Diagnosis and management of primary sclerosing cholangitis

Hepatology

Volumn 51, Issue 2, 2010, Pages 660-678

  Chapman, Roger ^a   Fevery, Johan ^b   Kalloo, Anthony ^c   Nagorney, David M ^d   Boberg, Kirsten Muri ^e   Shneider, Benjamin ^f   Gores, Gregory J ^d  

^a JOHN RADCLIFFE HOSPITAL   (United Kingdom)

^b UNIVERSITY HOSPITALS LEUVEN   (Belgium)

^c JOHNS HOPKINS HOSPITAL   (United States)

^d MAYO CLINIC   (United States)

^e OSLO UNIVERSITY HOSPITAL   (Norway)

^f CHILDREN S HOSPITAL OF PITTSBURGH   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AUTOANTIBODY; AZATHIOPRINE; BUDESONIDE; COLCHICINE; CORTICOSTEROID DERIVATIVE; CYCLOSPORIN; ETANERCEPT; METHOTREXATE; MYCOPHENOLIC ACID; PENICILLAMINE; PENTOXIFYLLINE; PIRFENIDONE; PREDNISOLONE; TACROLIMUS; URSODEOXYCHOLIC ACID;

AUTOIMMUNE PANCREATITIS; BACTERIAL COLONIZATION; BILE DUCT CARCINOMA; CANCER RISK; CHOLELITHIASIS; CLINICAL FEATURE; DIAGNOSTIC IMAGING; DIFFERENTIAL DIAGNOSIS; DISEASE ASSOCIATION; ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY; ENDOSCOPIC SPHINCTEROTOMY; ENTERITIS; FERTILITY; GALLBLADDER CANCER; HUMAN; LIVER BIOPSY; LIVER TRANSPLANTATION; METABOLIC BONE DISEASE; PHOTODYNAMIC THERAPY; PORTAL HYPERTENSION; PREGNANCY; PRIMARY SCLEROSING CHOLANGITIS; PRIORITY JOURNAL; REVIEW; SKIN PRURITUS;

ALGORITHMS; BILE DUCT NEOPLASMS; BILE DUCTS, INTRAHEPATIC; CHOLANGIOCARCINOMA; CHOLANGITIS, SCLEROSING; CONSTRICTION, PATHOLOGIC; HUMANS; INFLAMMATORY BOWEL DISEASES;

EID: 75449104123     PISSN: 02709139     EISSN: 15273350     Source Type: Journal    
DOI: 10.1002/hep.23294     Document Type: Review

References (193)

1. Eddy DM. A Manual for Assessing Health Care Practices and Designing Practice Guidelines. Philadelphia, PA: American College of Physicians; 1996:1-126.
2. Position and policy statement: American Gastroenterological Association policy statement on the use of medical practice guidelines by managed care organizations and insurance carriers. Gastroenterology 1995;108: 925-926.
3. Guyatt GH, Oxman AD, Vist GE, Kunz R, Falck-Ytter Y, Alonso-Coello P, et al. GRADE: an emerging consensus on rating quality of evidence and strength of recommendations. BMJ 2008;336:924-926.
4. Maggs JR, Chapman RW. An update on primary sclerosing cholangitis. Curr Opin Gastroenterol 2008;24:377-383.
5. Tischendorf JJ, Hecker H, Kruger M, Manns MP, Meier PN. Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study. Am J Gastroenterol 2007;102:107-114.
6. Bjornsson E, Olsson R, Bergquist A, Lindgren S, Braden B, Chapman RW, et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology 2008;134:975-980.
7. Gohlke F, Lohse AW, Dienes HP, Lohr H, Marker-Hermann E, Gerken G, et al. Evidence for an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis. J Hepatol 1996;24:699-705.
8. Abdalian R, Heathcote EJ. Sclerosing cholangitis: a focus on secondary causes. HEPATOLOGY 2006;44:1063-1074.
9. 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.
10. Broome U, Olsson R, Loof L, Bodemar G, Hultcrantz R, Danielsson A, et al. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis. Gut 1996;38:610-615.
11. Beuers U, Spengler U, Kruis W, Aydemir U, Wiebecke B, Heldwein W, et al. Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial. HEPATOLOGY 1992;16:707-714.
12. Bambha K, Kim WR, Talwalkar J, Torgerson H, Benson JT, Therneau TM, et al. Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community. Gastroenterology 2003; 125:1364-1369.
13. Chapman RW, Arborgh BA, Rhodes JM, Summerfield JA, Dick R, Scheuer PJ, et al. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut 1980;21:870-877.
14. Boberg KM, Fausa O, Haaland T, Holter E, Mellbye OJ, Spurkland A, 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.
15. Hov JR, Boberg KM, Karlsen TH. Autoantibodies in primary sclerosing cholangitis. World J Gastroenterol 2008;14:3781-3791.
16. van de Meeberg PC, Portincasa P, Wolfhagen FH, van Erpecum KJ, VanBerge-Henegouwen GP. Increased gall bladder volume in primary sclerosing cholangitis. Gut 1996;39:594-599.
17. Said K, Glaumann H, Bergquist A. Gallbladder disease in patients with primary sclerosing cholangitis. J Hepatol 2008;48:598-605.
18. Campbell WL, Ferris JV, Holbert BL, Thaete FL, Baron RL. Biliary tract carcinoma complicating primary sclerosing cholangitis: evaluation with CT, cholangiography, US, and MR imaging. Radiology 1998;207:41-50.
19. Ament AE, Haaga JR, Wiedenmann SD, Barkmeier JD, Morrison SC. Primary sclerosing cholangitis: CT findings. J Comput Assist Tomogr 1983;7:795-800.
20. Campbell WL, Peterson MS, Federle MP, Siqueira ES, Slivka A, Grazioli L, et al. Using CT and cholangiography to diagnose biliary tract carcinoma complicating primary sclerosing cholangitis. AJR Am J Roentgenol 2001;177:1095-1100.
21. DoddGD3rd, Baron RL, Oliver JH 3rd, Federle MP. End-stage primary sclerosing cholangitis: CT findings of hepatic morphology in 36 patients. Radiology 1999;211:357-362.
22. Johnson KJ, Olliff JF, Olliff SP. The presence and significance of lymphadenopathy detected by CT in primary sclerosing cholangitis. Br J Radiol 1998;71:1279-1282.
23. MacCarty RL, LaRusso NF, Wiesner RH, Ludwig J. Primary sclerosing cholangitis: findings on cholangiography and pancreatography. Radiology 1983;149:39-44.
24. Lee YM, Kaplan MM. Primary sclerosing cholangitis. N Engl J Med 1995;332:924-933.
25. Bangarulingam SY, Gossard AA, Petersen BT, Ott BJ, Lindor KD. Complications of endoscopic retrograde cholangiopancreatography in primary sclerosing cholangitis. Am J Gastroenterol 2009;104:855-860.
26. Berstad AE, Aabakken L, Smith HJ, Aasen S, Boberg KM, Schrumpf E. Diagnostic accuracy of magnetic resonance and endoscopic retrograde cholangiography in primary sclerosing cholangitis. Clin Gastroenterol Hepatol 2006;4:514-520.
27. Angulo P, Pearce DH, Johnson CD, Henry JJ, LaRusso NF, Petersen BT, et al. Magnetic resonance cholangiography in patients with biliary disease: its role in primary sclerosing cholangitis. J Hepatol 2000;33:520-527.
28. Burak KW, Angulo P, Lindor KD. Is there a role for liver biopsy in primary sclerosing cholangitis? Am J Gastroenterol 2003;98:1155-1158. 29. el-Shabrawi M, Wilkinson ML, Portmann B, Mieli-Vergani G, Chong SK, Williams R, et al. Primary sclerosing cholangitis in childhood. Gastroenterology 1987;92:1226-1235.
29. Minuk GY, Sutherland LR, Pappas G, Kelly JK, Martin SE. Autoimmune chronic active hepatitis (lupoid hepatitis) and primary sclerosing cholangitis in two yound adult females. Can J Gastroenterol 1988;2:22-27.
30. Rabinovitz M, Demetris AJ, Bou-Abboud CF, Van Thiel DH. Simultaneous occurrence of primary sclerosing cholangitis and autoimmune chronic active hepatitis in a patient with ulcerative colitis. Dig Dis Sci 1992;37:1606-1611.
31. Lawrence SP, Sherman KE, Lawson JM, Goodman ZD. A 39 year old man with chronic hepatitis. Semin Liver Dis 1994;14:97-105. 33. Debray D, Pariente D, Urvoas E, Hadchouel M, Bernard O. Sclerosing cholangitis in children. J Pediatr 1994;124:49-56.
32. Wilschanski M, Chait P, Wade JA, Davis L, Corey M, St Louis P, et al. Primary sclerosing cholangitis in 32 children: clinical, laboratory, and radiographic features, with survival analysis. HEPATOLOGY 1995;22: 1415-1422.
33. McNair AN, Moloney M, Portmann BC, Williams R, McFarlane IG. Autoimmune hepatitis overlapping with primary sclerosing cholangitis in five cases. Am J Gastroenterol 1998;93:777-784.
34. Gregorio GV, Portmann B, Karani J, Harrison P, Donaldson PT, Vergani D, et al. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. HEPATOLOGY 2001;33: 544-553.
35. Al-Chalabi T, Portmann BC, Bernal W, McFarlane IG, Heneghan MA. Autoimmune hepatitis overlap syndromes: an evaluation of treatment response, long-term outcome and survival. Aliment Pharmacol Ther 2008;28:209-220.
36. Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 1999;31:929-938.
37. Beuers U, Rust C. Overlap syndromes. Semin Liver Dis 2005;25:311-320.
38. 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.
39. Kaya M, Angulo P, Lindor KD. Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system. J Hepatol 2000;33:537-542.
40. Floreani A, Rizzotto ER, Ferrara F, Carderi I, Caroli D, Blasone L, et al. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. Am J Gastroenterol 2005;100:1516-1522.
41. Kamisawa T, Egawa N, Tsuruta K, Okamoto A, Funata N. Primary sclerosing cholangitis may be overestimated in Japan. J Gastroenterol 2005;40:318-319.
42. 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.
43. Mendes FD, Jorgensen R, Keach J, Katzmann JA, Smyrk T, Donlinger J, et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 2006;101:2070-2075.
44. 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.
45. Bjornsson E, Lindqvist-Ottosson J, Asztely M, Olsson R. Dominant strictures in patients with primary sclerosing cholangitis. Am J Gastroenterol 2004;99:502-508.
46. Okolicsanyi L, Fabris L, Viaggi S, Carulli N, Podda M, Ricci G. Primary sclerosing cholangitis: clinical presentations, natural history and prognostic variables: an Italian multicenter study. Eur J Gastroenterol Hepatol 1996;8:685-691.
47. Lindberg B, Arnelo U, Bergquist A, Thorne A, Hjerpe A, Granqvist S, et al. Diagnosis of biliary strictures in conjunction with endoscopic retrograde cholangiopancreaticography, with special reference to patients with primary sclerosing cholangitis. Endoscopy 2002;34:909-916.
48. Wiesner RH, Grambsch PM, Dickson ER, Ludwig J, MacCarty RL, Hunter EB, et al. Primary sclerosing cholangitis: natural history, prognostic factors and survival analysis. HEPATOLOGY 1989;10:430-436.
49. May GR, Bender CE, LaRusso NF, Wiesner RH. Nonoperative dilatation of dominant strictures in primary sclerosing cholangitis. AJR Am J Roentgenol 1985;145:1061-1064.
50. Kaya M, Petersen BT, Angulo P, Baron TH, Andrews JC, Gostout CJ, et al. Balloon dilation compared to stenting of dominant strictures in primary sclerosing cholangitis. Am J Gastroenterol 2001;96:1059-1066.
51. Ahrendt SA, Pitt HA, Kalloo AN, Venbrux AC, Klein AS, Herlong HF, et al. Primary sclerosing cholangitis: resect, dilate, or transplant? Ann Surg 1998;227:412-423.
52. Gaing AA, Geders JM, Cohen SA, Siegel JH. Endoscopic management of primary sclerosing cholangitis: review, and report of an open series. Am J Gastroenterol 1993;88:2000-2008.
53. Johnson GK, Geenen JE, Venu RP, Schmalz MJ, Hogan WJ. Endoscopic treatment of biliary tract strictures in sclerosing cholangitis: a larger series and recommendations for treatment. Gastrointest Endosc 1991;37:38-43.
54. Gaing AA, Geders JM, Cohen SA, Siegel JH. Endoscopic therapy for primary sclerosing cholangitis: Report of an ongoing series. Gastrointest Endosc 1992;38:261-262.
55. Kalloo AN. Primary sclerosing cholangitis: an overview. In: Cameron JL, ed. Current Surgical Therapy. 7th ed. St. Louis, MO: Mosby; 2001:474-476.
56. 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.
57. Gluck M, Cantone NR, Brandabur JJ, Patterson DJ, Bredfeldt JE, Kozarek RA. A twenty-year experience with endoscopic therapy for symptomatic primary sclerosing cholangitis. J Clin Gastroenterol 2008;42:1032-1039.
58. Myburgh JA. Surgical biliary drainage in primary sclerosing cholangitis. The role of the Hepp-Couinaud approach. Arch Surg 1994;129:1057-1062.
59. Cameron JL, Pitt HA, Zinner MJ, Herlong HF, Kaufman SL, Boitnott JK, et al. Resection of hepatic duct bifurcation and transhepatic stenting for sclerosing cholangitis. Ann Surg 1988;207:614-622.
60. Pawlick TM, Olbrecht VA, Pitt HA, Gleisner AL, Choti MA, Schulick RD, et al. Primary sclerosing cholangitis: role of extrahepatic biliary resection. J Am Coll Surg 2008;206:822-830.
61. 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.
62. Boomkens SY, de Rave S, Pot RG, Egberink HF, Penning LC, Rothuizen J, et al. The role of Helicobacter spp. in the pathogenesis of primary biliary cirrhosis and primary sclerosing cholangitis. FEMS Immunol Med Microbiol 2005;44:221-225.
63. 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.
64. 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.
65. Abraham SC, Kamath PS, Eghtesad B, Demetris AJ, Krasinskas AM. 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;30:1454-1461.
66. Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ. Primary biliary cirrhosis. HEPATOLOGY 2009;50:291-308.
67. Zein CO, Lindor KD, Angulo P. Prevalence and predictors of esophageal varices in patients with primary sclerosing cholangitis. HEPATOLOGY 2004;39:204-210.
68. Zapata-Colindres JC, Montano-Loza A, Zepeda-Gomez S, Uscanga L. Predictive factors for portal hypertension in patients with primary sclerosing cholangitis [in Spanish]. Gastroenterol Hepatol 2006;29:7-10.
69. Garcia-Tsao G, Sanyal AJ, Grace ND, Carey W. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. HEPATOLOGY 2007;46:922-938.
70. Angulo P, Therneau TM, Jorgensen A, DeSotel CK, Egan KS, Dickson ER, et al. Bone disease in patients with primary sclerosing cholangitis: prevalence, severity and prediction of progression. J Hepatol 1998;29: 729-735.
71. 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.
72. Collier J. Bone disorders in chronic liver disease. HEPATOLOGY 2007;46: 1271-1278.
73. Zein CO, Jorgensen RA, Clarke B, Wenger DE, Keach JC, Angulo P, et al. Alendronate improves bone mineral density in primary biliary cirrhosis: a randomized placebo-controlled trial. HEPATOLOGY 2005;42:762-771.
74. Fausa O, Schrumpf E, Elgjo K. Relationship of inflammatory bowel disease and primary sclerosing cholangitis. Semin Liver Dis 1991;11:31-39.
75. Loftus EV Jr, Harewood GC, Loftus CG, Tremaine WJ, Harmsen WS, Zinsmeister AR, et al. PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis. Gut 2005;54:91-96.
76. 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.
77. Broome U, Bergquist A. Primary sclerosing cholangitis, inflammatory bowel disease, and colon cancer. Semin Liver Dis 2006;26:31-41.
78. Kitiyakara T, Chapman RW. Chemoprevention and screening in primary sclerosing cholangitis. Postgrad Med J 2008;84:228-237.
79. Aadland E, Schrumpf E, Fausa O, Elgjo K, Heilo A, Aakhus T,et al. Primary sclerosing cholangitis: a long-term follow-up study. Scand J Gastroenterol 1987;22:655-664.
80. Schrumpf E, Elgjo K, Fausa O, Gjone E, Kolmannskog F, Ritland S. Sclerosing cholangitis in ulcerative colitis. Scand J Gastroenterol 1980; 15:689-697.
81. Verdonk RC, Dijkstra G, Haagsma EB, Shostrom VK, Van den Berg AP, Kleibeuker JH, et al. Inflammatory bowel disease after liver transplantation: risk factors for recurrence and de novo disease. Am J Transplant 2006;6:1422-1429.
82. Faubion WA Jr, Loftus EV, Sandborn WJ, Freese DK, Perrault J. Pediatric "PSC-IBD": a descriptive report of associated inflammatory bowel disease among pediatric patients with psc. J Pediatr Gastroenterol Nutr 2001;33:296-300.
83. 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;40:451-456.
84. Kartheuser AH, Dozois RR, LaRusso NF, Wiesner RH, Ilstrup DM, Schleck CD. Comparison of surgical treatment of ulcerative colitis associated with primary sclerosing cholangitis: ileal pouch-anal anastomosis versus Brooke ileostomy. Mayo Clin Proc 1996;71:748-756.
85. Penna C, Dozois R, Tremaine W, Sandborn W, LaRusso N, Schleck C, et al. Pouchitis after ileal pouch-anal anastomosis for ulcerative colitis occurs with increased frequency in patients with associated primary sclerosing cholangitis. Gut 1996;38:234-239.
86. Stahlberg D, Veress B, Tribukait B, Broome U. Atrophy and neoplastic transformation of the ileal pouch mucosa in patients with ulcerative colitis and primary sclerosing cholangitis: a case control study. Dis Colon Rectum 2003;46:770-778.
87. Eaden JA, Mayberry JF. Guidelines for screening and surveillance of asymptomatic colorectal cancer in patients with inflammatory bowel disease. Gut 2002;51(Suppl. 5):V10-V12.
88. Bansal P, Sonnenberg A. Risk factors of colorectal cancer in inflammatory bowel disease. Am J Gastroenterol 1996;91:44-48.
89. Brentnall TA, Haggitt RC, Rabinovitch PS, Kimmey MB, Bronner MP, Levine DS, et al. Risk and natural history of colonic neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis. Gastroenterology 1996;110:331-338.
90. 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.
91. Shetty K, Rybicki L, Brzezinski A, Carey WD, Lashner BA. The risk for cancer or dysplasia in ulcerative colitis patients with primary sclerosing cholangitis. Am J Gastroenterol 1999;94:1643-1649.
92. Soetikno RM, Lin OS, Heidenreich PA, Young HS, Blackstone MO. Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis: a meta-analysis. Gastrointest Endosc 2002;56:48-54.
93. Brackmann S, Andersen SN, Aamodt G, Langmark F, Clausen OP, Aadland E, et al. Relationship between clinical parameters and the colitiscolorectal cancer interval in a cohort of patients with colorectal cancer in inflammatory bowel disease. Scand J Gastroenterol 2008:1-10.
94. 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.
95. Vleggaar FP, Lutgens MW, Claessen MM. Review article: The relevance of surveillance endoscopy in long-lasting inflammatory bowel disease. Aliment Pharmacol Ther 2007;26(Suppl. 2):47-52.
96. Kaplan GG, Heitman SJ, Hilsden RJ, Urbanski S, Myers RP, Lee SS, 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;13:1401-1407.
97. 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.
98. Tung BY, Emond MJ, Haggitt RC, Bronner MP, Kimmey MB, Kowdley KV, 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.
99. Wolf JM, Rybicki LA, Lashner BA. The impact of ursodeoxycholic acid on cancer, dysplasia and mortality in ulcerative colitis patients with primary sclerosing cholangitis. Aliment Pharmacol Ther 2005;22:783-788.
100. Lindor KD, Kowdley KV, Luketic VA, Harrison ME, McCashland T, Befeler AS, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. HEPATOLOGY 2009;50:808-814.
101. Wiesner RH, LaRusso NF, Dozois RR, Beaver SJ. Peristomal varices after proctocolectomy in patients with primary sclerosing cholangitis. Gastro-enterology 1986;90:316-322.
102. Gorgun E, Remzi FH, Manilich E, Preen M, Shen B, Fazio VW. Surgical outcome in patients with primary sclerosing cholangitis undergoing ileal pouch-anal anastomosis: a case-control study. Surgery 2005;138:631-637; discussion 637-639.
103. 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.
104. Buckles DC, Lindor KD, Larusso NF, Petrovic LM, Gores GJ. In primary sclerosing cholangitis, gallbladder polyps are frequently malignant. Am J Gastroenterol 2002;97:1138-1142.
105. 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.
106. Bergquist A, Ekbom A, Olsson R, Kornfeldt D, Loof L, Danielsson A, et al. Hepatic and extrahepatic malignancies in primary sclerosing cholangitis. J Hepatol 2002;36:321-327.
107. 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.
108. Claessen MM, Vleggaar FP, Tytgat KM, Siersema PD, van Buuren HR. High lifetime risk of cancer in primary sclerosing cholangitis. J Hepatol 2009;50:158-164.
109. Miros M, Kerlin P, Walker N, Harper J, Lynch S, Strong R. Predicting cholangiocarcinoma in patients with primary sclerosing cholangitis before transplantation. Gut 1991;32:1369-1373.
110. Farges O, Malassagne B, Sebagh M, Bismuth H. Primary sclerosing cholangitis: liver transplantation or biliary surgery. Surgery 1995;117: 146-155.
111. Lazaridis KN, Gores GJ. Primary sclerosing cholangitis and cholangiocarcinoma. Semin Liver Dis 2006;26:42-51.
112. Melum E, Karlsen TH, Schrumpf E, Bergquist A, Thorsby E, Boberg KM, et al. Cholangiocarcinoma in primary sclerosing cholangitis is associated with NKG2D polymorphisms. HEPATOLOGY 2008;47:90-96.
113. Boberg KM, Bergquist A, Mitchell S, Pares A, Rosina F, Broome U, et al. Cholangiocarcinoma in primary sclerosing cholangitis: risk factors and clinical presentation. Scand J Gastroenterol 2002;37:1205-1211.
114. Broome U, Lofberg R, Veress B, Eriksson LS. Primary sclerosing cholangitis and ulcerative colitis: evidence for increased neoplastic potential. HEPATOLOGY 1995;22:1404-1408.
115. 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.
116. Steinberg W. The clinical utility of the CA 19-9 tumor-associated antigen. Am J Gastroenterol 1990;85:350-355.
117. Nichols JC, Gores GJ, LaRusso NF, Wiesner RH, Nagorney DM, Ritts RE Jr. Diagnostic role of serum CA 19-9 for cholangiocarcinoma in patients with primary sclerosing cholangitis. Mayo Clin Proc 1993;68: 874-879.
118. 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.
119. Chalasani N, Baluyut A, Ismail A, Zaman A, Sood G, Ghalib R, et al. Cholangiocarcinoma in patients with primary sclerosing cholangitis: a multicenter case-control study. HEPATOLOGY 2000;31:7-11.
120. Charatcharoenwitthaya P, Enders FB, Halling KC, Lindor KD. Utility of serum tumor markers, imaging, and biliary cytology for detecting cholangiocarcinoma in primary sclerosing cholangitis. HEPATOLOGY 2008; 48:1106-1117.
121. Siqueira E, Schoen RE, Silverman W, Martin J, Rabinovitz M, Weissfeld JL, et al. Detecting cholangiocarcinoma in patients with primary sclerosing cholangitis. Gastrointest Endosc 2002;56:40-47.
122. Tischendorf JJ, Kruger M, Trautwein C, Duckstein N, Schneider A, Manns MP, et al. Cholangioscopic characterization of dominant bile duct stenoses in patients with primary sclerosing cholangitis. Endoscopy 2006; 38:665-669.
123. Moreno Luna LE, Kipp B, Halling KC, Sebo TJ, Kremers WK, Roberts LR, et al. Advanced cytologic techniques for the detection of malignant pancreatobiliary strictures. Gastroenterology 2006;131:1064-1072.
124. Boberg KM, Jebsen P, Clausen OP, Foss A, Aabakken L, Schrumpf E. Diagnostic benefit of biliary brush cytology in cholangiocarcinoma in primary sclerosing cholangitis. J Hepatol 2006;45:568-574.
125. Keiding S, Hansen SB, Rasmussen HH, Gee A, Kruse A, Roelsgaard K, et al. Detection of cholangiocarcinoma in primary sclerosing cholangitis by positron emission tomography. HEPATOLOGY 1998;28:700-706.
126. Fevery J, Buchel O, Nevens F, Verslype C, Stroobants S, Van Steenbergen W. 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.
127. Khan SA, Miras A, Pelling M, Taylor-Robinson SD. Cholangiocarcinoma and its management. Gut 2007;56:1755-1756.
128. Kaya M, de Groen PC, Angulo P, Nagorney DM, Gunderson LL, Gores GJ, et al. Treatment of cholangiocarcinoma complicating primary sclerosing cholangitis: the Mayo Clinic experience.AmJ Gastroenterol 2001; 96:1164-1169.
129. Rosen CB, Nagorney DM. Cholangiocarcinoma complicating primary sclerosing cholangitis. Semin Liver Dis 1991;11:26-30.
130. Rosen CB, Nagorney DM, Wiesner RH, Coffey RJ Jr, LaRusso NF. Cholangiocarcinoma complicating primary sclerosing cholangitis. Ann Surg 1991;213:21-25.
131. Gores GJ, Nagorney DM, Rosen CB. Cholangiocarcinoma: is transplantation an option? For whom? J Hepatol 2007;47:455-459.
132. Rea DJ, Heimbach JK, Rosen CB, Haddock MG, Alberts SR, Kremers WK, et al. Liver transplantation with neoadjuvant chemoradiation is more effective than resection for hilar cholangiocarcinoma. Ann Surg 2005;242:451-458; discussion 458-461.
133. Fritscher-Ravens A, Broering DC, Knoefel WT, Rogiers X, Swain P, Thonke F, 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.
134. Gleeson FC, Rajan E, Levy MJ, Clain JE, Topazian MD, Harewood GC, et al. EUS-guided FNA of regional lymph nodes in patients with unresectable hilar cholangiocarcinoma. Gastrointest Endosc 2008;67:438-443.
135. Baron TH. Photodynamic therapy: standard of care for palliation of cholangiocarcinoma? Clin Gastroenterol Hepatol 2008;6:266-267.
136. Kahaleh M, Mishra R, Shami VM, Northup PG, Berg CL, Bashlor P, et al. Unresectable cholangiocarcinoma: comparison of survival in biliary stenting alone versus stenting with photodynamic therapy. Clin Gastroenterol Hepatol 2008;6:290-297.
137. Ortner MA, Liebetruth J, Schreiber S, Hanft M, Wruck U, Fusco V, et al. Photodynamic therapy of nonresectable cholangiocarcinoma. Gastroenterology 1998;114:536-542.
138. Helzberg JH, Petersen JM, Boyer JL. Improved survival with primary sclerosing cholangitis. A review of clinicopathologic features and comparison of symptomatic and asymptomatic patients. Gastroenterology 1987; 92:1869-1875.
139. Shetty K, Rybicki L, Carey WD. The Child-Pugh classification as a prognostic indicator for survival in primary sclerosing cholangitis. HEPATOLOGY 1997;25:1049-1053.
140. Kim WR, Poterucha JJ, Wiesner RH, LaRusso NF, Lindor KD, Petz J, 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;29:1643-1648.
141. Boberg KM, Rocca G, Egeland T, Bergquist A, Broome U, Caballeria L, et al. Time-dependent Cox regression model is superior in prediction of prognosis in primary sclerosing cholangitis. HEPATOLOGY 2002;35:652-657.
142. Majoie CB, Reeders JW, Sanders JB, Huibregtse K, Jansen PL. Primary sclerosing cholangitis: a modified classification of cholangiographic findings. AJR Am J Roentgenol 1991;157:495-497.
143. Ponsioen CY, Vrouenraets SM, Prawirodirdjo W, Rajaram R, Rauws EA, Mulder CJ, et al. Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population. Gut 2002; 51:562-566.
144. Rudolph G, Gotthardt D, Kloters-Plachky P, Kulaksiz H, Rost D, Stiehl A. Influence of dominant bile duct stenoses and biliary infections on outcome in primary sclerosing cholangitis. J Hepatol 2009;51:149-155.
145. Cullen SN, Chapman RW. The medical management of primary sclerosing cholangitis. Semin Liver Dis 2006;26:52-61.
146. Chazouilleres O, Poupon R, Capron JP, Metman EH, Dhumeaux D, Amouretti M, et al. Ursodeoxycholic acid for primary sclerosing cholangitis. J Hepatol 1990;11:120-123.
147. 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.
148. Stiehl A. Ursodeoxycholic acid therapy in treatment of primary sclerosing cholangitis. Scand J Gastroenterol Suppl 1994;204:59-61.
149. Lindor KD. Ursodiol for primary sclerosing cholangitis. Mayo Primary Sclerosing Cholangitis-Ursodeoxycholic Acid Study Group. N Engl J Med 1997;336:691-695.
150. Olsson R, Boberg KM, de Muckadell OS, Lindgren S, Hultcrantz R, Folvik G, et al. High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5-year multicenter, randomized, controlled study. Gastro-enterology 2005;129:1464-1472.
151. Lindor KD, Enders FB, Schmoll JA, Hoskin TL, Jorgensen RA, Petz JL, et al. Randomized, double-blind controlled trial of high-dose ursodeoxycholic acid for primary sclerosing cholangitis [Abstract]. HEPATOLOGY 2008;48:378A.
152. Cullen SN, Chapman RW. Review article: current management of primary sclerosing cholangitis. Aliment Pharmacol Ther 2005;21:933-948. 155. Woodward J, Neuberger J. Autoimmune overlap syndromes. HEPATOLOGY 2001;33:994-1002.
153. Boberg KM, Egeland T, Schrumpf E. Long-term effect of corticosteroid treatment in primary sclerosing cholangitis patients. Scand J Gastroenterol 2003;38:991-995.
154. Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008;134:706-715.
155. Wiesner RH, McDiarmid SV, Kamath PS, Edwards EB, Malinchoc M, Kremers WK, et al. MELD and PELD: application of survival models to liver allocation. Liver Transpl 2001;7:567-580.
156. Freeman RB, Edwards EB, Harper AM. Waiting list removal rates among patients with chronic and malignant liver diseases.AmJ Transplant 2006; 6:1416-1421.
157. Bergquist A, Glaumann H, Persson B, Broome U. Risk factors and clinical presentation of hepatobiliary carcinoma in patients with primary sclerosing cholangitis: a case-control study. HEPATOLOGY 1998;27:311-316.
158. Leidenius M, Hockersted K, Broome U, Ericzon BG, Friman S, Olausson M, et al. Hepatobiliary carcinoma in primary sclerosing cholangitis: a case control study. J Hepatol 2001;34:792-798.
159. Graziadei IW, Wiesner RH, Marotta PJ, Porayko MK, Hay JE, Charlton MR, et al. Long-term results of patients undergoing liver transplantation for primary sclerosing cholangitis. HEPATOLOGY 1999;30:1121-1127.
160. Brandsaeter B, Friman S, Broome U, Isoniemi H, Olausson M, Backman L, et al. Outcome following liver transplantation for primary sclerosing cholangitis in the Nordic countries. Scand J Gastroenterol 2003;38: 1176-1183.
161. Welsh FK, Wigmore SJ. Roux-en-Y Choledochojejunostomy is the method of choice for biliary reconstruction in liver transplantation for primary sclerosing cholangitis. Transplantation 2004;77:602-604.
162. Mathis KL, Dozois EJ, Larson DW, Cima RR, Sarmiento JM, Wolff BG, et al. Ileal pouch-anal anastomosis and liver transplantation for ulcerative colitis complicated by primary sclerosing cholangitis. Br J Surg 2008;95: 882-886.
163. Florman S, Schiano T, Kim L, Maman D, Levay A, Gondolesi G, et al. The incidence and significance of late acute cellular rejection (>1000 days) after liver transplantation. Clin Transplant 2004;18:152-155.
164. Campsen J, Zimmerman MA, Trotter JF, Wachs M, Bak T, Steinberg T, et al. Clinically recurrent primary sclerosing cholangitis following liver transplantation: a time course. Liver Transpl 2008;14:181-185.
165. Alabraba E, Nightingale P, Gunson B, Hubscher S, Olliff S, Mirza D, et al. A re-evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts. Liver Transpl 2009;15:330-340.
166. Wojcicki M, Milkiewicz P, Silva M. Biliary tract complications after liver transplantation: a review. Dig Surg 2008;25:245-257.
167. Alexander J, Lord JD, Yeh MM, Cuevas C, Bakthavatsalam R, Kowdley KV. Risk factors for recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl 2008;14:245-251.
168. Cholongitas E, Shusang V, Papatheodoridis GV, Marelli L, Manousou P, Rolando N, et al. Risk factors for recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl 2008;14:138-143.
169. Rowe IA, Webb K, Gunson BK, Mehta N, Haque S, Neuberger J. The impact of disease recurrence on graft survival following liver transplantation: a single centre experience. Transpl Int 2008;21:459-465.
170. GuichelaarMM,Kendall R, Malinchoc M, Hay JE. Bone mineral density before and after OLT: long-term follow-up and predictive factors. Liver Transpl 2006;12:1390-1402.
171. Dvorchik I, Subotin M, Demetris AJ, Fung JJ, Starzl TE, Wieand S, et al. Effect of liver transplantation on inflammatory bowel disease in patients with primary sclerosing cholangitis. HEPATOLOGY 2002;35:380-384.
172. Vera A, Gunson BK, Ussatoff V, Nightingale P, Candinas D, Radley S, et al. Colorectal cancer in patients with inflammatory bowel disease after liver transplantation for primary sclerosing cholangitis. Transplantation 2003;75:1983-1988.
173. Loftus EV Jr, Aguilar HI, Sandborn WJ, Tremaine WJ, Krom RA, Zinsmeister AR, et al. Risk of colorectal neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis following orthotopic liver transplantation. HEPATOLOGY 1998;27:685-690.
174. Gossard AA, Lindor KD. Pregnancy in a patient with primary sclerosing cholangitis. J Clin Gastroenterol 2002;35:353-355.
175. Janczewska I, Olsson R, Hultcrantz R, Broome U. Pregnancy in patients with primary sclerosing cholangitis. Liver 1996;16:326-330.
176. Riis L, Vind I, Politi P, Wolters F, Vermeire S, Tsianos E, et al. Does pregnancy change the disease course? A study in a European cohort of patients with inflammatory bowel disease.AmJ Gastroenterol 2006;101:1539-1545.
177. Kondrackiene J, Kupcinskas L. Intrahepatic cholestasis of pregnancy-current achievements and unsolved problems. World J Gastroenterol 2008;14:5781-5788.
178. 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;48:939-944.
179. United Network for Organ Sharing. UNOS Data Report. http://www. unos.org/data/. Accessed October 2009.
180. Feldstein AE, Perrault J, El-Youssif M, Lindor KD, Freese DK, Angulo P. Primary sclerosing cholangitis in children: a long-term follow-up study. HEPATOLOGY 2003;38:210-217.
181. Miloh T, Arnon R, Shneider B, Suchy F, Kerkar N. A retrospective single-center review of primary sclerosing cholangitis in children. Clin Gastroenterol Hepatol 2009;7:239-245.
182. Jacquemin E, De Vree JM, Cresteil D, Sokal EM, Sturm E, Dumont M, et al. The wide spectrum of multidrug resistance 3 deficiency: from neonatal cholestasis to cirrhosis of adulthood. Gastroenterology 2001;120: 1448-1458.
183. Ziol M, Barbu V, Rosmorduc O, Frassati-Biaggi A, Barget N, Hermelin B, et al. ABCB4 heterozygous gene mutations associated with fibrosing cholestatic liver disease in adults. Gastroenterology 2008; 135:131-141.
184. Bjornsson E, Angulo P. Cholangiocarcinoma in young individuals with and without primary sclerosing cholangitis. Am J Gastroenterol 2007; 102:1677-1682.
185. Ebbeson RL, Schreiber RA. Diagnosing autoimmune hepatitis in children: is the International Autoimmune Hepatitis Group scoring system useful? Clin Gastroenterol Hepatol 2004;2:935-940.
186. Floreani A, Zancan L, Melis A, Baragiotta A, Chiaramonte M. Primary sclerosing cholangitis (PSC): clinical, laboratory and survival analysis in children and adults. Liver 1999;19:228-233.
187. Chavhan GB, Roberts E, Moineddin R, Babyn PS, Manson DE. Primary sclerosing cholangitis in children: utility of magnetic resonance cholan-giopancreatography. Pediatr Radiol 2008;38:868-873.
188. Batres LA, Russo P, Mathews M, Piccoli DA, Chuang E, Ruchelli E. Primary sclerosing cholangitis in children: a histologic follow-up study. Pediatr Dev Pathol 2005;8:568-576.
189. Yerushalmi B, Sokol RJ, Narkewicz MR, Smith D, Karrer FM. Use of rifampin for severe pruritus in children with chronic cholestasis. J Pediatr Gastroenterol Nutr 1999;29:442-447.
190. Cies JJ, Giamalis JN. Treatment of cholestatic pruritus in children. Am J Health Syst Pharm 2007;64:1157-1162.
191. Shneider B, Emre S, Groszmann R, Karani J, McKiernan P, Sarin S, et al. Expert pediatric opinion on the Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. Pediatr Transplant 2006;10:893-907.
192. Bachrach LK, Ward LM. Clinical review: bisphosphonate use in childhood osteoporosis. J Clin Endocrinol Metab 2009;94:400-409.
193. LaRusso NF, Shneider BL, Black D, Gores GJ, James SP, Doo E, et al. Primary sclerosing cholangitis: summary of a workshop. HEPATOLOGY 2006;44:746-764.