Primary sclerosing cholangitis: A clinical review

American Journal of Gastroenterology

Volumn 93, Issue 4, 1998, Pages 515-523

  Ponsioen, Cyriel I J ^a   Tytgat, Guido N J ^a  

^a ACADEMIC MEDICAL CENTER   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

AZATHIOPRINE; COLCHICINE; IMMUNOSUPPRESSIVE AGENT; METHOTREXATE; PENICILLAMINE; PREDNISONE; URSODEOXYCHOLIC ACID;

BILE DUCT CARCINOMA; CHOLANGIOGRAPHY; CHOLANGITIS; CLINICAL FEATURE; CLINICAL TRIAL; COMMON BILE DUCT STENOSIS; DISEASE COURSE; ENDOPROSTHESIS; ENDOSCOPIC THERAPY; HEREDITY; HISTOPATHOLOGY; HUMAN; IMMUNOPATHOGENESIS; IMMUNOSUPPRESSIVE TREATMENT; INFECTION; ISCHEMIA; LIVER TRANSPLANTATION; MAGNETIC RESONANCE CHOLANGIOPANCREATOGRAPHY; PRIMARY SCLEROSING CHOLANGITIS; PRIORITY JOURNAL; REVIEW; SYMPTOMATOLOGY;

CHOLANGITIS, SCLEROSING; HUMANS;

EID: 0032056283     PISSN: 00029270     EISSN: None     Source Type: Journal    
DOI: 10.1111/j.1572-0241.1998.157_b.x     Document Type: Review

References (117)

1. 1. Porayko MK, LaRusso NF, Wiesner RH. Primary sclerosing cholangitis: A progressive disease? [Review]. Semin Liver Dis 1991; 11:18-25.
2. 2. Olsson R, Hagerstrand I, Broomé U, et al. Sampling variability of percutaneous liver biopsy in primary sclerosing cholangitis. J Clin Pathol 1995;48:933-5.
3. 3. Chapman RW, Marborgh BA, Rhodes JM, et al. Primary sclerosing cholangitis: A review of its clinical features, cholangiography, and hepatic histology. Gut 1980;21:870-7.
4. 4. Fleming KA. The hepatobiliary pathology of primary sclerosing cholangitis. Eur J Gastroenterol Hepatol 1992;4:266-71.
5. 5. Gohlke F, Lohse AW, Dienes HP, et al. Evidence for an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis. J Hepatol 1996;24:699-705.
6. 6. Rabinovitz M, Demetris AJ, Bou-Abboud CF, et al. Simultaneous occurrence of primary sclerosing cholangitis and autoimmune chronic active hepatitis in a patient with ulcerative colitis. Dig Dis Sci 1992;37:1606-11.
7. 7. 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.
8. 8. Broomé 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.
9. 9. Aadland E, Schrumpf E, Fausa O, et al. Primary sclerosing cholangitis: A long-term follow-up study. Scand J Gastroenterol 1987;22: 655-64.
10. 10. Fausa O, Schrumpf E, Elgjo K. Relationship of inflammatory bowel disease and primary sclerosing cholangitis [Review]. Semin Liver Dis 1991;11:31-9.
11. 11. Zetterquist H, Broomé U, Einarsson K, et al. HLA class II genes in primary sclerosing cholangitis and chronic inflammatory bowel disease: No HLA-DRw52a association in Swedish patients with sclerosing cholangitis. Gut 1992;33:942-6.
12. 12. Grunnet N, Rasmussen HH, Tage-Jensen U, et al. Association of primary sclerosing cholangitis with HLA-DRw52a? Tissue Antigens 1991;38:133-6.
13. 13. Lo SK, Fleming KA, Chapman RW. Prevalence of anti-neutrophil antibody in primary sclerosing cholangitis and ulcerative colitis using an alkaline phosphatase technique. Gut 1992;33:1370-5.
14. 14. Seibold F, Weber P, Klein R, et al. Clinical significance of antibodies against neutrophils in patients with inflammatory bowel disease and primary sclerosing cholangitis. Gut 1992;33:657-62.
15. 15. Bansi DS, Lo S, Chapman RW, et al. Absence of antineutrophil cytoplasmic antibodies in relatives of UK patients with primary sclerosing cholangitis and ulcerative colitis. Eur J Gastroenterol Hepatol 1996;8:111-6.
16. 16. Bansi DS, Fleming KA, Chapman RW. Importance of antineutrophil cytoplasmic antibodies in primary sclerosing cholangitis and ulcerative colitis: Prevalence, titre, and IgG subclass. Gut 1996;38:384-9.
17. 17. Stoffel MP, Csernok E, Herzberg C, et al. Anti-neutrophil cytoplasmic antibodies (ANCA) directed against bactericidal/permeability increasing protein (BPI): A new seromarker for inflammatory bowel disease and associated disorders. Clin Exp Immunol 1996;104:54-9.
18. 18. Majoie CB, Reeders JW, Sanders JB, et al. Primary sclerosing cholangitis: A modified classification of cholangiographic findings. AJR Am J Roentgenol 1991;157:495-7.
19. 19. Baron TH, Koehler RE, Rodgers WH, et al. Mast cell cholangiopathy: Another cause of sclerosing cholangitis. Gastroenterology 1995;109: 1677-81.
20. 20. Howe CD, Hill DB, Gubbins G. Polycystic liver disease mimicking sclerosing cholangitis during endoscopic retrograde cholangiopancreatography. Am J Gastroenterol 1994;89:128-9.
21. 21. Davis JJ, Heyman MB, Ferrell L, et al. Sclerosing cholangitis associated with chronic cryptosporidiosis in a child with a congenital immunodeficiency disorder. Am J Gastroenterol 1987;82:1196-1202.
22. 22. Cello JP. Acquired immunodeficiency syndrome cholangiopathy: Spectrum of disease. Am J Med 1989;86:539-46.
23. 23. Kemeney MM, Battifora H, Blayney DW, et al. Sclerosing cholangitis after continuous hepatic artery infusion of FUDR. Ann Surg 1985;202:176-81.
24. 24. Shea WJ, Demas BE, Goldberg HI, et al. Sclerosing cholangitis associated with hepatic arterial FUDR chemotherapy: Radiographic-histologic correlation. AJR 1986;146:717-21.
25. 25. Durand JM, Sahel J, Barthet M, et al. Cholangitis and paroxysmal nocturnal hemoglobinuria. Dig Dis Sci 1997;42:279-80 (letter).
26. 26. Sherlock S. Pathogenesis of sclerosing cholangitis: The role of non-immune factors. [Review]. Semin Liver Dis 1991;11:5-10.
27. 27. Ludwig J, MacCarthy RL, LaRusso NF, et al. Intrahepatic cholangiectases and large-duct obliteration in primary sclerosing cholangitis. Hepatology 1986;6:560-8.
28. 28. Ludwig J, LaRusso NF, Wiesner RH. Primary sclerosing cholangitis. Contemp Issues Surg Pathol 1986;8:193-213.
29. 29. Shepherd HA, Selby WS, Chapman RWG, et al. Ulcerative colitis and persistent liver dysfunction. Q J Med 1983;208:503-13.
30. 30. Olsson R, Danielsson A, Jarnerot G, et al. Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis. Gastroenterology 1991;100:1319-23.
31. 31. Farrant M, Williams R. Natural history and prognosis in primary sclerosing cholangitis. Eur J Gastroenterol Hepatol 1992;4:272-5.
32. 32. Farrant JM, Hayllar KM, Wilkinson ML, et al. Natural history and prognostic variables in primary sclerosing cholangitis. Gastroenterology 1991;100:1710-7.
33. 33. 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.
34. 34. Kashihara T, Sibamoto S, Fujimoro E, et al. Two cases of primary sclerosing cholangitis and review of the recent literature. Gastroenterol Endosc 1992;32:1194-1204.
35. 35. Utsunomiya T. Etiology of ulcerative colitis (in Japanese). Geka (Surgery) 1992;54:13-20.
36. 36. Pokorny CS, McCaughan GW, Gallagher ND, et al. Sclerosing cholangitis and biliary tract calculi - primary or secondary? Gut 1992;33:1376-80.
37. 37. Kaw M, Silverman WB, Rabinovitz M, et al. Biliary tract calculi in primary sclerosing cholangitis. Am J Gastroenterol 1995;90:72-5.
38. 38. Wiesner RH, Grambsch PM, Dickson ER, et al. Primary sclerosing cholangitis: Natural history, prognostic factors and survival analysis. Hepatology 1989;10:430-6.
39. 39. Dickson ER, Murtaugh PA, Wiesner RH, et al. Primary sclerosing cholangitis: Refinement and validation of survival models. Gastroenterology 1992;103:1893-901.
40. 40. MacCarty RL, LaRusso NF, Wiesner RH, et al. Primary sclerosing cholangitis: Findings on cholangiography and pancreaticography. Radiology 1983;149:39-44.
41. 41. Langnas AN, Grazi GL, Stratta RJ, et al. Primary sclerosing cholangitis: The emerging role for liver transplantation. Am J Gastroenterol 1990;85:1136-41.
42. 42. Abu-Elmagd KM, Malinchoc M, Dickson ER, et al. Efficacy of hepatic transplantation in patients with primary sclerosing cholangitis. Surg Gynecol Obstet 1993;177:335-44.
43. 43. Marsh JW, Iwatsuki S, Makowka L, et al. Orthotopic liver transplantation for primary sclerosing cholangitis. Ann Surg 1988;207:21-5.
44. 44. Rosen CB, Nagorney DM, Wiesner RH, et al. Cholangiocarcinoma complicating primary sclerosing cholangitis. Ann Surg 1991;213: 21-5.
45. 45. Nashan B, Schlitt HJ, Tusch G, et al. Biliary malignancies in primary sclerosing cholangitis: Timing for liver transplantation. Hepatology 1996;23:1105-11.
46. 46. Abu-Elmagd KM, Selby R, Iwatsuki S, et al. Cholangiocarcinoma and sclerosing cholangitis: Clinical characteristics and effect on survival after liver transplantation. Transplant Proc 1993;25:1124-5.
47. 47. Ferrari AP, Lichtenstein DR, Slivka A, et al. Brush cytology during ERCP for the diagnosis of biliary and pancreatic malignancies. Gastrointest Endosc 1994;40:140-5.
48. 48. Rupp M, Hawthorne CM, Ehya H. Brush cytology in biliary tract obstruction. Acta Cytologia 1990;34:221-6.
49. 49. Mansfield JC, Griffin SM, Wadehra V, et al. A prospective evaluation of cytology from biliary strictures. Gut 1997;40:671-7.
50. 50. Rabinovitz M, Zajko AB, 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.
51. 51. Rizzi PM, Ryder SD, Portmann B, et al. p53 Protein overexpression in cholangiocarcinoma arising in primary sclerosing cholangitis. Gut 1996;38:265-8.
52. 52. Levi S, Urbano-Ispizua A, Gill R, et al. Multiple K-ras codon 12 mutations in cholangiocarcinomas demonstrated with a sensitive polymerase chain reaction technique. Cancer Res 1991;51:3497-3502.
53. 53. Mehal WZ, Hattersley AT, Chapman RW, et al. A survey of cytomegalovirus (CMV) DNA in primary sclerosing cholangitis (PSC) liver tissues using a sensitive polymerase chain reaction (PCR) based assay. J Hepatol 1992;15:396-9.
54. 54. Morecki R, Glaser JH, Cho S, et al. Biliary atresia and reovirus type 3 infection. N Engl J Med 1982;307:481-484.
55. 55. Minuk GY, Rascanin N, Paul RW, et al. Reovirus type 3 infection in patients with primary biliary cirrhosis and primary sclerosing cholangitis. J Hepatol 1987;5:8-13.
56. 56. Boden RW, Rankin JG, Goulston SJM, et al. The liver in ulcerative colitis. Lancet 1959;2:245-8.
57. 57. Rankin JG, Boden RW, Goulston SJM, et al. The liver in ulcerative colitis. Treatment of pericholangitis with tetracycline. Lancet 1959; 2:1110-2.
58. 58. Palmer KR, Duerden BI, Holdsworth CD. Bacteriological and endotoxin studies in cases of ulcerative colitis submitted to surgery. Gut 1980;21:851-4.
59. 59. Ludwig J, Barham SS, LaRusso NF, et al. Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and ulcerative colitis. Hepatology 1981;1:632-40.
60. 60. Vinnik IE, Kern F Jr, Struthers JE Jr, et al. Experimental portal vein bacteremia. Proc Soc Exp Biol Med 1964;115:311-4.
61. 61. Mistilis SP, Skyring AP, Goulston SJ. Effect of long-term tetracycline therapy, steroid therapy and colectomy in pericholangitis associated with ulcerative colitis. Australas Ann Med 1965;14:286-94.
62. 62. Hobson CH, Butt TJ, Ferry DM, et al. Enterohepatic circulation of bacterial chemotactic peptide in rats with experimental colitis. Gastroenterology 1988;94:1006-13.
63. 63. Cangemi JR, Wiesner RH, Beaver SJ, et al. Effect of proctocolectomy for chronic ulcerative colitis on the natural history of primary sclerosing cholangitis. Gastroenterology 1989;96:790-4.
64. 64. Palmer RH. Bile acids, liver injury, and liver disease. Arch Intern Med 1972;130:606-17.
65. 65. Siegel JH, Barnes S, Morris JS. Bile acids in liver disease associated with inflammatory bowel disease. Digestion 1977;15:469-481.
66. 66. Cowen AE, Korman MG, Hofmann AF, et al. Metabolism of lithocholate in healthy man. I. Biotransformation and biliary excretion of intravenously administered lithocholate, lithocholylglycine, and their sulphates. Gastroenterology 1975;69:59-66.
67. 67. Mauad TH, Nieuwkerk CMJ, Dingemans KP, et al. Mice with homozygous dysruption of the mdr2 P-glycoprotein gene. A novel animal model for studies of nonsuppurative inflammatory cholangitis and hepatocarcinogenesis. Am J Pathol 1994;145:1237-45.
68. 68. Nieuwkerk CMJ. Pathophysiology of biliary lipid secretion. Amsterdam: University of Amsterdam Press, 1997:147.
69. 69. Ludwig J, LaRusso NF, Wiesner RH. The syndrome of primary sclerosing cholangitis. [Review]. Prog Liver Dis 1990;9:555-66.
70. 70. Record CO, Eddleston ALWF, Shilkin KB, et al. Intrahepatic sclerosing cholangitis associated with a familial immunodeficiency syndrome. Lancet 1973;ii:18-22.
71. 71. Waldram R, Kopelman H, Tsantoulas D, et al. Chronic pancreatitis, sclerosing cholangitis and sicca complex in two siblings. Lancet 1975;i:550-2.
72. 72. Quigley EMM, LaRusso NF, Ludwig J, et al. Familial occurrence of primary sclerosing cholangitis and ulcerative colitis. Gastroenterology 1983;85:1160-5.
73. 73. Jorge AD, Esley C, Ahumada J. Family incidence of primary sclerosing cholangitis associated with immunological diseases. Endoscopy 1987;19:114-7.
74. 74. Schrumpf E, Fausa O, Forre O, et al. HLA antigens and immuno-regulatory T-cells in ulcerative colitis associated with hepatobiliary disease. Scand J Gastroenterol 1982;17:187-91.
75. 75. Donaldson PT, Farrant JM, Wilkinson ML, et al. Dual association of HLA DR2 and DR3 with primary sclerosing cholangitis. Hepatology 1991;13:129-33.
76. 76. Prochazka EJ, Terasaki PI, Park MS, et al. Association of primary sclerosing cholangitis with HLA-DRw52a [see comments]. N Engl J Med 1990;322:1842-4.
77. 77. Farrant JM, Doherty DG, Donaldson PT, et al. Amino acid substitutions at position 38 of the DR beta polypeptide confer susceptibility to and protection from primary sclerosing cholangitis. Hepatology 1992;16:390-5.
78. 78. Broomé U, Hultcrantz R, Scheynius A. Lack of concomitant expression of ICAM-1 and HLA-DR on bile duct cells from patients with primary sclerosing cholangitis and primary biliary cirrhosis. Scand J Gastroenterol 1993;28:126-30.
79. 79. Whiteside TL, Lasky S, Si L, et al. Immunologic analysis of mononuclear cells in liver tissues and blood of patients with primary sclerosing cholangitis. Hepatology 1985;5:468-74.
80. 80. Van den Oord JJ, Sciot R, Desmet VJ. Expression of MHC products by normal and abnormal bile duct epithelium. J Hepatol 1986;3: 310-7.
81. 81. Broomé U, Glaumann H, Hultcrantz R, et al. Distribution of HLA-DR, HLA-DP, HLA-DQ antigens in liver tissue from patients with primary sclerosing cholangitis. Scand J Gastroenterol 1990;25:54-8.
82. 82. Chapman RW, Kelly PMA, Heryet A, et al. Expression of HLA-DR antigens on bile duct epithelium in primary sclerosing cholangitis. Gut 1988;29:422-7.
83. 83. Springer TA. Adhesion receptors of the immune system. Nature 1990;346:425-34.
84. 84. Ueno Y, Phillips JO, Ludwig J, et al. Development and characterization of a rodent model of immune-mediated cholangitis. Proc Nat Acad Sci USA 1996;93:216-220.
85. 85. Das KM, Vecchi M, Sakamaki S. A shared and unique epitope(s) on human colon, skin, and biliary epithelium detected by a monoclonal antibody. Gastroenterology 1990;98:464-9.
86. 86. Mandal A, Dasgupta A, Jeffers L, et al. Autoantibodies in sclerosing cholangitis against a shared peptide in biliary and colon epithelium. Gastroenterology 1994;106:185-92.
87. 87. Lindor KD, Wiesner RH, Colwell LJ, et al. The combination of prednisone and colchicine in patients with primary sclerosing cholangitis. Am J Gastroenterol 1991;86:57-61.
88. 88. Allison MC, Burroughs AK, Noone P, et al. Biliary lavage with corticosteroids in primary sclerosing cholangitis: A clinical, cholangiographic, and bacteriologic study. Hepatology 1986;3:118-22.
89. 89. Javett SL. Azathioprine in primary sclerosing cholangitis. Lancet 1971;i:810-1.
90. 90. Wagner A. Azathioprine treatment in primary sclerosing cholangitis. Lancet 1971;ii:663-4.
91. 91. Knox TA, Kaplan MM. Treatment of primary sclerosing cholangitis with oral methotrexate. Am J Gastroenterol 1991;86:546-52.
92. 92. Knox TA, Kaplan MM. A double-blind controlled trial of oral-pulse methotrexate therapy in the treatment of primary sclerosing cholangitis [see comments]. Gastroenterology 1994;106:494-9.
93. 93. LaRusso NF, Wiesner RH, Ludwig J, et al. Prospective trial of penicillamine in primary sclerosing cholangitis. Gastroenterology 1988;95:1036-42.
94. 94. Myers RN, Cooper TH, Padis N. Primary sclerosing cholangitis. Complete gross and histologic reversal after long-term steroid therapy. Am J Gastroenterol 1970;53:527-38.
95. 95. Burgert SC, Brown BP, Kirkpatrick RB, et al. Positive corticosteroid response in patients with primary sclerosing cholangitis. Gastroenterology 1984;86:A1037.
96. 96. Kaplan MM, Aroia S, Pincus SH. Primary sclerosing cholangitis and low-dose oral pulse methotrexate. Ann Intern Med 1987;106:231-5.
97. 97. Lindor KD, Jorgensen RA, Anderson ML, et al. Ursodeoxycholic acid and methotrexate for primary sclerosing cholangitis: A pilot study. Am J Gastroenterol 1996;91:511-5.
98. 98. van Milligen de Wit AWM. Primary sclerosing cholangitis: Aspects of pathogenesis and therapy; Dept. of Gastroenterology & Hepatology. Amsterdam: University of Amsterdam Press, 1997:176.
99. 99. Stiehl A, Walker S, Stiehl L, et al. Effect of ursodeoxycholic acid on liver and bile duct disease in primary sclerosing cholangitis. A 3-year pilot study with a placebo-controlled study period. J Hepatol 1994; 20:57-64.
100. 100. 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.
101. 101. Bansi D, Christie J, Fleming K, et al. High-dose ursodeoxycholic acid in primary sclerosing cholangitis: A randomised double-blind, placebo-controlled trial. Gastroenterology 1996;110:A1146.
102. 102. Lindor KD, the Mayo PSC/UDCA Study Group. Ursodiol for primary sclerosing cholangitis. N Engl J Med 1997;336:691-5.
103. 103. De Maria N, Colantoni A, Rosenbloom E, et al. Ursodeoxycholic acid does not improve the clinical course of primary sclerosing cholangitis over a 2-year period. Hepato-Gastroenterology 1996;43:1472-9.
104. 104. May GR, Bender CE, LaRusso NF, et al. Nonoperative dilatation of dominant strictures in primary sclerosing cholangitis. Am J Radiol 1985;145:1061-4.
105. 105. 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.
106. 106. Lee JG, Schutz SM, Leung JW, et al. Endoscopic therapy of sclerosing cholangitis. Hepatology 1995;21:661-7.
107. 107. Gaing AA, Geders JM, Cohen SA, et al. Endoscopic management of primary sclerosing cholangitis: review, and report of an open series [Review]. Am J Gastroenterol 1993;88:2000-8.
108. 108. van Milligen de Wit AWM, van Bracht J, Rauws EAJ, et al. Endoscopic stent therapy for dominant extrahepatic bile duct strictures in primary sclerosing cholangitis. Gastrointest Endosc 1996;44:293-9.
109. 109. Siegel JH, Guelrud M. Endoscopic cholangiopancreatoplasty: Hydrostatic balloon dilation in the bile duct and the pancreas. Gastrointest Endosc 1983;29:99-103.
110. 110. van Milligen de Wit AWM, Rauws EAJ, van Bracht J, et al. Lack of complications following short-term stent therapy for extrahepatic bile duct strictures in primary sclerosing cholangitis. Gastrointest Endosc 1997;46:344-7.
111. 111. LaRusso NF, Wiesner RH, Ludwig J, et al. Primary sclerosing cholangitis. N Engl J Med 1984;310:899-903.
112. 112. Way LW, Sleisenger MH. Sclerosing cholangitis. in: Sleisenger M, Fordtran JS (eds): Gastrointestinal disease, pathophysiology and management. Philadelphia: Saunders, 1989: p 1722.
113. 113. Feith MP, Klompmaker IJ, Maring JK, et al. Biliary reconstruction during liver transplantation in patients with primary sclerosing cholangitis. Transplant Proc 1997;29:560-1.
114. 114. Harrison RF, Davies MH, Neuberger JM, et al. Fibrous and obliterative cholangitis in liver allografts: Evidence of recurrent primary sclerosing cholangitis? Hepatology 1994;20:356-61.
115. 115. Campbell WL, Sheng R, Zajko AB, et al. Intrahepatic biliary strictures after liver transplantation. Radiology 1994;191:735-40.
116. 116. Letourneau JG, Day DL, Hunter DW, et al. Biliary complications after liver transplantation in patients with preexisting sclerosing cholangitis. Radiology 1988;167:349-51.
117. 117. Sheng R, Campbell WL, Zajko AB, et al. Cholangiographic features of biliary strictures after liver transplantation for primary sclerosing cholangitis: evidence of recurrent disease. AJR Am J Roentgenol 1996;166:1109-13.