Boberg K.M., Bergquist A., Mitchell S., et al. Cholangiocarcinoma in primary sclerosing cholangitis: risk factors and clinical presentation. Scand J Gastroenterol 2002, 37:1205-1211.
121. Ahrendt S.A., Pitt H.A., Nakeeb A., et al. Diagnosis and management of cholangiocarcinoma in primary sclerosing cholangitis. J Gastrointest Surg 1999, 3:357-367. discussion 67-68.
122. MacCarty R.L., LaRusso N.F., Wiesner R.H., et al. Primary sclerosing cholangitis: findings on cholangiography and pancreatography. Radiology 1983, 149:39-44.
123. EASL clinical practice guidelines: management of cholestatic liver diseases. J Hepatol 2009, 51:237-267.
124. Chapman R.W., Fevery J., Kalloo A.N., et al. AASLD guidelines: diagnosis and management of primary sclerosing cholangitis (PSC). Hepatology 2010, 51:660-678.
125. Angulo P., Pearce D.H., Johnson C.D., et al. Magnetic resonance cholangiography in patients with biliary disease: its role in primary sclerosing cholangitis. J Hepatol 2000, 33:520-527.
126. Moff S.L., Kamel I.R., Eustace J., et al. Diagnosis of primary sclerosing cholangitis: a blinded comparative study using magnetic resonance cholangiography and endoscopic retrograde cholangiography. Gastrointest Endosc 2006, 64:219-223.
127. Vitellas K.M., El-Dieb A., Vaswani K.K., et al. MR cholangiopancreatography in patients with primary sclerosing cholangitis: interobserver variability and comparison with endoscopic retrograde cholangiopancreatography. AJR Am J Roentgenol 2002, 179:399-407.
128. Webster G.J., Pereira S.P., Chapman R.W. Autoimmune pancreatitis/IgG4-associated cholangitis and primary sclerosing cholangitis-overlapping or separate diseases?. J Hepatol 2009, 51:398-402.
129. Bjornsson E. Immunoglobulin G4-associated cholangitis. Curr Opin Gastroenterol 2008, 24:389-394.
130. Mendes F.D., Jorgensen R., Keach J., et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 2006, 101:2070-2075.
131. Bjornsson E., Olsson R., Bergquist A., et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology 2008, 134:975-980.
132. Angulo P., Maor-Kendler Y., Lindor K.D. Small-duct primary sclerosing cholangitis: a long-term follow-up study. Hepatology 2002, 35:1494-1500.
133. Boberg K.M., Schrumpf E., Fausa O., et al. Hepatobiliary disease in ulcerative colitis. An analysis of 18 patients with hepatobiliary lesions classified as small-duct primary sclerosing cholangitis. Scand J Gastroenterol 1994, 29:744-752.
134. Broome U., Glaumann H., Lindstom E., et al. Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC). J Hepatol 2002, 36:586-589.
135. Rabinovitz M., Demetris A.J., Bou-Abboud C.F., et al. Simultaneous occurrence of primary sclerosing cholangitis and autoimmune chronic active hepatitis in a patient with ulcerative colitis. Dig Dis Sci 1992, 37:1606-1611.
136. Lawrence S.P., Sherman K.E., Lawson J.M., et al. A 39 year old man with chronic hepatitis. Semin Liver Dis 1994, 14:97-105.
137. Perdigoto R., Carpenter H.A., Czaja A.J. Frequency and significance of chronic ulcerative colitis in severe corticosteroid-treated autoimmune hepatitis. J Hepatol 1992, 14:325-331.
138. Floreani A., Rizzotto E.R., Ferrara F., et al. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. Am J Gastroenterol 2005, 100:1516-1522.
139. Debray D., Pariente D., Urvoas E., et al. Sclerosing cholangitis in children. J Pediatr 1994, 124:49-56.
140. Wilschanski M., Chait P., Wade J.A., et al. Primary sclerosing cholangitis in 32 children: clinical, laboratory, and radiographic features, with survival analysis. Hepatology 1995, 22:1415-1422.
141. Feldstein A.E., Perrault J., El-Youssif M., et al. Primary sclerosing cholangitis in children: a long-term follow-up study. Hepatology 2003, 38:210-217.
142. Batres L.A., Russo P., Mathews M., et al. Primary sclerosing cholangitis in children: a histologic follow-up study. Pediatr Dev Pathol 2005, 8:568-576.
143. Miloh T., Arnon R., Shneider B., et al. A retrospective single-center review of primary sclerosing cholangitis in children. Clin Gastroenterol Hepatol 2009, 7:239-245.
144. Gregorio G.V., Portmann B., Karani J., et al. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology 2001, 33:544-553.
145. McNair A.N., Moloney M., Portmann B.C., et al. Autoimmune hepatitis overlapping with primary sclerosing cholangitis in five cases. Am J Gastroenterol 1998, 93:777-784.
146. Boberg K.M., Egeland T., Schrumpf E. Long-term effect of corticosteroid treatment in primary sclerosing cholangitis patients. Scand J Gastroenterol 2003, 38:991-995.
147. Rosen C.B., Nagorney D.M., Wiesner R.H., et al. Cholangiocarcinoma complicating primary sclerosing cholangitis. Ann Surg 1991, 213:21-25.
148. Bergquist A., Glaumann H., Persson B., et al. Risk factors and clinical presentation of hepatobiliary carcinoma in patients with primary sclerosing cholangitis: a case-control study. Hepatology 1998, 27:311-316.
149. Chalasani N., Baluyut A., Ismail A., et al. Cholangiocarcinoma in patients with primary sclerosing cholangitis: a multicenter case-control study. Hepatology 2000, 31:7-11.
150. Blechacz B.R., Sanchez W., Gores G.J. A conceptual proposal for staging ductal cholangiocarcinoma. Curr Opin Gastroenterol 2009, 25:238-239.
151. Khan S.A., Davidson B.R., Goldin R., et al. Guidelines for the diagnosis and treatment of cholangiocarcinoma: consensus document. Gut 2002, 51:VI1-VI9.
152. Malhi H., Gores G.J. Review article: the modern diagnosis and therapy of cholangiocarcinoma. Aliment Pharmacol Ther 2006, 23:1287-1296.
153. Campbell W.L., Ferris J.V., Holbert B.L., et al. Biliary tract carcinoma complicating primary sclerosing cholangitis: evaluation with CT, cholangiography, US, and MR imaging. Radiology 1998, 207:41-50.
154. Teefey S.A., Baron R.L., Rohrmann C.A., et al. Sclerosing cholangitis: CT findings. Radiology 1988, 169:635-639.
155. Zhang Y., Uchida M., Abe T., et al. Intrahepatic peripheral cholangiocarcinoma: comparison of dynamic CT and dynamic MRI. J Comput Assist Tomogr 1999, 23:670-677.
156. Lee M.G., Park K.B., Shin Y.M., et al. Preoperative evaluation of hilar cholangiocarcinoma with contrast-enhanced three-dimensional fast imaging with steady-state precession magnetic resonance angiography: comparison with intraarterial digital subtraction angiography. World J Surg 2003, 27:278-283.
157. Kluge R., Schmidt F., Caca K., et al. Positron emission tomography with [(18)F]fluoro-2-deoxy-d-glucose for diagnosis and staging of bile duct cancer. Hepatology 2001, 33:1029-1035.
158. Prytz H., Keiding S., Bjornsson E., et al. Dynamic FDG-PET is useful for detection of cholangiocarcinoma in patients with PSC listed for liver transplantation. Hepatology 2006, 44:1572-1580.
159. Anderson C.D., Rice M.H., Pinson C.W., et al. Fluorodeoxyglucose PET imaging in the evaluation of gallbladder carcinoma and cholangiocarcinoma. J Gastrointest Surg 2004, 8:90-97.
160. Fevery J., Buchel O., Nevens F., et al. Positron emission tomography is not a reliable method for the early diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis. J Hepatol 2005, 43:358-360.
161. Tischendorf J.J., Kruger M., Trautwein C., et al. Cholangioscopic characterization of dominant bile duct stenoses in patients with primary sclerosing cholangitis. Endoscopy 2006, 38:665-669.
162. Tischendorf J.J., Meier P.N., Schneider A., et al. Transpapillary intraductal ultrasound in the evaluation of dominant bile duct stenoses in patients with primary sclerosing cholangitis. Scand J Gastroenterol 2007, 42:1011-1017.
163. Charatcharoenwitthaya P., Enders F.B., Halling K.C., et al. Utility of serum tumor markers, imaging, and biliary cytology for detecting cholangiocarcinoma in primary sclerosing cholangitis. Hepatology 2008, 48:1106-1117.
164. Moreno Luna L.E., Kipp B., Halling K.C., et al. Advanced cytologic techniques for the detection of malignant pancreatobiliary strictures. Gastroenterology 2006, 131:1064-1072.
165. Kipp B.R., Stadheim L.M., Halling S.A., et al. A comparison of routine cytology and fluorescence in situ hybridization for the detection of malignant bile duct strictures. Am J Gastroenterol 2004, 99:1675-1681.
166. Baron T.H., Harewood G.C., Rumalla A., et al. A prospective comparison of digital image analysis and routine cytology for the identification of malignancy in biliary tract strictures. Clin Gastroenterol Hepatol 2004, 2:214-219.
167. Bonney G.K., Craven R.A., Prasad R., et al. Circulating markers of biliary malignancy: opportunities in proteomics?. Lancet Oncol 2008, 9:149-158.
168. Mullighan C.G., Goorha S., Radtke I., et al. Genome-wide analysis of genetic alterations in acute lymphoblastic leukaemia. Nature 2007, 446:758-764.
169. Miller G., Socci N.D., Dhall D., et al. Genome wide analysis and clinical correlation of chromosomal and transcriptional mutations in cancers of the biliary tract. J Exp Clin Cancer Res 2009, 28:62.
170. Spratlin J.L., Serkova N.J., Eckhardt S.G. Clinical applications of metabolomics in oncology: a review. Clin Cancer Res 2009, 15:431-440.
171. Alvaro D. Serum and bile biomarkers for cholangiocarcinoma. Curr Opin Gastroenterol 2009, 25:279-284.
172. Alvaro D., Macarri G., Mancino M.G., et al. Serum and biliary insulin-like growth factor I and vascular endothelial growth factor in determining the cause of obstructive cholestasis. Ann Intern Med 2007, 147:451-459.
173. Stiehl A., Walker S., Stiehl L., et al. Effect of ursodeoxycholic acid on liver and bile duct disease in primary sclerosing cholangitis. A 3-year pilot study with a placebo-controlled study period. J Hepatol 1994, 20:57-64.
174. Mitchell S.A., Bansi D.S., Hunt N., et al. A preliminary trial of high-dose ursodeoxycholic acid in primary sclerosing cholangitis. Gastroenterology 2001, 121:900-907.
175. Beuers U., Spengler U., Kruis W., et al. Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial. Hepatology 1992, 16:707-714.
176. Lindor K.D. Ursodiol for primary sclerosing cholangitis. Mayo Primary Sclerosing Cholangitis-Ursodeoxycholic Acid Study Group. N Engl J Med 1997, 336:691-695.
177. Olsson R., Boberg K.M., de Muckadell O.S., et al. High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5-year multicenter, randomized, controlled study. Gastroenterology 2005, 129:1464-1472.
178. Lindor K.D., Kowdley K.V., Luketic V.A., et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology 2009, 50:808-814.
179. Shi J., Li Z., Zeng X., et al. Ursodeoxycholic acid in primary sclerosing cholangitis: meta-analysis of randomized controlled trials. Hepatol Res 2009, 39:865-873.
180. Cullen S.N., Rust C., Fleming K., et al. High dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis is safe and effective. J Hepatol 2008, 48:792-800.
181. Harnois D.M., Angulo P., Jorgensen R.A., et al. High-dose ursodeoxycholic acid as a therapy for patients with primary sclerosing cholangitis. Am J Gastroenterol 2001, 96:1558-1562.
182. Rudolph G., Kloeters-Plachky P., Rost D., et al. The incidence of cholangiocarcinoma in primary sclerosing cholangitis after long-time treatment with ursodeoxycholic acid. Eur J Gastroenterol Hepatol 2007, 19:487-491.
183. Brandsaeter B., Isoniemi H., Broome U., et al. Liver transplantation for primary sclerosing cholangitis; predictors and consequences of hepatobiliary malignancy. J Hepatol 2004, 40:815-822.
184. Tung B.Y., Emond M.J., Haggitt R.C., et al. Ursodiol use is associated with lower prevalence of colonic neoplasia in patients with ulcerative colitis and primary sclerosing cholangitis. Ann Intern Med 2001, 134:89-95.
185. Pardi D.S., Loftus E.V., Kremers W.K., et al. Ursodeoxycholic acid as a chemopreventive agent in patients with ulcerative colitis and primary sclerosing cholangitis. Gastroenterology 2003, 124:889-893.
186. Sjoqvist U., Tribukait B., Ost A., et al. Ursodeoxycholic acid treatment in IBD-patients with colorectal dysplasia and/or DNA-aneuploidy: a prospective, double-blind, randomized controlled pilot study. Anticancer Res 2004, 24:3121-3127.
187. Cullen S.N., Chapman R.W. The medical management of primary sclerosing cholangitis. Semin Liver Dis 2006, 26:52-61.
188. Luth S, Kanzler S, Frenzel C, et al. Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. J Clin Gastroenterol; in press.
189. Ghazale A., Chari S.T., Zhang L., et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008, 134:706-715.
190. Church N.I., Pereira S.P., Deheragoda M.G., et al. Autoimmune pancreatitis: clinical and radiological features and objective response to steroid therapy in a UK series. Am J Gastroenterol 2007, 102:2417-2425.
191. Hirano K., Tada M., Isayama H., et al. Long-term prognosis of autoimmune pancreatitis with and without corticosteroid treatment. Gut 2007, 56:1719-1724.
192. Stiehl A., Rudolph G., Kloters-Plachky P., et al. Development of dominant bile duct stenoses in patients with primary sclerosing cholangitis treated with ursodeoxycholic acid: outcome after endoscopic treatment. J Hepatol 2002, 36:151-156.
193. Baluyut A.R., Sherman S., Lehman G.A., et al. Impact of endoscopic therapy on the survival of patients with primary sclerosing cholangitis. Gastrointest Endosc 2001, 53:308-312.
194. Gluck M., Cantone N.R., Brandabur J.J., et al. A twenty-year experience with endoscopic therapy for symptomatic primary sclerosing cholangitis. J Clin Gastroenterol 2008, 42:1032-1039.
195. Kaya M., Petersen B.T., Angulo P., et al. Balloon dilation compared to stenting of dominant strictures in primary sclerosing cholangitis. Am J Gastroenterol 2001, 96:1059-1066.
196. Stiehl A. Primary sclerosing cholangitis: the role of endoscopic therapy. Semin Liver Dis 2006, 26:62-68.
197. Ponsioen C.Y., Lam K., van Milligen de Wit A.W., et al. Four years experience with short term stenting in primary sclerosing cholangitis. Am J Gastroenterol 1999, 94:2403-2407.
198. van Milligen de Wit A.W., Rauws E.A., van Bracht J., et al. Lack of complications following short-term stent therapy for extrahepatic bile duct strictures in primary sclerosing cholangitis. Gastrointest Endosc 1997, 46:344-347.
199. Bjoro K., Brandsaeter B., Foss A., et al. Liver transplantation in primary sclerosing cholangitis. Semin Liver Dis 2006, 26:69-79.
200. Bjoro K., Schrumpf E. Liver transplantation for primary sclerosing cholangitis. J Hepatol 2004, 40:570-577.
201. Uemura T., Ikegami T., Sanchez E.Q., et al. Late acute rejection after liver transplantation impacts patient survival. Clin Transplant 2008, 22:316-323.
202. Graziadei I.W., Wiesner R.H., Marotta P.J., et al. Long-term results of patients undergoing liver transplantation for primary sclerosing cholangitis. Hepatology 1999, 30:1121-1127.
203. Neuberger J. Incidence, timing, and risk factors for acute and chronic rejection. Liver Transpl Surg 1999, 5:S30-S36.
204. Narumi S., Roberts J.P., Emond J.C., et al. Liver transplantation for sclerosing cholangitis. Hepatology 1995, 22:451-457.
205. Shaked A., Colonna J.O., Goldstein L., et al. The interrelation between sclerosing cholangitis and ulcerative colitis in patients undergoing liver transplantation. Ann Surg 1992, 215:598-603.
206. Miki C., Harrison J.D., Gunson B.K., et al. Inflammatory bowel disease in primary sclerosing cholangitis: an analysis of patients undergoing liver transplantation. Br J Surg 1995, 82:1114-1117.
207. Kugelmas M., Spiegelman P., Osgood M.J., et al. Different immunosuppressive regimens and recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl 2003, 9:727-732.
208. Hoffman C.E.E. Verschluss der Gallenwege durch Verdickung der Wandungen. Arch Pathol Anat Physiol 1867, 49:206-215.
209. Smith M.P., Loe R.H. Sclerosing cholangitis; review of recent case reports and associated diseases and four new cases. Am J Surg 1965, 110:239-246.
210. Warren K.W., Athanassiades S. Monge JI Primary sclerosing cholangitis. A study of forty-two cases. Am J Surg 1966, 111:23-38.
211. McFarlane I.G., Wojcicka B.M., Tsantoulas D.C., et al. Leukocyte migration inhibition in response to biliary antigens in primary biliary cirrhosis, sclerosing cholangitis, and other chronic liver diseases. Gastroenterology 1979, 76:1333-1340.
212. Chapman R.W., Arborgh B.A., Rhodes J.M., et al. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut 1980, 21:870-877.
213. Schrumpf E., Elgjo K., Fausa O., et al. Sclerosing cholangitis in ulcerative colitis. Scand J Gastroenterol 1980, 15:689-697.
214. NIH National Institutes of Health Consensus Development Conference Statement: liver transplantation-June 20-23, 1983. Hepatology 1984, 4:107S-1010S.
215. Wee A., Ludwig J., Coffey R.J., et al. Hepatobiliary carcinoma associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hum Pathol 1985, 16:719-726.
216. Wee A., Ludwig J. Pericholangitis in chronic ulcerative colitis: primary sclerosing cholangitis of the small bile ducts?. Ann Intern Med 1985, 102:581-587.
217. Lerut J., Demetris A.J., Stieber A.C., et al. Intrahepatic bile duct strictures after human orthotopic liver transplantation. Recurrence of primary sclerosing cholangitis or unusual presentation of allograft rejection?. Transpl Int 1988, 1:127-130.
218. Broome U., Lindberg G., Lofberg R. Primary sclerosing cholangitis in ulcerative colitis-a risk factor for the development of dysplasia and DNA aneuploidy?. Gastroenterology 1992, 102:1877-1880.
219. Bergquist A., Lindberg G., Saarinen S., et al. Increased prevalence of primary sclerosing cholangitis among first-degree relatives. J Hepatol 2005, 42:252-256.
220. Hov J.R., Boberg K.M., Karlsen T.H. Autoantibodies in primary sclerosing cholangitis. World J Gastroenterol 2008, 14:3781-3791.
221. Ueno Y., Phillips J.O., Ludwig J., et al. Development and characterization of a rodent model of immune-mediated cholangitis. Proc Natl Acad Sci USA 1996, 93:216-220.
222. Boberg K.M., Fausa O., Haaland T., et al. Features of autoimmune hepatitis in primary sclerosing cholangitis: an evaluation of 114 primary sclerosing cholangitis patients according to a scoring system for the diagnosis of autoimmune hepatitis. Hepatology 1996, 23:1369-1376.
223. Colombo C. Liver disease in cystic fibrosis. Curr Opin Pulm Med 2007, 13:529-536.