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. 1982 Mar;47(3):548–554.

Complement activation in chronic liver disease.

L E Munoz, D De Villiers, D Markham, K Whaley, H C Thomas
PMCID: PMC1536431  PMID: 7083631

Abstract

Patients with HBsAg positive chronic active liver disease (CALD) and primary biliary cirrhosis (PBC) exhibit increased C3d concentrations and changes in the serum concentrations of the complement components consistent with activation of the classical and alternative pathways. In these patients the concentrations of the regulatory proteins, C3b inactivator (C3bINA) and beta IH globulin, are normal. Patients with HBsAg negative CALD and alcohol induced liver disease (ALD) exhibit no evidence of an increased level of complement system activation. In these patients diminished serum concentrations of complement components appear to be related to diminished hepatic synthetic function. C4 synthesis may be specifically reduced in autoimmune chronic active liver disease.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Epstein O., De Villiers D., Jain S., Potter B. J., Thomas H. C., Sherlock S. Reduction of immune complexes and immunoglobulins induced by D-penicillamine in primary biliary cirrhosis. N Engl J Med. 1979 Feb 8;300(6):274–278. doi: 10.1056/NEJM197902083000602. [DOI] [PubMed] [Google Scholar]
  2. Fearon D. T., Austen K. F., Ruddy S. Properdin factor D. II. Activation to D by properdin. J Exp Med. 1974 Aug 1;140(2):426–436. doi: 10.1084/jem.140.2.426. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Jaffe C. J., Vierling J. M., Jones E. A., Lawley T. J., Frank M. M. Receptor specific clearance by the reticuloendothelial system in chronic liver diseases. Demonstration of defective C3b-specific clearance in primary biliary cirrhosis. J Clin Invest. 1978 Nov;62(5):1069–1077. doi: 10.1172/JCI109212. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Lachmann P. J., Müller-Eberhard H. J. The demonstration in human serum of "conglutinogen-activating factor" and its effect on the third component of complement. J Immunol. 1968 Apr;100(4):691–698. [PubMed] [Google Scholar]
  5. Mancini G., Carbonara A. O., Heremans J. F. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235–254. doi: 10.1016/0019-2791(65)90004-2. [DOI] [PubMed] [Google Scholar]
  6. Pangburn M. K., Schreiber R. D., Müller-Eberhard H. J. Human complement C3b inactivator: isolation, characterization, and demonstration of an absolute requirement for the serum protein beta1H for cleavage of C3b and C4b in solution. J Exp Med. 1977 Jul 1;146(1):257–270. doi: 10.1084/jem.146.1.257. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Perrin L. H., Lambert P. H., Miescher P. A. Complement breakdown products in plasma from patients with systemic lupus erythematosus and patients with membranoproliferative or other glomerulonephritis. J Clin Invest. 1975 Jul;56(1):165–176. doi: 10.1172/JCI108065. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Potter B. J., Elias E., Jones E. A. Hypercatabolism of the third component of complement in patients with primary biliary cirrhosis. J Lab Clin Med. 1976 Sep;88(3):427–439. [PubMed] [Google Scholar]
  9. Potter B. J., Elias E., Thomas H. C., Sherlock S. Complement metabolism in chronic liver disease: catabolism of C1q in chronic active liver disease and primary biliary cirrhosis. Gastroenterology. 1980 May;78(5 Pt 1):1034–1040. [PubMed] [Google Scholar]
  10. Ruddy S., Austen K. F. C3 inactivator of man. I. Hemolytic measurement by the inactivation of cell-bound C3. J Immunol. 1969 Mar;102(3):533–543. [PubMed] [Google Scholar]
  11. Sachar D. B. Metronidazole for Crohn's disease--breakthrough or ballyhoo? Gastroenterology. 1980 Aug;79(2):393–395. [PubMed] [Google Scholar]
  12. Teisberg P., Gjone E. Circulating conversion products of C3 in liver disease. Evidence for in vivo activation of the complement system. Clin Exp Immunol. 1973 Aug;14(4):509–514. [PMC free article] [PubMed] [Google Scholar]
  13. Thomas H. C., De Villiers D., Potter B., Hodgson H., Jain S., Jewell D. P., Sherlock S. Immune complexes in acute and chronic liver disease. Clin Exp Immunol. 1978 Feb;31(2):150–157. [PMC free article] [PubMed] [Google Scholar]
  14. Thomas H. C., Potter B. J., Elias E., Sherlock S. Metabolism of the third component of complement in acute type B hepatitis, HBs antigen positive glomerulonephritis, polyarteritis nodosum, and HBs antigen positive and negative chronic active liver disease. Gastroenterology. 1979 Apr;76(4):673–679. [PubMed] [Google Scholar]
  15. Thomas H. C., Potter B. J., Sherlock S. Is primary biliary cirrhosis an immune complex disease? Lancet. 1977 Dec 17;2(8051):1261–1263. doi: 10.1016/s0140-6736(77)92666-6. [DOI] [PubMed] [Google Scholar]
  16. Thompson R. A., Lachmann P. J. A second case of human C3b inhibitor (KAF) deficiency. Clin Exp Immunol. 1977 Jan;27(1):23–29. [PMC free article] [PubMed] [Google Scholar]
  17. Wands J. R., Dienstag J. L., Bhan A. K., Feller E. R., Isselbacher K. J. Circulating immune complexes and complement activation in primary biliary cirrhosis. N Engl J Med. 1978 Feb 2;298(5):233–237. doi: 10.1056/NEJM197802022980502. [DOI] [PubMed] [Google Scholar]
  18. Weiler J. M., Daha M. R., Austen K. F., Fearon D. T. Control of the amplification convertase of complement by the plasma protein beta1H. Proc Natl Acad Sci U S A. 1976 Sep;73(9):3268–3272. doi: 10.1073/pnas.73.9.3268. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Whaley K., Ruddy S. Modulation of C3b hemolytic activity by a plasma protein distinct from C3b inactivator. Science. 1976 Sep 10;193(4257):1011–1013. doi: 10.1126/science.948757. [DOI] [PubMed] [Google Scholar]
  20. Whaley K., Ruddy S. Modulation of the alternative complement pathways by beta 1 H globulin. J Exp Med. 1976 Nov 2;144(5):1147–1163. doi: 10.1084/jem.144.5.1147. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Whaley K., Schur P. H., Ruddy S. C3b inactivator in the rheumatic diseases. Measurement by radial immunodiffusion and by inhibition of formation of properdin pathway C3 convertase. J Clin Invest. 1976 Jun;57(6):1554–1563. doi: 10.1172/JCI108426. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Whaley K., Thompson R. A. Requirements for beta1H globulin and C3b inactivator in the control of the alternative complement pathway in human serum. Immunology. 1978 Dec;35(6):1045–1049. [PMC free article] [PubMed] [Google Scholar]
  23. Zubler R. H., Lange G., Lambert P. H., Miescher P. A. Detection of immune complexes in unheated sera by modified 125I-Clq binding test. Effect of heating on the binding of Clq by immune complexes and application of the test to systemic lupus erythematosus. J Immunol. 1976 Jan;116(1):232–235. [PubMed] [Google Scholar]

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