Systematic review: Management options for primary sclerosing cholangitis and its variant forms - IgG4-associated cholangitis and overlap with autoimmune hepatitis

Alimentary Pharmacology and Therapeutics

Volumn 33, Issue 12, 2011, Pages 1273-1291

  Culver, E L ^a   Chapman, R W ^a  

^a JOHN RADCLIFFE HOSPITAL   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ANTIBIOTIC AGENT; AZATHIOPRINE; BEZAFIBRATE; BUDESONIDE; CLADRIBINE; COLCHICINE; COLESTYRAMINE; CORTICOSTEROID; CYCLOSPORIN A; ETANERCEPT; FLUOROURACIL; INFLIXIMAB; METHOTREXATE; METHYLPREDNISOLONE; METRONIDAZOLE; MINOCYCLINE; MODAFINIL; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; NICOTINE; PENTOXIFYLLINE; PIRFENIDONE; PLACEBO; PREDNISOLONE; PREDNISONE; RITUXIMAB; SILYMARIN; TACROLIMUS; UNINDEXED DRUG; URSODEOXYCHOLIC ACID; VANCOMYCIN;

ARTICLE; AUTOIMMUNE HEPATITIS; BACTERIAL CHOLANGITIS; BALLOON DILATATION; BILE DUCT CARCINOMA; BILE DUCT OBSTRUCTION; BILE DUCT STENT; CANCER RISK; CANCER SCREENING; CANCER SURVIVAL; CHOLANGIOGRAPHY; CHOLANGITIS; CHOLESTASIS; COLORECTAL CARCINOMA; CONTINUOUS INFUSION; CROHN DISEASE; DISEASE COURSE; DRUG DOSE COMPARISON; DRUG DOSE REDUCTION; DRUG MEGADOSE; ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY; FATIGUE; GALLBLADDER CARCINOMA; HUMAN; IMMUNOGLOBULIN G SUBCLASS 4 ASSOCIATED CHOLANGITIS; IMMUNOSUPPRESSIVE TREATMENT; LIVER BIOPSY; LIVER CELL CARCINOMA; LIVER CIRRHOSIS; LIVER FAILURE; LIVER TRANSPLANTATION; LOW DRUG DOSE; MALABSORPTION; METABOLIC BONE DISEASE; MONOTHERAPY; NONHUMAN; PANCREAS CARCINOMA; PATIENT COMPLIANCE; PRIMARY SCLEROSING CHOLANGITIS; PRIORITY JOURNAL; PROGNOSIS; PRURITUS; SPHINCTEROTOMY; STENT; SURVIVAL RATE; SYSTEMATIC REVIEW; ULCERATIVE COLITIS;

ANIMALS; CHOLANGITIS, SCLEROSING; DISEASE PROGRESSION; FEMALE; HEPATITIS, AUTOIMMUNE; HUMANS; IMMUNOGLOBULIN G; IMMUNOSUPPRESSIVE AGENTS; MALE; MICE; SYNDROME; URSODEOXYCHOLIC ACID;

EID: 79956071011     PISSN: 02692813     EISSN: 13652036     Source Type: Journal    
DOI: 10.1111/j.1365-2036.2011.04658.x     Document Type: Article

References (206)

1. Chapman R, Cullen S,. Etiopathogenesis of primary sclerosing cholangitis. World J Gastroenterol 2008; 14: 3350-9.
2. Karlsen TH, Franke A, Melum E, et al. Genome-wide association analysis in primary sclerosing cholangitis. Gastroenterology 2010; 138: 1102-11.
3. Broome U, Olsson R, Loof L, et al. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis. Gut 1996; 38: 610-5. (Pubitemid 26111384)
4. Abdalian R, Heathcote EJ,. Sclerosing cholangitis: a focus on secondary causes. Hepatology 2006; 44: 1063-74. (Pubitemid 44748357)
5. Boberg KM, 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: 1369-76. (Pubitemid 26181218)
6. Chapman RW, Cottone M, Selby WS, et al. Serum autoantibodies, ulcerative colitis and primary sclerosing cholangitis. Gut 1986; 27: 86-91. (Pubitemid 16134481)
7. Mulder AHL, Horst G, Haagsma EB, et al. Prevalence and characterization of neutrophil cytoplasmic antibodies in autoimmune liver diseases. Hepatology 1993; 17: 411-7. (Pubitemid 23072815)
8. Vitellas KM, El-Dieb A, Vaswani KK, 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. (Pubitemid 34785643)
9. Dave M, Elmunzer BJ, Dwamena BA, et al. Primary sclerosing cholangitis: meta-analysis of diagnostic performance of MR Cholangiopancreatography. Radiology 2010; 256: 387-96.
10. Harrison RF, Hubscher SG,. The spectrum of bile duct lesions in end-stage primary sclerosing cholangitis. Histopathology 1991; 19: 321-7.
11. Burak KW, Angulo P, Lindor KD,. Is there a role for liver biopsy in primary sclerosing cholangitis? Am J Gastroenterol 2003; 98: 1155-8. (Pubitemid 36733781)
12. Bjornsson E, Olsson R, Bergquist A, et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology 2008; 134: 975-80. (Pubitemid 351451987)
13. 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: 586-9. (Pubitemid 34499395)
14. Angulo P, Maor-Kendler Y, Lindor K, et al. Small-duct primary sclerosing cholangitis: a long term follow-up study. Hepatology 2002; 35: 1494-500. (Pubitemid 34966904)
15. Bjornsson E, Bomberg KM, Cullen S, et al. Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis. Gut 2002; 51: 731-5. (Pubitemid 35216864)
16. 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: 1157-66.
17. Charatcharoenwitthaya P, Angulo P, Enders FB, Lindor KD,. Impact of inflammatory bowel disease and ursodeoxycholic acid therapy on small-duct primary sclerosing cholangitis. Hepatology 2008; 47: 133.
18. Gregorio GV, Portmann B, Karani J, et al. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology 2001; 33: 544-53. (Pubitemid 32185215)
19. Al-Chalabi T, Portmann BC, Bernal W, et al. Autoimmune hepatitis overlap syndromes: an evaluation of treatment response, long-term outcome and survival. Aliment Pharmacol Ther 2008; 28: 209-20. (Pubitemid 351861961)
20. van Buuren HR, van Hoogstraten HJE, Terkivatan T, et al. High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis. J Hepatol 2000; 33: 543-8.
21. Kaya M, Angulo P, Lindor KD,. Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system. J Hepatol 2000; 33: 537-42.
22. Floreani A, Rizzotto ER, Ferrara F, et al. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. Am J Gastroenterol 2005; 100: 1516-22. (Pubitemid 41032356)
23. Lewin M, Vilgrain V, Ozeme V, et al. Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: a prospective magnetic resonance imaging and histological study. Hepatology 2009; 50: 528-37.
24. Abdalian R, Dhar P, Jhaverik K, et al. Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: evaluating the role of routine magnetic resonance imaging. Hepatology 2008; 47: 949-57. (Pubitemid 351449686)
25. Abdo AA, Bain VG, Kichian K, et al. Evolution of autoimmune hepatitis to primary sclerosing cholangitis: a sequential syndrome. Hepatology 2002; 36: 1393-9. (Pubitemid 35403530)
26. Boberg KM, Egeland T, Schrumpf E,. Long-term effect of corticosteroid treatment in primary sclerosing cholangitis patients. Scand J Gastroenterol 2003; 38: 991-5. (Pubitemid 37158028)
27. Bjornsson E, Chari ST, Smyrk TC, et al. Immunoglobulin G4 associated cholangitis: description of an emerging clinical entity based on review of the literature. Hepatology 2007; 45: 1547-54. (Pubitemid 46918339)
28. Ghazale A, Chari ST, Zhang L, et al. Immunoglobulin-4 associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008; 134: 706-15. (Pubitemid 351312724)
29. Dastis SN, Latinne D, Sempoux C, et al. Ulcerative colitis associated with IgG4 cholangitis: similar features in two HLA identical siblings. J Hepatol 2009; 51: 601-5.
30. Narula N, Vasudev M, Marshall JK,. IgG(4)-related sclerosing disease: a novel mimic of inflammatory bowel disease. Dig Dis Sci 2010; 55: 3047-51.
31. Mendes FD, Jorgensen R, Keach J, et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 2006; 101: 2070-5. (Pubitemid 44337633)
32. 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.
33. Oh HC, Kim MH, Lee KT, et al. Clinical clues to suspicion of IgG4-associated sclerosing cholangitis disguised as primary sclerosing cholangitis or hilar cholangiocarcinoma. J Gastroenterol Hepatol 2010; 25: 1831-7.
34. Chung H, Watanabe T, Kudo M, et al. Identification and characterization of IgG4-associated autoimmune hepatitis. Liver Intern 2010; 30: 222-31.
35. Topazian M, Witzig TE, Smyrk TC, et al. Rituximab therapy for refractory biliary strictures in immunoglobulin G4 associated cholangitis. Clin Gastroenterol Hepatol 2008; 6: 364-6. (Pubitemid 351318380)
36. Kim WR, Therneau TM, Wiesner RH, et al. A revised natural history model for primary sclerosing cholangitis. Mayo Clin Proc 2000; 75: 688-94. (Pubitemid 30441497)
37. Kim WR, Poterucha JJ, Wiesner RH, et al. The relative role of the Child-Pugh classification and the Mayo natural history model in the assessment of survival in patients with primary sclerosing cholangitis. Hepatology 1999; 29: 1643-8. (Pubitemid 29247033)
38. Bambha K, Kim WR, Talwalkar J, et al. Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community. Gastroenterology 2003; 125: 1364-9. (Pubitemid 37409756)
39. Ponsioen CY, Vrouenraets SM, Prawirodirdjo W, et al. Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population. Gut 2002; 51: 562-6.
40. Mendes FD, Kim WR, Pedersen R, et al. Mortality attributable to cholestatic liver disease in the United States. Hepatology 2008; 47: 1241-7. (Pubitemid 351547910)
41. Paumgartner G, Beuers U,. Ursodeoxycholic acid in cholestatic liver diseases. Hepatology 2002; 36: 525-31.
42. Martinez JD, Stratagoules ED, La Rue JM, et al. Different bile acids exhibit distinct biological effects: the tumour promoter deoxycholic acid induces apoptosis and the chemopreventive agent ursodeoxycholic acid inhibits cell proliferation. Nutr Cancer 1998; 31: 111-8. (Pubitemid 28428071)
43. Beuers U, Spengler U, Kruis W, et al. Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial. Hepatology 1992; 16: 707-14.
44. Chazouilleres O, Poupon R, Capron JP, et al. Ursodeoxycholic acid for primary sclerosing cholangitis. J Hepatol 1990; 11: 120-3. (Pubitemid 20250837)
45. O'Brien CB, Senior JR, Arora-Mirchandani R, et al. Ursodeoxycholic acid for the treatment of primary sclerosing cholangitis: a 30-month pilot study. Hepatology 1991; 14: 838-47.
46. Stiehl A,. Ursodeoxycholic acid therapy in treatment of primary sclerosing cholangitis. Scand J Gastroenterol Suppl 1994; 204: 59-61. (Pubitemid 24302613)
47. Lindor KD,. Ursodiol for primary sclerosing cholangitis. Mayo primary sclerosing cholangitis-ursodeoxycholic acid study group. N Engl J Med 1997; 336: 691-5.
48. Lindor KD, Kowdley KV, Luketic VAC, et al. High dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology 2009; 50: 808-14.
49. Mitchell SA, Bansi DS, Hunt N, et al. A preliminary trial of high-dose ursodeoxycholic acid in primary sclerosing cholangitis. Gastroenterology 2001; 121: 900-7. (Pubitemid 32959399)
50. Olsson R, Boberg KM, de Muckadell OS, et al. High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5-year multicentre, randomized, controlled study. Gastroenterology 2005; 129: 1464-72. (Pubitemid 41597389)
51. Cullen SN, 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.
52. Lindor KD, Enders FB, Schmoll JA, et al. Randomized, double-blind controlled trial of high-dose ursodeoxycholic acid for primary sclerosing cholangitis. Hepatology 2008; 47: 133-42. (Pubitemid 351171050)
53. 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: 1238-51.
54. Beneditti A, Alvaro D, Bassotti C, et al. Cytotoxicity of bile salts against biliary epithelium: a study in isolated bile ductile fragments and isolated perfused rat liver. Hepatology 1997; 26: 9-21. (Pubitemid 27280301)
55. 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.
56. Tung BY, Emond MJ, Haggitt RC, 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. (Pubitemid 32096335)
57. Pardi DS, Loftus EV Jr, Kremers WK, et al. Ursodeoxycholic acid as a chemopreventive agent in patients with ulcerative colitis and primary sclerosing cholangitis. Gastroenterology 2003; 124: 889-93. (Pubitemid 36389790)
58. Wolf JM, Rybicki LA, Lashner BA,. The impact of ursodeoxycholic acid cancer, dysplasia and mortality in ulcerative colitis patients with primary sclerosing cholangitis. Aliment Pharmacol Ther 2005; 22: 783-8. (Pubitemid 41564357)
59. 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: 487-91. (Pubitemid 46725755)
60. Brandsaeter B, Isoniemi H, Broome′ U, et al. Liver transplantation for primary sclerosing cholangitis; predictors and consequences of hepatobiliary malignancy. J Hepatol 2004; 40: 815-22. (Pubitemid 38507363)
61. Halilbasic E, Fiorotto R, Fickert P, et al. Side chain structure determines unique physiologic and therapeutic properties of norursodeoxycholic acid in Mdr-/- mice. Hepatology 2009; 49: 1972-82.
62. Glaser SS, Alpini G, Hightower NC,. Activation of the cholehepatic shunt as a potential therapy for primary sclerosing cholangitis. Hepatology 2009; 49: 1790-2.
63. Cullen SN, Chapman RW,. Review article: current management of primary sclerosing cholangitis. Aliment Pharmacol Ther 2005; 21: 933-48. (Pubitemid 40532978)
64. Chapman R, Fevery J, Kalloo A, et al. AASLD practice guidelines: diagnosis and management of primary sclerosing cholangitis. Hepatology 2010; 51: 660-78.
65. Bjornsson E, Lindqvist-Ottosson J, Asztely M, et al. Dominant strictures in patients with primary sclerosing cholangitis. Am J Gastroenterol 2004; 99: 502-8. (Pubitemid 38524130)
66. 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: 151-6. (Pubitemid 34146420)
67. Rudolph G, Gotthardt D, Klöters-Plachky P, et al. Influence of dominant bile duct stenoses and biliary infections on outcome in primary sclerosing cholangitis. J Hepatol 2009; 51: 149-55.
68. Lindberg B, Arnelo U, Bergquist A, et al. Diagnosis of biliary strictures in conjunction with endoscopic retrograde cholangiopancreaticography, with special reference to patients with primary sclerosing cholangitis. Endoscopy 2002; 34: 909-16. (Pubitemid 35331183)
69. Gaing AA, Geders JM, Cohen SA, et al. Endoscopic management of primary sclerosing cholangitis: review, and report of an open series. Am J Gastroenterol 1993; 88: 2000-8. (Pubitemid 23352160)
70. Johnson GK, Geenen JE, Venu RP, et al. Endoscopic treatment of biliary tract strictures in sclerosing cholangitis: a larger series and recommendations for treatment. Gastrointest Endosc 1991; 37: 38-43.
71. Kaya M, Petersen BT, Angulo P, et al. Balloon dilation compared to stenting of dominant strictures in primary sclerosing cholangitis. Am J Gastroenterol 2001; 96: 1059-66. (Pubitemid 32319137)
72. Gaing AA, Geders JM, Cohen SA, et al. Endoscopic therapy for primary sclerosing cholangitis: report of an ongoing series. Gastrointest Endosc 1992; 38: 261-2.
73. Baluyut AR, Sherman S, Lehman GA, et al. Impact of endoscopic therapy on the survival of patients with primary sclerosing cholangitis. Gastrointest Endosc 2001; 53: 308-12. (Pubitemid 32207314)
74. Stiehl A, Rudolph G, Sauer P, et al. 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: 560-6. (Pubitemid 27112721)
75. van Milligen de Wit AW, van Bracht J, Rauws EA, et al. Endoscopic stent therapy for dominant extrahepatic bile duct strictures in primary sclersosing cholangitis. Gastrointest Endosc 1996; 44: 293-9. (Pubitemid 26318331)
76. Bangarulingam SY, Gossard AA, Petersen BT, et al. Complications of endoscopic retrograde cholangiopancreatography in primary sclerosing cholangitis. Am J Gastroenterol 2009; 104: 855-60.
77. Eickhoff A, Schilling D, Jakobs R, et al. Long-term outcome of percutaneous transhepatic drainage for benign bile duct stenoses. Rocz Akad Med Bialymst 2005; 50: 155-60.
78. Ahrendt SA, Pitt HA, Kalloo AN, et al. Primary sclerosing cholangitis: resect, dilate, or transplant? Ann Surg 1998; 227: 412-23.
79. Angulo P, Lindor KD,. Primary sclerosing cholangitis. Hepatology 1999; 30: 325-32. (Pubitemid 29300604)
80. Farges O, Malassagne B, Sebagh M, et al. Primary sclerosing cholangitis: liver transplantation or biliary surgery. Surgery 1995; 117: 146-55.
81. Muiesan P, Shanmugam RP, Delvin J, et al. Orthotopic liver transplantation for primary sclerosing cholangitis. Transplant Proc 1994; 26: 3574-6.
82. Narumi S, Roberts JP, Emond JC, et al. Liver transplant for sclerosing cholangitis. Hepatology 1995; 22: 451-7.
83. Tsai S, Pawlik TM,. Primary sclerosing cholangitis: the role of extrahepatic biliary resection. Adv Surg 2009; 43: 175-88.
84. Pawlick TM, Olbrecht VA, Pitt HA, et al. Primary sclerosing cholangitis: role of extrahepatic biliary resection. J Am Coll Surg 2008; 206: 822-30.
85. Cameron JL, Pitt HA, Zinner MJ, et al. Resection of hepatic duct bifurcation and transhepatic stenting for sclerosing cholangitis. Ann Surg 1988; 207: 614-22.
86. Myburgh JA,. Surgical biliary drainage in primary sclerosing cholangitis. The role of the Hepp-Couinaud approach. Arch Surg 1994; 129: 1057-62. (Pubitemid 24320799)
87. Kaplan GG, Laupland KB, Butzner D, et al. The burden of large and small duct primary sclerosing cholangitis in adults and children: a population-based analysis. Am J Gastroenterol 2007; 102: 1042-9. (Pubitemid 46705300)
88. 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. (Pubitemid 43004884)
89. Negm AA, Schott A, Vonberg RP, et al. Routine bile collection for microbiological analysis during cholangiography and its impact on the management of cholangitis. Gastrointest Endosc 2010; 72: 284-91.
90. Abraham SC, Kamath PS, Eghtesad B, et al. Liver transplantation in precirrhotic biliary tract disease: portal hypertension is frequently associated with nodular regenerative hyperplasia and obliterative portal venopathy. Am J Surg Pathol 2006; 30: 1454-61. (Pubitemid 44655482)
91. Bergquist A, Ekbom A, Olsson R,. Hepatic and extrahepatic malignancies in primary sclerosing cholangitis. J Hepatol 2002; 36: 321-7. (Pubitemid 34194790)
92. Maggs JR, Chapman RW,. An update on primary sclerosing cholangitis. Curr Opin Gastroenterol 2008; 24: 377-83. (Pubitemid 351535898)
93. Burak K, Angulo P, Pasha TM, et al. Incidence and risk factors for cholangiocarcinoma in primary sclerosing cholangitis. Am J Gastroenterol 2004; 99: 523-6. (Pubitemid 38524134)
94. Boberg KM, Bergquist A, Mitchell S, et al. Cholangiocarcinoma in primary sclerosing cholangitis: risk factors and clinical presentation. Scand J Gastroenterol 2002; 37: 1205-11. (Pubitemid 35189610)
95. Kornfeld D, Ekbom A, Ihre T,. Survival and risk of cholangiocarcinoma in patients with primary sclersosing cholangitis. A population based study. Scand J Gastroenterol 1997; 32: 1042-5. (Pubitemid 27433668)
96. Helzberg JH, Petersen JM, Boyer JL,. Improved survival with primary sclersosing cholangitis. A review of clinicopathologic features and comparison of symptomatic and asymptomatic patients. Gastroenterol 1987; 92: 1869-75. (Pubitemid 17072235)
97. Lazaridis KN, Gores GJ,. Primary sclerosing cholangitis and cholangiocarcinoma. Semin Liver Dis 2006; 26: 42-51. (Pubitemid 43320726)
98. Yachimski P, Pratt DS,. Cholangiocarcinoma: natural history, treatment and strategies for surveillance in high risk patients. J Clin Gastroenterol 2008; 42: 178-90.
99. Charatcharoenwitthaya P, Enders FB, Halling KC, et al. Utility of serum tumor markers, imaging, and biliary cytology for detecting cholangiocarcinoma in primary sclerosing cholangitis. Hepatology 2008; 48: 1106-17.
100. Angulo P, Pearce DH, Johnson CD, et al. Magnetic resonance cholangiography in patients with biliary disease: its role in primary sclerosing cholangitis. J Hepatol 2000; 33: 520-7.
101. Rabinovitz M, Zajko AM, Hassanein T, et al. Diagnostic value of brush cytology in the diagnosis of bile duct carcinoma: a study of 65 patients with bile duct strictures. Hepatology 1990; 12: 747-52. (Pubitemid 20349261)
102. Boberg KM, Jebsen P, Clausen OP, et al. Diagnostic benefit of biliary brush cytology in cholangiocarcinoma in primary sclerosing cholangitis. J Hepatol 2006; 45: 568-74. (Pubitemid 44307380)
103. Moreno Luna LE, Kipp B, Halling KC, et al. Advanced cytological techniques for the detection of malignant pancreatobiliary strictures. Gastroenterology 2006; 131: 1064-72. (Pubitemid 44498705)
104. Kipp BR, Stadheim LM, Halling SA, et al. A comparison of routine cytology and fluorescence in situ hybridisation for the detection of malignant bile duct strictures. Am J Gastroenterol 2004; 99: 1675-81. (Pubitemid 39237490)
105. Levy C, Lymp J, Angulo P, et al. The value of serum CA 19-9 in predicting cholangiocarcinomas in patients with primary sclerosing cholangitis. Dig Dis Sci 2005; 50: 1734-40. (Pubitemid 41203674)
106. Steinberg W,. The clinical utility of the CA 19-9 tumor-associated antigen. Am J Gastroenterol 1990; 85: 350-5. (Pubitemid 20140602)
107. Fritscher-Ravens A, Broering DC, Knoefel WT, et al. EUS-guided fine-needle aspiration of suspected hilar cholangiocarcinoma in potentially operable patients with negative brush cytology. Am J Gastroenterol 2004; 99: 45-51. (Pubitemid 38679100)
108. Gleeson FC, Rajan E, Levy MJ, et al. EUS-guided FNA of regional lymph nodes in patients with unresectable hilar cholangiocarcinoma. Gastrointest Endosc 2008; 67: 438-43. (Pubitemid 351260042)
109. Fevery J, Verslype C, Lai G, et al. Incidence, diagnosis and therapy of cholangiocarcinoma in patients with primary sclerosing cholangitis. Dig Dis Sci 2007; 52: 3123-35. (Pubitemid 47512534)
110. Meza-Junco J, Montano-Loza AJ, Ma M, et al. Cholangiocarcinoma: has there been any progress. Can J Gastroenterol 2010; 24: 52-7.
111. IIwatsuki S, Todo S, Marsh JW, et al. Treatment of hilar cholangiocarcinoma (Klatskin tumours) with hepatic resection or transplantation. J Am Coll Surg 1998; 187: 358-64. (Pubitemid 28467610)
112. Rea DJ, Heimbach JK, Rosen CB, et al. Liver transplantation with neoadjuvant chemoradiation is more effective than resection for hilar cholangiocarcinoma. Ann Surg 2005; 242: 451-8; discussion 458-461.
113. Hemibach JK, Gores GJ, Nagorney DM, et al. Liver transplantation for perihilar cholangiocarcinoma after aggressive neoadjuvant therapy: a new paradigm for liver and biliary malignancies? Surgery 2006; 140: 331-4.
114. Gores GJ, Nagorney DM, Rosen CB,. Cholangiocarcinoma: is transplantation an option? For whom? J Hepatol 2007; 47: 455-9. (Pubitemid 47331754)
115. Sudan D, DeRoover A, Chinnakotla S, et al. Radiochemotherapy and transplantation allow long-term survival for non-resectable hilar cholangiocarcinoma. Am J Transplant 2002; 2: 774-9. (Pubitemid 36342621)
116. Kahaleh M, Mishra R, Shami VM, et al. Unresectable cholangiocarcinoma: comparison of survival in biliary stenting alone versus stenting with photodynamic therapy. Clin Gastroenterol Hepatol 2008; 6: 290-7.
117. Brandt DJ, MacCarty RL, Charboneau JW, et al. Gallbladder disease in patients with primary sclerosing cholangitis. Am J Roentgenol 1988; 150: 571-4. (Pubitemid 18075395)
118. Said K, Glaumann H, Bergquist A,. Gallbladder disease in patients with primary sclerosing cholangitis. J Hepatol 2008; 48: 598-605.
119. Buckles DC, Lindor KD, Larusso NF, et al. In primary sclerosing cholangitis, gallbladder polyps are frequently malignant. Am J Gastroenterol 2002; 97: 1138-42. (Pubitemid 34478111)
120. Lewis JT, Talwalker JA, Rosen CB, et al. Prevalence and risk factors for gallbladder neoplasia in patients with primary sclerosing cholangitis: evidence for metaplasia-dysplasia-carcinoma sequence. Am J Surg Pathol 2007; 31: 907-13. (Pubitemid 47165145)
121. Leung UC, Wong PY, Roberts RH, et al. Gallbladder polyps in sclerosing cholangitis: does the 1 cm rule apply? ANZ J Surg 2007; 77: 355-7. (Pubitemid 46709712)
122. Beuers U, Boberg K, Chapman RW, et al. EASL clinical practice guidelines: management of cholestatic liver diseases. J Hepatol 2009; 51: 237-67.
123. Harnois DM, Gores GJ, Ludwig J, et al. Are patients with cirrhotic stage primary sclerosing cholangitis at risk for the development of hepatocellular cancer? J Hepatol 1997; 27: 512-6. (Pubitemid 27390899)
124. Bruix J, Sherman M,. ASSLD practice guidelines: management of hepatocellular carcinoma. Hepatology 2005; 42: 1208-35.
125. Jones EA, Bergasa NV,. The pruritis of cholestasis. Hepatology 1999; 29: 1003-6.
126. Datta DV, Sherlock S,. Cholestyramine for long term relief of pruritus complicating intrahepatic cholestasis. Gastroenterology 1966; 50: 323-32.
127. Polter DE, Gruhl V, Eigenbrodt EH, et al. Beneficial effects of cholestyramine in sclersosing cholangitis. Gastroenterology 1980; 79: 326-33. (Pubitemid 10046377)
128. Khurana S, Singh P,. Rifampin is safe for the treatment of pruritus due to chronic cholestasis: a meta-analysis of prospective randomized-controlled trials. Liver Int 2006; 26: 943-8. (Pubitemid 44323412)
129. Tandon P, Rowe BH, Vandermeer B, et al. The efficacy and safety of bile acid binding agents, opioid antagonists or rifampicin in the treatment of cholestasis-associated pruritus. Am J Gastroenterol 2007; 102: 1528-36. (Pubitemid 46976283)
130. Wolfhagen FH, Sternieri E, Hop WC, et al. Oral naltrexone treatment for cholestatic pruritus: a double-blind, placebo-controlled study. Gastroenterology 1997; 113: 1264-9. (Pubitemid 27418980)
131. Bergasa NV, Alling DW, Talbot TL, et al. Effects of naloxone infusions in patients with the pruritus of cholestasis. A double-blind, randomized, controlled trial. Ann Intern Med 1995; 123: 161-7.
132. Jones EA, Molenaar HA, Oosting J,. Ondansetron and pruritus in chronic liver disease: a controlled study. Hepatogastroenterology 2007; 54: 1196-9. (Pubitemid 47013934)
133. Mayo MJ, Handem I, Saldana S, Jacobe H, Getachew Y, Rush AJ,. Sertraline as a first-line treatment for cholestatic pruritus. Hepatology 2007; 45: 666-74. (Pubitemid 46450618)
134. Epstein MP, Kaplan MM,. A pilot study of etanercept in the treatment of primary sclerosing cholangitis. Dig Dis Sci 2004; 49: 1-4. (Pubitemid 38183736)
135. Jones DE, Newton JL,. An open study of modafinil for the treatment of daytime solomnence and fatigue in primary biliary cirrhosis. Aliment Pharmocol Ther 2007; 25: 471-6. (Pubitemid 46184461)
136. Angulo P, Therneau TM, Jorgensen A, et al. Bone disease in patients with primary sclerosing cholangitis: prevalence, severity and prediction of progression. J Hepatol 1998; 29: 729-35. (Pubitemid 28505929)
137. Porayko MK, Wiesner RH, Hay JE, et al. Bone disease in liver transplant recipients: incidence, timing, and risk factors. Transplant Proc 1991; 23: 1462-5.
138. Pares A, Guanabens N,. Osteoporosis in primary biliary cirrhosis: pathogenesis and treatment. Clin Liver Dis 2008; 12: 407-24. (Pubitemid 351635892)
139. Pereira SP, O'Donohue J, Moniz C, et al. Transdermal hormone replacement therapy imporves vertebral bone density in primary biliary cirrhosis: results of a one year controlled trial. Aliment Pharmacol Therap 2007; 25: 471-6.
140. Wiesner RH, Grambsch PM, Dickson ER, et al. Primary sclerosing cholangitis: natural history, prognostic factors and survival analysis. Hepatology 1989; 10: 430-6. (Pubitemid 19255462)
141. Chapman RW, Arborgh BA, Rhodes JM, et al. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut 1980; 21: 870-7. (Pubitemid 11198911)
142. Fausa O, Schrumpf E, Elgjo K,. Relationship of inflammatory bowel disease and primary sclerosing cholangitis. Semin Liver Dis 1991; 11: 31-9.
143. Verdonk RC, Dijkstra G, Haagsma EB, et al. Inflammatory bowel disease after liver transplantation: risk factors for recurrence and de novo disease. Am J Transplant 2006; 6: 1422-9. (Pubitemid 44030325)
144. Loftus EV Jr, Harewood GC, Loftus CG, et al. PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis. Gut 2005; 54: 91-6. (Pubitemid 40007225)
145. Broome U, Bergquist A,. Primary sclerosing cholangitis, inflammatory bowel disease, and colon cancer. Semin Liver Dis 2006; 26: 31-41. (Pubitemid 43320725)
146. Lundqvist K, Broome U,. Differences in colonic disease activity in patients with ulcerative colitis with and without primary sclerosing cholangitis: a case control study. Dis Colon Rectum 1997; 40: 451-6. (Pubitemid 27172624)
147. Penna C, Dozois R, Tremaine W, et al. Pouchitis after ileal pouch-anal anastomosis for ulcerative colitis occurs with increased frequency in patients with associated primary sclerosing cholangitis. Gut 1996; 38: 234-9. (Pubitemid 26046819)
148. Wiesner RH, LaRusso NF, Dozois RR, et al. Peristomal varices after proctocolectomy in patients with primary sclerosing cholangitis. Gastroenterology 1986; 90: 316-22. (Pubitemid 16136670)
149. Schrumpf E, Elgjo K, Fausa O, et al. Sclerosing cholangitis in ulcerative colitis. Scand J Gastroenterol 1980; 15: 689-97. (Pubitemid 11225943)
150. Olsson R, Danielsson A, Jarnerot G, et al. Prevalence of primsary sclerosing cholangitis in ulcerative colitis. Gastroenterology 1991; 100: 1319-23.
151. Rabinovitz M, Gavaler JS, Schade RR, et al. 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. (Pubitemid 20075094)
152. 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. (Pubitemid 41115048)
153. Broome U, Lofberg R, Veress B, Eriksson LS,. Primary sclerosing cholangitis and ulcerative colitis: evidence for increased neoplastic potential. Hepatology 1995; 22: 1404.
154. Naggengast F, Grubben M, van Munster I,. Role of bile acids in colorectal carcinogenesis. Eur J Cancer 1995; 31: 1067-70.
155. Shetty K, Rybicki L, Brzezinski A, et al. The risk for cancer or dysplasia in ulcerative colitis patients with primary sclerosing cholangitis. Am J Gastroenterol 1999; 94: 1643-9. (Pubitemid 29260459)
156. Marchesa P, Lashner BA, Lavery IC, et al. The risk of cancer and dysplasia among ulcerative colitis patients with primary sclerosing cholangitis. Am J Gastroenterol 1997; 92: 1285.
157. Loftus EV Jr, Aguilar HI, Sandborn W, et al. Risk of colorectal neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis following orthotopic liver transplantation. Hepatology 1998; 27: 685-90. (Pubitemid 28103543)
158. Cairns S, Scholefield JH, Steele RJ, et al. Guidelines for colorectal cancer screening and surveillance in moderate and high risk groups. Gut 2010; 59: 666-89.
159. Kaplan GG, Heitman SJ, Hilsden RJ, et al. Population-based analysis of practices and costs of surveillance for colonic dysplasia in patients with primary sclerosing cholangitis and colitis. Inflamm Bowel Dis 2007; 13: 1401-7. (Pubitemid 350206853)
160. Kitiyakara T, Chapman RW,. Chemoprevention and screening in primary sclerosing cholangitis. Postgrad Med J 2008; 84: 228-37. (Pubitemid 351839229)
161. MacLean AR,. Outcome of patients undergoing liver transplantation for primary sclerosing cholangitis. Dis Colon Rectum 2003; 46: 1124-8. (Pubitemid 36992998)
162. Bjøro K, Brandsaeter B, Foss A, Schrumpf E,. Liver transplantation in primary sclerosing cholangitis. Semin Liver Dis 2006; 26: 69-79. (Pubitemid 43320729)
163. Wiesner RH, McDiarmid SV, Kamath PS, et al. MELD and PELD: application of survival models to liver allocation. Liver Transpl 2001; 7: 567-80. (Pubitemid 32677880)
164. Freeman RB, Edwards EB, Harper AM,. Waiting list removal rates among patients with chronic and malignant liver diseases. Am J Transplant 2006; 6: 1416-21. (Pubitemid 44030324)
165. Neuberger J, Gimson A, Davies M, et al. Selection of patients for liver transplantation and allocation of donated livers in the UK. Gut 2008; 57: 252-7. (Pubitemid 351158055)
166. Brandsaeter B, Friman S, Broome U, et al. Outcome following liver transplantation for primary sclerosing cholangitis in the Nordic countries. Scand J Gastroenterol 2003; 38: 1176-83. (Pubitemid 37518603)
167. Graziadei IW, Wiesner RH, Marotta PJ, et al. Long-term results of patients undergoing liver transplantation for primary sclerosing cholangitis. Hepatology 1999; 30: 1121-7. (Pubitemid 29510847)
168. Roberts MS, Angus DC, Bryce CL, et al. Survival after liver transplantation in the United states: a disease specific analysis of the UNOS database. Liver Transpl 2004; 10: 886-97. (Pubitemid 38979259)
169. Campsen J, Zimmerman MA, Trotter JF, et al. Clinically recurrent primary sclerosing cholangitis following liver transplantation: a time course. Liver Transpl 2008; 14: 181-5. (Pubitemid 351267274)
170. Graziadei IW, Wiesner RH, Batts KP, et al. Recurrence of primary sclerosing cholangitis following liver transplantation. Hepatology 1999; 29: 1050-6. (Pubitemid 29168735)
171. Tamura S, Sugawara Y, Kaneko J, et al. Recurrence of primary sclerosing cholangitis after living donor transplantation. Liver Int 2007; 27: 86-94. (Pubitemid 46139432)
172. Wojcicki M, Milkiewicz P, Silva M,. Biliary tract complications after liver transplantation: a review. Dig Surg 2008; 25: 245-57.
173. Alabraba E, Nightingale P, Gunson B, et al. A re-evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts. Liver Transplant 2009; 15: 330-40.
174. La Russo NF, Shneider BL, Black D, et al. Primary sclerosing cholangitis: summary of a workshop. Hepatology 2006; 44: 746-64. (Pubitemid 44433732)
175. Lo SK, Hermann R, Chapman RW, et al. Ursodeoxycholic acid in primary sclerosing cholangitis: a double blind placebo controlled trial. Hepatology 1992; 16: 92A.
176. 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. (Pubitemid 24043086)
177. 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: 1472-9. (Pubitemid 27007335)
178. Van Hoogstraten HJ, Wolfhagen FH, van de Meeberg PC, et al. Ursodeoxycholic acid therapy for primary sclersosing cholangitis: results of a 2 year randomised controlled trial to evaluate single vs. multiple daily disease. J Hepatol 1998; 29: 417-23. (Pubitemid 28396658)
179. 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: 1558-62. (Pubitemid 32455090)
180. Okolicsanyi L, Groppo M, Floreani A, et al. Treatment of primary sclerosing cholangitis with low dose ursodeoxycholic acid: results of a retrospective Italian multicentre survey. Dig Liver Dis 2003; 35: 325-31. (Pubitemid 41110859)
181. Angulo P, Batts KP, Jorgensen RA, et al. Oral budesonide in the treatment of primary sclerosing cholangitis. Am J Gastroenterol 2000; 95: 2333-7.
182. Knox TA, Kaplan MM,. Treatment of primary sclerosing cholangitis with oral methotrexate. Am J Gastroenterol 1991; 86: 546.
183. Knox TA, Kaplan MM,. A double-blind controlled trial of oral-pulse methotrexate therapy in the treatment of primary sclerosing cholangitis. Gastroenterology 1994; 106: 494-9. (Pubitemid 24041229)
184. Sandborn WJ, Wiesner RH, Tremaine WJ, et al. Ulcerative colitis disease activity following treatment of associated primary sclerosing cholangitis with cyclosporin. Gut 1993; 34: 242-6. (Pubitemid 23049120)
185. Van Thiel DH, Carroll P, Abu-Elmagd K, et al. Tacrolimus (FK506), a treatment for primary sclerosing cholangitis: results of an open-label preliminary trial. Am J Gastroenterol 1995; 90: 455-9.
186. Olsson R, Broome U, Danielsson A, et al. Colchicine treatment of primary sclerosing cholangitis. Gastroenterology 1995; 108: 1199-203.
187. LaRusso NF, Wiesner RH, Ludwig J, et al. Prospective trial of penicillamine in primary sclerosing cholangitis. Gastroenterology 1988; 95: 1036-42.
188. Bharucha AE, Jorgensen R, Lichtman SN, et al. A pilot study of pentoxifylline for the treatment of primary sclerosing cholangitis. Am J Gastroenterol 2000; 95: 2338-42.
189. Duchini A, Younossi ZM, Saven A, et al. An open-label pilot trial of cladibrine (2-cholordeoxyadenosine) in patients with primary sclerosing cholangitis. J Clin Gastroenterol 2000; 31: 292-6.
190. Angulo P, MacCarty RL, Sylvestre PB, et al. Pirfenidone in the treatment of primary sclerosing cholangitis. Dig Dis Sci 2002; 47: 157-61. (Pubitemid 34075033)
191. Angulo P, Bharucha AE, Jorgensen RA, et al. Oral nicotine in treatment of primary sclerosing cholangitis: a pilot study. Dig Dis Sci 1999; 44: 602-7. (Pubitemid 29137974)
192. Vleggaar FP, van Buuren HR, van Berge Henegouwen GP, et al. No beneficial effects of transdermal nicotine in patients with primary sclerosing cholangitis: results of a randomized double-blind placebo-controlled cross-over study. Eur J Gastroenterol Hepatol 2001; 13: 171-5. (Pubitemid 32158211)
193. Talwalkar JA, Angulo P, Keach JC, Lindor KD, et al. Mycophenolate mofetil for the treatment of primary sclerosing cholangitis. Am J Gastroenterol 2005; 100: 308-12. (Pubitemid 40314514)
194. Talwalkar JA, Gossard AA, Keach JC, et al. Tacrolimus for the treatment of primary sclerosing cholangitis. Liver Int 2007; 27: 451-3. (Pubitemid 46534566)
195. Hommes DW, Erkelens W, Ponsioen C, et al. A double-blind, placebo-controlled, randomized study of infliximab in primary sclerosing cholangitis. J Clin Gastroenterol 2008; 42: 522-6. (Pubitemid 351572276)
196. Angulo P, Jorgensen RA, Kowdley KV, Lindor KD,. Silmarin in the treatment of patients with primary sclerosing cholangitis: an open label pilot study. Dig Dis Sci 2008; 53: 1716-20. (Pubitemid 351624429)
197. Mizuno S, Hirano K, Tada M, et al. Bezafibrate for the treatment of primary sclerosing cholangitis. J Gastroenterol 2010; 45: 758-62.
198. Lindor KD, Wiesner RH, Colwell L, et al. The combination of prednisone and colchicine in patients with primary sclerosing cholangitis. Am J Gastroenterol 1991; 86: 57.
199. Kyokane K, Ichihara T, Horisawa M, et al. Successful treatment of primary sclerosing cholangitis with cyclosporine and corticosteroid. Hepatogastroenterology 1994; 41: 449.
200. Lindor KD, Jorgensen RA, Anderson ML, et al. Ursodeoxycholic acid and methotrexate for primary sclerosing cholangitis: a pilot study. Am J Gastroenterol 1996; 91: 511.
201. Schramm C, Schirmacher P, Helmreich-Becker I, et al. Combined therapy with azathioprine, prednisolone, and ursodiol in patients with primary sclerosing cholangitis. Ann Intern Med 1999; 131: 943.
202. van Hoogstraten HJ, Vleggaar FP, Boland GJ, et al. Budesonide or prednisone in combination with ursodeoxycholic acid in primary sclerosing cholangitis: a randomized double-blind pilot study. Belgian-Dutch PSC Study Group. Am J Gastroenterol 2000; 95: 2015.
203. Sterling RK, Salvatori JJ, Luketic VA, et al. 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: 943-9. (Pubitemid 39593275)
204. Davies YK, Cox KM, Abdullah BA, et al. Long-term treatment of primary sclerosing cholangitis in children with oral vancomycin: an immunomodulating antibiotic. J Pediatr Gastroenterol Nutr 2008; 47: 61.
205. Silveira MG, Torok NJ, Gossard AA, et al. Minocycline in the treatment of patients with primary sclerosing cholangitis: results of a pilot study. Am J Gastroenterol 2009; 104: 83.
206. Farkkila M, Karvonen AL, Nurmi H, et al. Metronidazole and ursodeoxycholic acid for primary sclerosing cholangitis: a randomized placebo-controlled trial. Hepatology 2004; 40: 1379.