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. 1985 Aug;143(2):193–195.

Primary Sclerosing Cholangitis—An Immunologically Mediated Disease?

R W G Chapman 1, D P Jewell 1
PMCID: PMC1306270  PMID: 4036118

Abstract

Primary sclerosing cholangitis (PSC) is an uncommon, chronic disorder characterized by inflammatory fibrosis usually involving the entire biliary tree. The etiology has been unknown, but PSC is closely associated with ulcerative colitis, which coexists in more than two thirds of patients with PSC. In recent studies 3% to 5% of all patients with ulcerative colitis had PSC.

We propose that PSC is, at least in part, an immunologically mediated disease; it is closely associated with human leckocyte antigens B8 and DR3, and circulating autoantibodies to colon and portal tract are frequently present. The anticolon antibody cross-reacts with enteric Escherichia coli. The disease may possibly be triggered in susceptible patients with ulcerative colitis by immunization with antigens shared between enteric microorganisms and the biliary system.

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Selected References

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  1. Asquith P., Mackintosh P., Stokes P. L., Holmes G. K., Cooke W. T. Histocompatibility antigens in patients with inflammatory-bowel disease. Lancet. 1974 Jan 26;1(7848):113–115. doi: 10.1016/s0140-6736(74)92342-3. [DOI] [PubMed] [Google Scholar]
  2. BROBERGER O., PERLMANN P. Demonstration of an epithelial antigen in colon by means of fluorescent antibodies from children with ulcerative colitis. J Exp Med. 1962 Jan 1;115:13–26. doi: 10.1084/jem.115.1.13. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. BROBERGER O., PERLMANN P. In vitro studies of ulcerative colitis. I. Reactions of patients' serum with human fetal colon cells in tissue cultures. J Exp Med. 1963 May 1;117:705–716. doi: 10.1084/jem.117.5.705. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Bangaru B., Morecki R., Glaser J. H., Gartner L. M., Horwitz M. S. Comparative studies of biliary atresia in the human newborn and reovirus-induced cholangitis in weanling mice. Lab Invest. 1980 Nov;43(5):456–462. [PubMed] [Google Scholar]
  5. Bodenheimer H. C., Jr, LaRusso N. F., Thayer W. R., Jr, Charland C., Staples P. J., Ludwig J. Elevated circulating immune complexes in primary sclerosing cholangitis. Hepatology. 1983 Mar-Apr;3(2):150–154. doi: 10.1002/hep.1840030203. [DOI] [PubMed] [Google Scholar]
  6. CAREY J. B. BILE ACIDS, CIRRHOSIS, AND HUMAN EVOLUTION. Gastroenterology. 1964 Apr;46:490–493. [PubMed] [Google Scholar]
  7. Chapman R. W., Arborgh B. A., Rhodes J. M., Summerfield J. A., Dick R., Scheuer P. J., Sherlock S. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut. 1980 Oct;21(10):870–877. doi: 10.1136/gut.21.10.870. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Danzi J. T., Makipour H., Farmer R. G. Primary sclerosing cholangitis. A report of nine cases and clinical review. Am J Gastroenterol. 1976 Feb;65(2):109–116. [PubMed] [Google Scholar]
  9. De Aizpurua H. J., Cosgrove L. J., Ungar B., Toh B. H. Autoantibodies cytotoxic to gastric parietal cells in serum of patients with pernicious anemia. N Engl J Med. 1983 Sep 15;309(11):625–629. doi: 10.1056/NEJM198309153091102. [DOI] [PubMed] [Google Scholar]
  10. Dew M. J., van Berge Henegouwen G. P., Huybregts A. W., Allan R. N. Hepatotoxic effect of bile acids in inflammatory bowel disease. Gastroenterology. 1980 Jun;78(6):1393–1401. [PubMed] [Google Scholar]
  11. Dobersen M. J., Scharff J. E., Ginsberg-Fellner F., Notkins A. L. Cytotoxic autoantibodies to beta cells in the serum of patients with insulin-dependent diabetes mellitus. N Engl J Med. 1980 Dec 25;303(26):1493–1498. doi: 10.1056/NEJM198012253032601. [DOI] [PubMed] [Google Scholar]
  12. Ebringer R. W., Cawdell D. R., Cowling P., Ebringer A. Sequential studies in ankylosing spondylitis. Association of Klebsiella pneumoniae with active disease. Ann Rheum Dis. 1978 Apr;37(2):146–151. doi: 10.1136/ard.37.2.146. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Ebringer R. W. HLA-B27 and the link with rheumatic diseases: recent developments. Clin Sci (Lond) 1980 Dec;59(6):405–410. doi: 10.1042/cs0590405. [DOI] [PubMed] [Google Scholar]
  14. Eddleston A. L., Williams R. HLA and liver disease. Br Med Bull. 1978 Sep;34(3):295–300. doi: 10.1093/oxfordjournals.bmb.a071515. [DOI] [PubMed] [Google Scholar]
  15. Holzbach R. T., Marsh M. E., Freedman M. R., Fazio V. W., Lavery I., Jagelman D. A. Portal vein bile acids in patients with severe inflammatory bowel disease. Gut. 1980 May;21(5):428–435. doi: 10.1136/gut.21.5.428. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. James S. P., Hoofnagle J. H., Strober W., Jones E. A. NIH conference: Primary biliary cirrhosis: a model autoimmune disease. Ann Intern Med. 1983 Oct;99(4):500–512. doi: 10.7326/0003-4819-99-4-500. [DOI] [PubMed] [Google Scholar]
  17. KUNIN C. M. SEPARATION, CHARACTERIZATION, AND BIOLOGICAL SIGNIFICANCE OF A COMMON ANTIGEN IN ENTEROBACTERIACEAE. J Exp Med. 1963 Oct 1;118:565–586. doi: 10.1084/jem.118.4.565. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Lawley T. J., Hall R. P., Fauci A. S., Katz S. I., Hamburger M. I., Frank M. M. Defective Fc-receptor functions associated with the HLA-B8/DRw3 haplotype: studies in patients with dermatitis herpetiformis and normal subjects. N Engl J Med. 1981 Jan 22;304(4):185–192. doi: 10.1056/NEJM198101223040401. [DOI] [PubMed] [Google Scholar]
  19. Ludwig J., Barham S. S., LaRusso N. F., Elveback L. R., Wiesner R. H., McCall J. T. Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hepatology. 1981 Nov-Dec;1(6):632–640. doi: 10.1002/hep.1840010612. [DOI] [PubMed] [Google Scholar]
  20. McFarlane I. G., Wojcicka B. M., Tsantoulas D. C., Portmann B. C., Eddleston A. L., Williams R. Leukocyte migration inhibition in response to biliary antigens in primary biliary cirrhosis, sclerosing cholangitis, and other chronic liver diseases. Gastroenterology. 1979 Jun;76(6):1333–1340. [PubMed] [Google Scholar]
  21. Nagai T., Das K. M. Demonstration of an assay for specific cytolytic antibody in sera from patients with ulcerative colitis. Gastroenterology. 1981 Jun;80(6):1507–1512. [PubMed] [Google Scholar]
  22. Perlmann P., Hammarström S., Lagercrantz R., Gustafsson B. E. Antigen from colon of germfree rats and antibodies in human ulcerative colitis. Ann N Y Acad Sci. 1965 Jun 30;124(1):377–394. doi: 10.1111/j.1749-6632.1965.tb18972.x. [DOI] [PubMed] [Google Scholar]
  23. Quigley E. M., LaRusso N. F., Ludwig J., MacSween R. N., Birnie G. G., Watkinson G. Familial occurrence of primary sclerosing cholangitis and ulcerative colitis. Gastroenterology. 1983 Nov;85(5):1160–1165. [PubMed] [Google Scholar]
  24. Schrumpf E., Elgjo K., Fausa O., Gjone E., Kolmannskog F., Ritland S. Sclerosing cholangitis in ulcerative colitis. Scand J Gastroenterol. 1980;15(6):689–697. doi: 10.3109/00365528009181516. [DOI] [PubMed] [Google Scholar]
  25. Schrumpf E., Fausa O., Førre O., Dobloug J. H., Ritland S., Thorsby E. HLA antigens and immunoregulatory T cells in ulcerative colitis associated with hepatobiliary disease. Scand J Gastroenterol. 1982 Mar;17(2):187–191. doi: 10.3109/00365528209182038. [DOI] [PubMed] [Google Scholar]
  26. Shepherd H. A., Selby W. S., Chapman R. W., Nolan D., Barbatis C., McGee J. O., Jewell D. P. Ulcerative colitis and persistent liver dysfunction. Q J Med. 1983 Autumn;52(208):503–513. [PubMed] [Google Scholar]
  27. Siegel J. H., Barnes S., Morris J. S. Bile acids in liver disease associated with inflammatory bowel disease. Digestion. 1977;15(6):469–481. doi: 10.1159/000198038. [DOI] [PubMed] [Google Scholar]
  28. Thorpe M. E., Scheuer P. J., Sherlock S. Primary sclerosing cholangitis, the biliary tree, and ulcerative colitis. Gut. 1967 Oct;8(5):435–448. doi: 10.1136/gut.8.5.435. [DOI] [PMC free article] [PubMed] [Google Scholar]
  29. Warren K. W., Athanassiades S., Monge J. I. Primary sclerosing cholangitis. A study of forty-two cases. Am J Surg. 1966 Jan;111(1):23–38. doi: 10.1016/0002-9610(66)90339-4. [DOI] [PubMed] [Google Scholar]
  30. Wiesner R. H., LaRusso N. F. Clinicopathologic features of the syndrome of primary sclerosing cholangitis. Gastroenterology. 1980 Aug;79(2):200–206. [PubMed] [Google Scholar]

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