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1) Karlsen TH, Schrumpf E, Boberg KM. Update on primary sclerosing cholangitis. Dig Liver Dis. 2010; 42: 390-400
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2) Mendes F, Lindor KD. Primary sclerosing cholangitis: overview and update. Nat Rev Gastroenterol Hepatol. 2010; 7: 611-9
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3) Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010; 51: 660-78
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4) Card TR, Solaymani-Dodaran M, West J. Incidence and mortality of primary sclerosing cholangitis in the UK: a population-based cohort study. J Hepatol. 2008; 48: 939-44
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5) Lindkvist B, Benito de Valle M, Gullberg B, et al. Incidence and prevalence of primary sclerosing cholangitis in a defined adult population in Sweden. Hepatology. 2010; 52: 571-7
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6) 滝川 一. PSCの全国疫学調査. 厚生労働科学研究補助金難治性疾患克服研究事業報告書. 2008
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7) Hirano K, Tada M, Isayama H, et al. Clinical features of primary sclerosing cholangitis with onset age above 50 years. J Gastroenterol. 2008; 43: 729-33
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8) Ye BD, Yang SK, Boo SJ, et al. Clinical characteristics of ulcerative colitis associated with primary sclerosing cholangitis in Korea. Inflamm Bowel Dis. 2011; 17: 1901-6
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9) 西野隆義, 白戸 泉, 白戸美穂, 他. 炎症性腸疾患の合併からみた原発性硬化性胆管炎の病態. 胆と膵. 2010; 31: 737-41
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10) Singal AK, Stanca CM, Clark V, et al. Natural history of small duct primary sclerosing cholangitis: a case series with review of the literature. Hepatol Int. 2011; 5: 808-13
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11) Bjornsson E, Olsson R, Bergquist A, et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology. 2008; 134: 975-80
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12) Bergquist A, Montgomery SM, 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: 939-43
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13) Karlsen TH, Franke A, Melum E, et al. Genome-wide association analysis in primary sclerosing cholangitis. Gastroenterology. 2010; 138: 1102-11
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14) 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: 17-9
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15) Franke A, Balschun T, Karlsen TH, et al. Sequence variants in IL10, ARPC2 and multiple other loci contribute to ulcerative colitis susceptibility. Nat Genet. 2008; 40: 1319-23
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16) 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
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17) 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
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18) Juran BD, Atkinson EJ, Schlicht EM, et al. Genetic polymorphisms of matrix metalloproteinase 3 in primary sclerosing cholangitis. Liver Int. 2011; 31: 787-93
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19) Aron JH, Bowlus CL. The immunobiology of primary sclerosing cholangitis. Semin Immunopathol. 2009; 31: 383-97
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20) Dave M, Elmunzer BJ, Dwamena BA, et al. Primary sclerosing cholangitis: meta-analysis of diagnostic performance of MR cholangiopancreatography. Radiology. 2010; 256: 387-96
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21) Chavhan GB, Roberts E, Moineddin R, et al. Primary sclerosing cholangitis in children: utility of magnetic resonance cholangiopancreatography. Pediatr Radiol. 2008; 38: 868-73
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22) Gotthardt D, Stiehl A. Endoscopic retrograde cholangiopancreatography in diagnosis and treatment of primary sclerosing cholangitis. Clin Liver Dis. 2010; 14: 349-58
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23) Nakazawa T, Ohara H, Sano H, et al. Cholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis. Gastrointest Endosc. 2004; 60: 937-44
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24) Kamisawa T, Takuma K, Anjiki H, et al. Sclerosing cholangitis associated with autoimmune pancreatitis differs from primary sclerosing cholangitis. World J Gastroenterol. 2009; 15: 2357-60
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25) Kalaitzakis E, Levy M, Kamisawa T, et al. Endoscopic retrograde cholangiography does not reliably distinguish IgG4-associated cholangitis from primary sclerosing cholangitis or cholangiocarcinoma. Clin Gastroenterol Hepatol. 2011; 9: 800-3
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26) Alderlieste YA, van den Elzen BD, Rauws EA, et al. Immunoglobulin G4-associated cholangitis: one variant of immunoglobulin G4-related systemic disease. Digestion. 2009; 79: 220-8
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27) Mendes F, Jorgensen R, Keach J, et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol. 2006; 101: 2070-5
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28) Webster GJ, Pereira SP, Chapman RW. Autoimmune pancreatitis/IgG4-associated cholangitis and primary sclerosing cholangitis—overlapping or separate diseases? J Hepatol. 2009; 51: 398-402
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29) Zen Y, Quaglia A, Portmann B. Immunoglobulin G4-positive plasma cell infiltration in explanted livers for primary sclerosing cholangitis. Histopathology. 2011; 58: 414-22
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30) Zhang L, Lewis JT, Abraham SC, et al. IgG4+ plasma cell infiltrates in liver explants with primary sclerosing cholangitis. Am J Surg Pathol. 2010; 34: 88-94
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31) Ghazale A, Chari ST, Zhang L, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008; 134: 706-15
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32) Fevery J, Verslype C. An update on cholangiocarcinoma associated with primary sclerosing cholangitis. Curr Opin Gastroenterol. 2010; 26: 236-45
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33) Sinakos E, Saenger AK, Keach J, et al. Many patients with primary sclerosing cholangitis and increased serum levels of carbohydrate antigen 19-9 do not have cholangiocarcinoma. Clin Gastroenterol Hepatol. 2011; 9: 434-9 e1
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34) Lankisch TO, Metzger J, Negm AA, et al. Bile proteomic profiles differentiate cholangiocarcinoma from primary sclerosing cholangitis and choledocholithiasis. Hepatology. 2011; 53: 875-84
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35) Albiin N, Smith IC, Arnelo U, et al. Detection of cholangiocarcinoma with magnetic resonance spectroscopy of bile in patients with and without primary sclerosing cholangitis. Acta Radiol. 2008; 49: 855-62
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36) Torres J, de Chambrun GP, Itzkowitz S, et al. Review article: colorectal neoplasia in patients with primary sclerosing cholangitis and inflammatory bowel disease. Aliment Pharmacol Ther. 2011; 34: 497-508
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37) Soetikno RM, Lin OS, Heidenreich PA, 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
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38) Claessen MM, Vleggaar FP, Tytgat KM, et al. High lifetime risk of cancer in primary sclerosing cholangitis. J Hepatol. 2009; 50: 158-64
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39) 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
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40) Poropat G, Giljaca V, Stimac D, et al. Bile acids for primary sclerosing cholangitis. Cochrane Database Syst Rev. 2011: CD003626
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41) Lindor KD, Kowdley KV, Luketic VA, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology. 2009; 50: 808-14
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42) Sinakos E, Marschall HU, Kowdley KV, et al. Bile acid changes after high-dose ursodeoxycholic acid treatment in primary sclerosing cholangitis: Relation to disease progression. Hepatology. 2010; 52: 197-203
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43) Mizuno S, Hirano K, Tada M, et al. Bezafibrate for the treatment of primary sclerosing cholangitis. J Gastroenterol. 2010; 45: 758-62
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44) Gotthardt DN, Rudolph G, Kloters-Plachky P, et al. Endoscopic dilation of dominant stenoses in primary sclerosing cholangitis: outcome after long-term treatment. Gastrointest Endosc. 2010; 71: 527-34
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45) Tanaka A, Takamori Y, Toda G, et al. Outcome and prognostic factors of 391 Japanese patients with primary sclerosing cholangitis. Liver Int. 2008; 28: 983-9
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46) Egawa H, Ueda Y, Ichida T, et al. Risk factors for recurrence of primary sclerosing cholangitis after living donor liver transplantation in Japanese registry. Am J Transplant. 2011; 11: 518-27
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47) Graziadei IW. Live donor liver transplantation for primary sclerosing cholangitis: is disease recurrence increased? Curr Opin Gastroenterol. 2011; 27: 301-5
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