Clinical features and management of primary sclerosing cholangitis

World Journal of Gastroenterology

Volumn 14, Issue 21, 2008, Pages 3338-3349

  Silveira, Marina G ^a   Lindor, Keith D ^a  

^a MAYO CLINIC   (United States)

Author keywords

Cholangiocarcinoma; Cholestasis; Diagnosis; Liver transplantation; Sclerosing cholangitis; Therapy

Indexed keywords

ALKALINE PHOSPHATASE; ANTIBIOTIC AGENT; ANTIPERINUCLEAR FACTOR; AZATHIOPRINE; BUDESONIDE; CA 19-9 ANTIGEN; CAPECITABINE; COLESTYRAMINE; FLUOROURACIL; METHOTREXATE; NEUTROPHIL CYTOPLASMIC ANTIBODY; PLACEBO; PREDNISOLONE; PREDNISONE; TACROLIMUS; URSODEOXYCHOLIC ACID; VITAMIN D;

ABDOMINAL PAIN; ABSENCE OF SIDE EFFECTS; ALANINE AMINOTRANSFERASE BLOOD LEVEL; ALKALINE PHOSPHATASE BLOOD LEVEL; ANTIBODY DETECTION; ARTICLE; ASPARTATE AMINOTRANSFERASE BLOOD LEVEL; AUTOIMMUNE HEPATITIS; BACTERIAL CHOLANGITIS; BACTERIAL INFECTION; BILE DUCT CARCINOMA; BILE DUCT DILATATION; BILE DUCT OBSTRUCTION; CANCER SURGERY; CHOLANGIOGRAPHY; CHOLEDOCHOJEJUNOSTOMY; CHOLELITHIASIS; CLINICAL FEATURE; CLINICAL TRIAL; CONTINUOUS INFUSION; CROHN DISEASE; DIARRHEA; DIET SUPPLEMENTATION; DISEASE ASSOCIATION; DRUG MEGADOSE; ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY; ENTERITIS; FATIGUE; FEVER; HISTOPATHOLOGY; HUMAN; IMMUNOGLOBULIN G4 RELATED SCLEROSING CHOLANGITIS; JAUNDICE; LIVER CELL CARCINOMA; LIVER TRANSPLANTATION; LOW DRUG DOSE; MAGNETIC RESONANCE CHOLANGIOGRAPHY; OSTEOPENIA; OSTEOPOROSIS; PERISTOMAL VARICES; PREGNANCY; PRIMARY SCLEROSING CHOLANGITIS; PRURITUS; SCLEROSING CHOLANGITIS; SMALL DUCT PRIMARY CHOLANGITIS; ULCERATIVE COLITIS; VARICOSIS;

EID: 54349092389     PISSN: 10079327     EISSN: None     Source Type: Journal    
DOI: 10.3748/wjg.14.3338     Document Type: Article

References (164)

1. Lee YM, Kaplan MM. Primary sclerosing cholangitis. N Engl J Med 1995; 332: 924-933
2. Talwalkar JA, Lindor KD. Primary sclerosing cholangitis. Inflamm Bowel Dis 2005; 11: 62-72
3. LaRusso NF, Shneider BL, Black D, Gores GJ, James SP, Doo E, Hoofnagle JH. Primary sclerosing cholangitis: summary of a workshop. Hepatology 2006; 44: 746-764
4. Gautam M, Cheruvattath R, Balan V. Recurrence of autoimmune liver disease after liver transplantation: a systematic review. Liver Transpl 2006; 12: 1813-1824
5. Farrant JM, Hayllar KM, Wilkinson ML, Karani J, Portmann BC, Westaby D, Williams R. Natural history and prognostic variables in primary sclerosing cholangitis. Gastroenterology 1991; 100: 1710-1717
6. Chapman RW, Arborgh BA, Rhodes JM, Summerfield JA, Dick R, Scheuer PJ, Sherlock S. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut 1980; 21: 870-877
7. Wiesner RH, Grambsch PM, Dickson ER, Ludwig J, MacCarty RL, Hunter EB, Fleming TR, Fisher LD, Beaver SJ, LaRusso NF. Primary sclerosing cholangitis: natural history, prognostic factors and survival analysis. Hepatology 1989; 10: 430-436
8. Wiesner RH, LaRusso NF. Clinicopathologic features of the syndrome of primary sclerosing cholangitis. Gastroenterology 1980; 79: 200-206
9. Broome U, Olsson R, Loof L, Bodemar G, Hultcrantz R, Danielsson A, Prytz H, Sandberg-Gertzen H, Wallerstedt S, Lindberg G. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis. Gut 1996; 38: 610-615
10. Okolicsanyi L, Fabris L, Viaggi S, Carulli N, Podda M, Ricci G. 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
11. Broome U, Bergquist A. Primary sclerosing cholangitis, inflammatory bowel disease, and colon cancer. Semin Liver Dis 2006; 26: 31-41
12. Rasmussen HH, Fallingborg JF, Mortensen PB, Vyberg M, Tage-Jensen U, Rasmussen SN. Hepatobiliary dysfunction and primary sclerosing cholangitis in patients with Crohn's disease. Scand J Gastroenterol 1997; 32: 604-610
13. Fausa O, Schrumpf E, Elgjo K. Relationship of inflammatory bowel disease and primary sclerosing cholangitis. Semin Liver Dis 1991; 11: 31-39
14. Rabinovitz M, Gavaler JS, Schade RR, Dindzans VJ, Chien MC, Van Thiel DH. Does primary sclerosing cholangitis occurring in association with inflammatory bowel disease differ from that occurring in the absence of inflammatory bowel disease? A study of sixty-six subjects. Hepatology 1990; 11: 7-11
15. Loftus EV Jr, Harewood GC, Loftus CG, Tremaine WJ, Harmsen WS, Zinsmeister AR, Jewell DA, Sandborn WJ. PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis. Gut 2005; 54: 91-96
16. Saarinen S, Olerup O, Broome U. Increased frequency of autoimmune diseases in patients with primary sclerosing cholangitis. Am J Gastroenterol 2000; 95: 3195-3199
17. Kaplan GG, Laupland KB, Butzner D, Urbanski SJ, Lee SS. The burden of large and small duct primary sclerosing cholangitis in adults and children: a population-based analysis. Am J Gastroenterol 2007; 102: 1042-1049
18. Bergquist A, Said K, Broome U. Changes over a 20-year period in the clinical presentation of primary sclerosing cholangitis in Sweden. Scand J Gastroenterol 2007; 42: 88-93
19. Charatcharoenwitthaya P, Lindor KD. Primary sclerosing cholangitis: diagnosis and management. Curr Gastroenterol Rep 2006; 8: 75-82
20. Bjornsson E, Angulo P. Cholangiocarcinoma in young individuals with and without primary sclerosing cholangitis. Am J Gastroenterol 2007; 102: 1677-1682
21. Younossi ZM, Boparai N, Price LL, Kiwi ML, McCormick M, Guyatt G. Health-related quality of life in chronic liver disease: the impact of type and severity of disease. Am J Gastroenterol 2001; 96: 2199-2205
22. Kim WR, Lindor KD, Malinchoc M, Petz JL, Jorgensen R, Dickson ER. Reliability and validity of the NIDDK-QA instrument in the assessment of quality of life in ambulatory patients with cholestatic liver disease. Hepatology 2000; 32: 924-929
23. Cullen SN, Chapman RW. Review article: current management of primary sclerosing cholangitis. Aliment Pharmacol Ther 2005; 21: 933-948
24. Dickson ER, Murtaugh PA, Wiesner RH, Grambsch PM, Fleming TR, Ludwig J, LaRusso NF, Malinchoc M, Chapman RW, Kaplan MM. Primary sclerosing cholangitis: refinement and validation of survival models. Gastroenterology 1992; 103: 1893-1901
25. Kim WR, Therneau TM, Wiesner RH, Poterucha JJ, Benson JT, Malinchoc M, LaRusso NF, Lindor KD, Dickson ER. A revised natural history model for primary sclerosing cholangitis. Mayo Clin Proc 2000; 75: 688-694
26. Kamath PS, Wiesner RH, Malinchoc M, Kremers W, Therneau TM, Kosberg CL, D'Amico G, Dickson ER, Kim WR. A model to predict survival in patients with end-stage liver disease. Hepatology 2001; 33: 464-470
27. Chapman RW, Cottone M, Selby WS, Shepherd HA, Sherlock S, Jewell DP. Serum autoantibodies, ulcerative colitis and primary sclerosing cholangitis. Gut 1986; 27: 86-91
28. Mulder AH, Horst G, Haagsma EB, Limburg PC, Kleibeuker JH, Kallenberg CG. Prevalence and characterization of neutrophil cytoplasmic antibodies in autoimmune liver diseases. Hepatology 1993; 17: 411-417
29. Bansi D, Chapman R, Fleming K. Antineutrophil cytoplasmic antibodies in chronic liver diseases: prevalence, titre, specificity and IgG subclass. J Hepatol 1996; 24: 581-586
30. Chapman RW. The enigma of anti-neutrophil antibodies in ulcerative colitis primary sclerosing cholangitis: important genetic marker or epiphenomenon? Hepatology 1995; 21: 1473-1474
31. Wiesner RH. Current concepts in primary sclerosing cholangitis. Mayo Clin Proc 1994; 69: 969-982
32. Bilbao MK, Dotter CT, Lee TG, Katon RM. Complications of endoscopic retrograde cholangiopancreatography (ERCP). A study of 10,000 cases. Gastroenterology 1976; 70: 314-320
33. Freeman ML, Nelson DB, Sherman S, Haber GB, Herman ME, Dorsher PJ, Moore JP, Fennerty MB, Ryan ME, Shaw MJ, Lande JD, Pheley AM. Complications of endoscopic biliary sphincterotomy. N Engl J Med 1996; 335: 909-918
34. Talwalkar JA, Angulo P, Johnson CD, Petersen BT, Lindor KD. Cost-minimization analysis of MRC versus ERCP for the diagnosis of primary sclerosing cholangitis. Hepatology 2004; 40: 39-45
35. Mehta SN, Reinhold C, Barkun AN. Magnetic resonance cholangiopancreatography. Gastrointest Endosc Clin N Am 1997; 7: 247-270
36. Fulcher AS, Turner MA, Franklin KJ, Shiffman ML, Sterling RK, Luketic VA, Sanval AJ. Primary sclerosing cholangitis: evaluation with MR cholangiography-a case-contol study. Radiology 2000; 215: 71-80
37. Moff SL, Kamel IR, Eustace J, Lawler LP, Kantsevoy S, Kalloo AN, Thuluvath PT. Diagnosis of primary sclerosing cholangitis: a blinded comparative study using magnetic resonance cholangiography and endoscopic retrograde cholangiography. Gastrointest Endosc 2006; 64: 219-223
38. Petrovic BD, Nikolaidis P, Hammond NA, Martin JA, Petrovic PV, Desai PM, Miller FH. Correlation Between Findings on MRCP and Gadolinium-Enhanced MR of the Liver and a Survival Model for Primary Sclerosing Cholangitis. Dig Dis Sci 2007; 52: 3499-3506
39. Angulo P, Pearce DH, Johnson CD, Henry JJ, LaRusso NF, Petersen BT, Lindor KD. Magnetic resonance cholangiography in patients with biliary disease: its role in primary sclerosing cholangitis. J Hepatol 2000; 33: 520-527.
40. Ludwig J. Surgical pathology of the syndrome of primary sclerosing cholangitis. Am J Surg Pathol 1989; 13 Suppl 1: 43-49
41. Scheuer PJ. Ludwig Symposium on biliary disorders - part II. Pathologic features and evolution of primary biliary cirrhosis and primary sclerosing cholangitis. Mayo Clin Proc 1998; 73: 179-183
42. Burak KW, Angulo P, Lindor KD. Is there a role for liver biopsy in primary sclerosing cholangitis? Am J Gastroenterol 2003; 98: 1155-1158
43. Gossard AA, Angulo P, Lindor KD. Secondary cholangitis. cholangitis: a comparison to primary sclerosing cholangitis. Am J Gastroenterol 2005; 100: 1330-1333
44. Ludwig J, Barham SS, LaRusso NF, Elveback LR,, Wiesner RH, McCal l JT. Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hepatology 1981; 1: 632-640
45. Angulo P, Lindor KD. Primary sclerosing cholangitis. Hepatology 1999; 30: 325-332
46. Bergquist A, Glaumann H, Stal P, Wang GS, Broome U. Biliary dysplasia, cell proliferation and nuclear DNA-fragmentation in primary sclerosing cholangitis with and without cholangiocarcinoma. J Intern Med 2001; 249: 69-75
47. Fleming KA, Boberg KM, Glaumann H, Berqquist A, Smith D, Clausen OP. Biliary dysplasia as a maker of Cholangiocarcinoma in primary sclerosing cholangitis. J Hepatol 2001; 34: 360-365
48. Bjornsson E, Chapman RW. Sclerosing cholangitis. Curr Opin Gastroenterol 2003; 19: 270-275
49. Kim WR, Ludwig J, Lindor KD. Variant forms of cholestatic diseases involving small bile ducts in adults. Am J Gastroenterol 2000; 95: 1130-1138
50. Bjornsson E, Boberg KM, Cullen S, Fleming K, Clausen OP, Fausa O, Schrumpf E, Chapman RW. Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis. Gut 2002; 51: 731-735
51. Boberg KM, Schrumpf E, Fausa O, Elgjo K, Kolmannskog F, Haaland T, Holter E. 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
52. Angulo P, Maor-Kendler Y, Lindor KD. Small-duct primary sclerosing cholangitis: a long-term follow-up study. Hepatology 2002; 35: 1494-1500
53. van Buuren HR, van Hoogstraten HJE, TerKivatan T, Schalm SW, Vleggaar FP. High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis. J Hepatol 2000; 33: 543-548
54. Bjornsson E, Chari ST, Smyrk TC, Lindor K. Immunoglobulin G4 associated cholangitis: description of an emerging clinical entity based on review of the literature. Hepatology 2007; 45: 1547-1554
55. Chari ST, Smyrk TC, Levy MT, Topazian MD, Takahashi N, Zhang L, Clain JE, Pearson RK, Petersen BT, Vege SS, Farnell MB. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. CLin Gastroenterol Hepatol 2006; 4: 1010-1016: quiz 934
56. Okazaki K, Uchida K, Chiba T. Recent concent of autoimmune-related pancreatitis. J Gastroenterol 2001; 36: 293-302
57. Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A. Extrapancreatic lesions in autoimmune pancreatitis. J Clin Gastroenterol 2005; 39: 904-907
58. Zen Y, Harada K, Sasaki M, Sato Y, Tsuneyama K, Haratake J, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Morimoto H, Miwa A, Uchiyama A, Portmann BC, Nakanuma Y. IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis? Am J Surg Pathol 2004; 28: 1193-1203
59. Mendes FD, Jorgensen R, Keach J, Katzmann JA, Smyrk T, Donlinger J, Chari S, Lindor KD. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 2006; 101: 2070-2075
60. LaRusso NF, Wiesner RH, Ludwig J, MacCarty RL, Beaver SJ, Zinsmeister AR. Prospective trial of penicillamine in primary sclerosing cholangitis. Gastroenterology 1988; 95: 1036-1042
61. Knoss TA, Kaplan MM. A double-blind controlled trial of oral-pulse methotrexate therapy in the treatment of primary sclerosing cholangitis. Gastroenterology 1994: 106: 494-499
62. Angulo P, Batts KP, Jorgensen RA, LaRusso NA, Lindor KD. Oral budesonide in the treatment of primary sclerosing cholangitis. Am J Gastroenterol 2000; 95: 2333-2337
63. Olsson R, Broome U, Danielsson A, Hagerstrand J, Jarnerot G, Loof L, Prytz H, Ryden BO, Wallerstedt S. Colchicine treatment of primary sclerosing cholangitis. Gastroenterology 1995; 108: 1199-1203
64. Duchini A, Younissi ZM, Saven A, Bordin GM, Knowles HJ, Pockros PJ. An open-label pilot trial of cladibrine (2-cholordeoxyadenosine) in patients with primary sclerosing cholangitis. J Clin Gastroenterol 2000; 31: 292-296
65. Sandborn WL, Wiesner RH, Tremaine WJ, Larusso NF. Ulcerative colitis disease activity following treatment of associated primary sclerosing cholangitis with cyclosporin. Gut 1993; 34: 242-246
66. Talwalkar JA, Angulo P, Keach JC, Petz JL, Jorgensen RA, Lindor KD. Mycophenolate mofetil for the treatment of primary sclerosing cholangitis. Am J Gastroenterol 2005; 100: 308-312
67. Sterling RK, Salvatori JJ, Luketic VA, Sanyal AJ, Fulcher AS, Stravitz, RT, Contos MJ, Mills AS, Shiffman ML. A prospective, randomized-controlled pilot study of ursodeoxycholic acid combined with mycophenolate mofetil in the treatment of primary sclerosing cholangitis. Aliment Pharmacol Ther 2004; 20: 943-949
68. Epstein MP, Kaplan MM. A pilot study of etanercept in the treatment of primary sclerosing cholangitis. Dig Dis Sci 2004; 49: 1-4
69. Angulo P, Bharucha AE, Jorgensen RA, De Sotel CK, Sandborn WJ, Larusso NF, Lindor KD. Oral nicotine in treatment of primary sclerosing cholangitis: a pilot study. Dig Dis Sci 1999; 44: 602-607
70. Vleggaar FP, van Buuren HR, van Berge Henegouwen GP, Hop WC, van Erpecum KJ. No beneficial effects of transdermal nicotine in patients with primary sclerosing cholangitis: results of a randomized doucle-blind placebo-controlled cross-over study. Eur J Gastroenterol Hepatol 2001; 13: 171-175
71. Angulo P. Silymarin in the treatment of primary sclerosing cholangitis: a pilot study. Gastroenterology 2001; 120 (suppl 1): 2004; 40: 39-45
72. Angulo P, MacCarty RL, Sylvestre PB, Jorgensen RA, Wiesner RH, LaRusso NA, Lindor KD. Pirfenidone in the treatment of primary sclerosing cholangitis. Dig Dis Sci 2002; 47: 157-61
73. Bharucha AE, Jorgensen R, Lichtman SN, LaRusso NF, Lindor KD. A pilot study of pentoxifylline for the treatment of primary sclerosing cholangitis. Am J Gastroenterol 2000; 95: 2338-2342
74. Van Thiel DH, Carroll P, Abu-Elmagd K, Rodriguez-Rilo H, Irish W, McMichael J, Starzl TE. Tacrolimus (FK 506), a treatment for primary sclerosing cholangitis: results of an open-label preliminary trial. Am J Gastroenterol 1995; 90: 455-459
75. Talwalkar JA, Gossard AA, Keach JC, Jorgensen RA, Petz JL, Lindor RN. Tacrolimus for the treatment of primary sclerosing cholangitis. Liver Int 2007; 27: 451-453
76. Beuers U, Spengler U, Kruis W, Aydemir U, Wiebecke B, Heldwein W, Weinzierl M, Pape GR, Sauerbruch T, Paumgartner G. Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial. Hepatology 1992; 16: 707-714
77. Lindor KD. Ursodiol for primary sclerosing cholangitis. Mayo Primary Sclerosing Cholangitis-Ursodeoxycholic Acid Study Group. N Engl J Med 1997; 336: 691-695
78. 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: 838-847
79. Stiehl A, Walker S, Stiehl L, Rudolph G, Hofmann WJ, Theilmann L. 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
80. De Maria N, Colantoni A, Rosenbloom E, Van Thiel DH. Ursodeoxycholic acid does not improve the clinical course of primary sclerosing cholangitis over a 2-year period. Hepatogastroenterology 1996; 43: 1472-1479
81. van Hoogstraten HJ, Wolfhagen FH, van de Meeberg PC, Kuiper H, Nix GA, Becx MC, Hoek AC, van Houte DP, Rijk MC, Salemans JM, Scherpenisse J, Schrijver M, Smit AM, Spoelstra P, Stadhouders PH, Tan TG, Hop WC, ten Kate FJ, vanBerge-Henegouwen GP, Schalm SW, van Buuren HR. Ursodeoxycholic acid therapy for primary sclerosing cholangitis: results of a 2-year randomized controlled trial to evaluate single versus multiple daily doses. J Hepatol 1998; 29: 417-423
82. 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: 900-907
83. Harnois DM, Angulo P, Jorgensen RA, Larusso NF, Lindor KD. High-dose ursodeoxycholic acid as a therapy for patients with primary sclerosing cholangitis. Am J Gastroenterol 2001; 96: 1558-1562
84. Okolicsanyi L, Groppo M, Floreani A, Morselli-Labate AM, Rusticali AG, Battocchia A, Colombo M, Galatola G, Gasbarrini G, Podda M, Ricci G, Rosina F, Zuin M. Treatment of primary sclerosing cholangitis with lowdose ursodeoxycholic acid: results of a retrospective Italian multicentre survey. Dig Liver Dis 2003; 35: 325-331
85. Olsson R, Boberg KM, de Muckadell OS, Lindgren S, Hultcrantz R, Folvik G, Bell H, Gangsoy-Kristiansen M, Matre J, Rydning A, Wikman O, Danielsson A, Sandberg-Gertzen H, Ung KA, Eriksson A, Loof L, Prytz H, Marschall HU, Broome U. High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5-year multicenter, randomized, controlled study. Gastroenterology 2005; 129: 1464-1472
86. Hoofnagle JH. Primary sclerosing cholangitis. Hepatology 2005; 41: 955
87. Fong DG, Lindor KD. Future directions in the medical treatment of primary sclerosing cholangitis: the need for combination drug therapy. Am J Gastroenterol 2000; 95: 1861-1862
88. Lindor KD, Wiesner RH, Colwell LJ, Steiner B, Beaver S, LaRusso NF. The combination of prednisone and colchicine in patients with primary sclerosing cholangitis. Am J Gastroenterol 1991; 86: 57-61
89. Lindor KD, Jorgensen RA, Anderson ML, Gores GJ, Hofmann AF, LaRusso NF. Ursodeoxycholic acid and methotrexate for primary sclerosing cholangitis: a pilot study. Am J Gastroenterol 1996; 91: 511-515
90. van Hoogstraten HJ, Vleggaar FP, Boland GJ, van Steenbergen W, Griffioen P, Hop WC, van Hattum J, van Berge Henegouwen GP, Schalm SW, van Buuren HR. 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: 2015-2022
91. 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: 943-946
92. Farkkila M, Karvonen AL, Nurmi H, Nuutinen H, Taavitsainen M, Pikkarainen P, Karkkainen P. Metronidazole and ursodeoxycholic acid for primary sclerosing cholangitis: a randomized placebo-controlled trial. Hepatology 2004; 40: 1379-1386
93. Pall H, Zaman MM, Ander s son C, Freedman SD. Decreased peroxisome proliferator activated receptor alpha is associated with bile duct injury in cystic fibrosis transmembrane conductance regulator-/- mice. J Pediatr Gastroenterol Nutr 2006; 42: 275-281
94. Fickert P, Wagner M, Marschall HU, Fuchsbichler A, Zollner G, Tsybrovskyy O, Zatloukal K, Liu J, Waalkes MP, Cover C, Denk H, Hofmann AF, Jaeschke H, Trauner M. 24-norUrsodeoxycholic acid is superior to ursodeoxycholic acid in the treatment of sclerosing cholangitis in Mdr2 (Abcb4) knockout mice. Gastroenterology 2006; 130: 465-481
95. Kaya M, Petersen BT, Angulo P, Baron TH, Andrews JC, Gostout CJ, Lindor KD. Balloon dilation compared to stenting of dominant strictures in primary sclerosing cholangitis. Am J Gastroenterol 2001; 96: 1059-1066
96. Linder S, Soderlund C. Endoscopic therapy in primary sclerosing cholangitis: outcome of treatment and risk of cancer. Hepatogastroenterology 2001; 48: 387-392
97. Stiehl A, Rudolph G, Sauer P, Benz C, Stremmel W, Walker S, Theilmann L. Efficacy of ursodeoxycholic acid treatment and endoscopic dilation of major duct stenoses in primary sclerosing cholangitis. An 8-year prospective study. J Hepatol 1997; 26: 560-566
98. Baluyut AR, Sherman S, Lehman GA, Hoen H, Chalasani N. Impact of endoscopic therapy on the survival of patients with primary sclerosing cholangitis. Gastrointest Endosc 2001; 53: 308-312
99. McEntee G, Wiesner RH, Rosen C, Cooper J, Wahlstrom E. A comparative study of patients undergoing liver transplantation for primary sclerosing cholangitis and primary biliary cirrhosis. Transplant Proc 1991; 23: 1563-1564
100. Muiesan P, Shanmugam RP, Devlin J, Rela M, Heaton ND, Saxena R, Portmann B, Tan KC, Williams R. Orthotopic liver transplantation for primary sclerosing cholangitis. Transplant Proc 1994; 26: 3574-3576
101. Farges O, Malassagne B, Sebagh M, Bismuth H. Primary sclerosing cholangitis: liver transplantation or biliary surgery. Surgery 1995; 117: 146-155
102. Narumi S, Roberts JP, Emond JC, Lake J, Ascher NL. Liver transplantation for sclerosing cholangitis. Hepatology 1995; 22: 451-457
103. Ahrendt SA, Pitt HA, Kalloo AN, Venbrux AC, Klein AS, Herlong HF, Coleman J, Lillemoe KD, Cameron JL. Primary sclerosing cholangitis: resect, dilate, or transplant? Ann Surg 1998; 227: 412-423
104. Nashan B, Schlitt HJ, Tusch G, Oldhafer KJ, Ringe B, Wagner S, Pichlmayr R. Biliary malignancies in primary sclerosing cholangitis: timing for liver transplantation. Hepatology 1996; 23: 1105-1111
105. Roberts MS, Angus DC, Bryce CL, Valenta Z, Weissfeld L. Survival after liver transplantation in the United States: a disease-specific analysis of the UNOS database. Liver Transpl 2004; 10: 886-897
106. Merion RM. When is a patient too well and when is a patient too sick for a liver transplant? Liver Transpl 2004; 10: S69-S73
107. Khettry U, Keaveny A, Goldar-Najafi A, Lewis WD, Pomfret EA, Pomposelli JJ, Jenkins RL, Gordon FD. Liver transplantation for primary sclerosing cholangitis: a longterm clinicopathologic study. Hum Pathol 2003; 34: 1127-1136
108. Brandsaeter B, Schrumpf E, Clausen OP, Abildgaard A, Hafsahl G, Bjoro K. Recurrent sclerosing cholangitis or ischemic bile duct lesions--a diagnostic challenge? Liver Transpl 2004; 10: 1073-1074
109. Gordon F. Recurrent primary sclerosing cholangitis: Clinical diagnosis and long-term management issues. Liver Transpl 2006; 12: S73-S75
110. Tamura S, Sugawara Y, Kaneko J, Matsui Y, Togashi J, Makuuchi M. Recurrence of primary sclerosing cholangitis after living donor liver transplantation. Liver Int 2007; 27: 86-94
111. Jones EA, Bergasa NV. The pruritus of cholestasis. Hepatology 1999; 29: 1003-1006
112. Polter DE, Gruhl V, Eigenbrodt EH, Combes B. Beneficial effect of cholestyramine in sclerosing cholangitis. Gastroenterology 1980; 79: 326-333
113. Tabibian N. Rifampin as antipruritic agent in primary sclerosing cholangitis. Am J Gastroenterol 1989; 84: 340
114. Wolfhagen FH, Sternieri E, Hop WC, Vitale G, Bertolotti M, Van Buuren HR. Oral naltrexone treatment for cholestatic pruritus: a double-blind, placebo-controlled study. Gastroenterology 1997; 113: 1264-1269
115. Bergasa NV, Alling DW, Talbot TL, Swain MG, Yurdaydin C, Turner ML, Schmitt JM, Walker EC, Jones EA. Effects of naloxone infusions in patients with the pruritus of cholestasis. A double-blind, randomized, controlled trial. Ann Intern Med 1995; 123: 161-167
116. Jones EA, Molenaar HA, Oosting J. Ondansetron and pruritus in chronic liver disease: a controlled study. Hepatogastroenterology 2007; 54: 1196-1199
117. Mayo MJ, Handem I, Saldana S, Jacobe H, Getachew Y, Rush AJ. Sertraline as a first-line treatment for cholestatic pruritus. Hepatology 2007; 45: 666-674
118. Poupon RE, Chretien Y, Chazouilleres O, Poupon R, Chwalow J. Quality of life in patients with primary biliary cirrhosis. Hepatology 2004; 40: 489-494
119. Angulo P, Therneau TM, Jorgensen A, DeSotel CK, Egan KS, Dickson ER, Hay JE, Lindor KD. Bone disease in patients with primary sclerosing cholangitis: prevalence, severity and prediction of progression. J Hepatol 1998; 29: 729-735
120. Campbell MS, Lichtenstein GR, Rhim AD, Pazianas M, Faust T. Severity of liver disease does not predict osteopenia or low bone mineral density in primary sclerosing cholangitis. Liver Int 2005; 25: 311-316
121. Porayko MK, Wiesner RH, Hay JE, Krom RA, Dickson ER, Beaver S, Schwerman L. Bone disease in liver transplant recipients: incidence, timing, and risk factors. Transplant Proc 1991; 23: 1462-1465
122. Brandt DJ, MacCarty RL, Charboneau JW, LaRusso NF, Wiesner RH, Ludwig J. Gallbladder disease in patients with primary sclerosing cholangitis. AJR Am J Roentgenol 1988; 150: 571-574
123. Leung UC, Wong PY, Roberts RH, Koea JB. Gall bladder polyps in sclerosing cholangitis: does the 1-cm rule apply? ANZ J Surg 2007; 77: 355-357
124. 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: 151-156
125. Stiehl A, Rost D. Endoscopic treatment of dominant stenoses in patients with primary sclerosing cholangitis. Clin Rev Allergy Immunol 2005; 28: 159-165
126. Hirota WK, Petersen K, Baron TH, Goldstein JL, Jacobson BC, Leighton JA, Mallery JS, Waring JP, Fanelli RD, Wheeler-Harbough J, Faigel DO. Guidelines for antibiotic prophylaxis for GI endoscopy. Gastrointest Endosc 2003; 58: 475-482
127. Bjornsson ES, Kilander AF, Olsson RG. Bile duct bacterial isolates in primary sclerosing cholangitis and certain other forms of cholestasis--a study of bile cultures from ERCP. Hepatogastroenterology 2000; 47: 1504-1508
128. Lee YM, Kaplan MM. Management of primary sclerosing cholangitis. Am J Gastroenterol 2002; 97: 528-534
129. 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
130. 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: 523-526
131. Harnois DM, Gores GJ, Ludwig J, Steers JL, LaRusso NF, Wiesner RH. Are patients with cirrhotic stage primary sclerosing cholangitis at risk for the development of hepatocellular cancer? J Hepatol 1997; 27: 512-516
132. Wee A, Ludwig J, Coffey RJ Jr, LaRusso NF, Wiesner RH. Hepatobiliary carcinoma associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hum Pathol 1985; 16: 719-726
133. Kaya M, de Groen PC, Angulo P, Nagorney DM, Gunderson LL, Gores GJ, Haddock MG, Lindor KD. Treatment of cholangiocarcinoma complicating primary sclerosing cholangitis: the Mayo Clinic experience. Am J Gastroenterol 2001; 96: 1164-1169
134. Boberg KM, Bergquist A, Mitchell S, Pares A, Rosina F, Broome U, Chapman R, Fausa O, Egeland T, Rocca G, Schrumpf E. Cholangiocarcinoma in primary sclerosing cholangitis: risk factors and clinical presentation. Scand J Gastroenterol 2002; 37: 1205-1211
135. Ahrendt SA, Pitt HA, Nakeeb A, Klein AS, Lillemoe KD, Kalloo AN, Cameron JL. Diagnosis and management of cholangiocarcinoma in primary sclerosing cholangitis. J Gastrointest Surg 1999; 3: 357-367; discussion 367-368
136. Bergquist A, Ekbom A, Olsson R, Kornfeldt D, Loof L, Danielsson A, Hultcrantz R, Lindgren S, Prytz H, Sandberg-Gertzen H, Almer S, Granath F, Broome U. Hepatic and extrahepatic malignancies in primary sclerosing cholangitis. J Hepatol 2002; 36: 321-327
137. Ramage JK, Donaghy A, Farrant JM, Iorns R, Williams R. Serum tumor markers for the diagnosis of cholangiocarcinoma in primary sclerosing cholangitis. Gastroenterology 1995; 108: 865-869
138. Hultcrantz R, Olsson R, Danielsson A, Jarnerot G, Loof L, Ryden BO, Wahren B, Broome U. A 3-year prospective study on serum tumor markers used for detecting cholangiocarcinoma in patients with primary sclerosing cholangitis. J Hepatol 1999; 30: 669-673
139. Levy C, Lymp J, Angulo P, Gores GJ, Larusso N, Lindor KD. The value of serum CA 19-9 in predicting cholangiocarcinomas in patients with primary sclerosing cholangitis. Dig Dis Sci 2005; 50: 1734-1740
140. Fevery J, Verslype C, Lai G, Aerts R, Van Steenbergen W. Incidence, diagnosis, and therapy of cholangiocarcinoma in patients with primary sclerosing cholangitis. Dig Dis Sci 2007; 52: 3123-3135
141. Brandsaeter B, Isoniemi H, Broome U, Olausson M, Backman L, Hansen B, Schrumpf E, Oksanen A, Ericzon BG, Hockerstedt K, Makisalo H, Kirkegaard P, Friman S, Bjoro K. Liver transplantation for primary sclerosing cholangitis; predictors and consequences of hepatobiliary malignancy. J Hepatol 2004; 40: 815-822
142. Ghali P, Marotta PJ, Yoshida EM, Bain VG, Marleau D, Peltekian K, Metrakos P, Deschenes M. Liver transplantation for incidental cholangiocarcinoma: analysis of the Canadian experience. Liver Transpl 2005; 11: 1412-1416
143. Robles R, Figueras J, Turrion VS, Margarit C, Moya A, Varo E, Calleja J, Valdivieso A, Valdecasas JC, Lopez P, Gomez M, de Vicente E, Loinaz C, Santoyo J, Fleitas M, Bernardos A, Llado L, Ramirez P, Bueno FS, Jaurrieta E, Parrilla P. Spanish experience in liver transplantation for hilar and peripheral cholangiocarcinoma. Ann Surg 2004; 239: 265-271
144. Meyer CG, Penn I, James L. Liver transplantation for cholangiocarcinoma: results in 207 patients. Transplantation 2000; 69: 1633-1637
145. Rea DJ, Heimbach JK, Rosen CB, Haddock MG, Alberts SR, Kremers WK, Gores GJ, Nagorney DM. Liver transplantation with neoadjuvant chemoradiation is more effective than resection for hilar cholangiocarcinoma. Ann Surg 2005; 242: 451-458; discussion 458-461
146. Sudan D, DeRoover A, Chinnakotla S, Fox I, Shaw B Jr, McCashland T, Sorrell M, Tempero M, Langnas A. Radiochemotherapy and transplantation allow long-term survival for nonresectable hilar cholangiocarcinoma. Am J Transplant 2002; 2: 774-779
147. Heimbach JK, Gores GJ, Nagorney DM, Rosen CB. Liver transplantation for perihilar cholangiocarcinoma after aggressive neoadjuvant therapy: a new paradigm for liver and biliary malignancies? Surgery 2006; 140: 331-334
148. 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: 487-491
149. Broome U, Chapman RW. Ulcerative colitis: sclerosing cholangitis today, cancer tomorrow? Gut 1997; 41: 571-572
150. Jayaram H, Satsangi J, Chapman RW. Increased colorectal neoplasia in chronic ulcerative colitis complicated by primary sclerosing cholangitis: fact or fiction? Gut 2001; 48: 430-434
151. Broome U, Lofberg R, Veress B, Eriksson LS. Primary sclerosing cholangitis and ulcerative colitis: evidence for increased neoplastic potential. Hepatology 1995; 22: 1404-1408
152. 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: 1877-8180
153. Kornfeld D, Ekbom A, Ihre T. Is there an excess risk for colorectal cancer in patients with ulcerative colitis and concomitant primary sclerosing cholangitis? A population based study. Gut 1997; 41: 522-525
154. Loftus EV Jr, Sandborn WJ, Tremaine WJ, Mahoney DW, Zinsmeister AR, Offord KP, Melton LJ 3rd. Risk of colorectal neoplasia in patients with primary sclerosing cholangitis. Gastroenterology 1996; 110: 432-440
155. Nuako KW, Ahlquist DA, Sandborn WJ, Mahoney DW, Siems DM, Zinsmeister AR. Primary sclerosing cholangitis and colorectal carcinoma in patients with chronic ulcerative colitis: a case-control study. Cancer 1998; 82: 822-826
156. Loftus EV Jr, Aguilar HI, Sandborn WJ, Tremaine WJ, Krom RA, Zinsmeister AR, Graziadei IW, Wiesner RH. Risk of colorectal neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis following orthotopic liver transplantation. Hepatology 1998; 27: 685-690
157. Tung BY, Emond MJ, Haggitt RC, Bronner MP, Kimmey MB, Kowdley KV, Brentnall TA. 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
158. 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: 889-893
159. Mir-Madjlessi SH, Farmer RG, Sivak MV Jr. Bile duct carcinoma in patients with ulcerative colitis. Relationship to sclerosing
160. Lewis JT, Talwalkar JA, Rosen CB, Smyrk TC, Abraham SC. Prevalence and risk factors for gallbladder neoplasia in patients with primary sclerosing cholangitis: evidence for a metaplasia-dysplasia-carcinoma sequence. Am J Surg Pathol 2007; 31: 907-913
161. Bergquist A, Glaumann H, Persson B, Broome U. Risk factors and clinical presentation of hepatobiliary carcinoma in patients with primary sclerosing cholangitis: a casecontrol study. Hepatology 1998; 27: 311-316
162. Gossard AA, Lindor KD. Pregnancy in a patient with primary sclerosing cholangitis. J Clin Gastroenterol 2002; 35: 353-355
163. Christensen KL, Andersen BN, Vilstrup H. Primary sclerosing cholangitis with itching treated during pregnancy with ursodeoxycholic acid. Ugeskr Laeger 1997; 159: 7151-7153
164. Janczewska I, Olsson R, Hultcrantz R, Broome U. Pregnancy in patients with primary sclerosing cholangitis. Liver 1996; 16: 326-330