Complement Components in Rheumatic Diseases

  • Peter H. Schur


The complement system consists of a group of at least 18 serum proteins that interact sequentially to affect systems of inflammation (Fearon, 1981; Frank and Atkinson, 1975; Muller-Eberhard, 1974; Schur, 1977). Activation of complement is accompanied by a series of specific and limited enzymatic reactions that result in the cleavage of certain components, which subsequently generate new enzymatic activities and lead to the formation of complement complexes that have additional biological activities. Some of these activities are carried out on cells or immune complex surfaces and some in fluid; in some cases, other proteins inhibit these activities, at least in part.


Systemic Lupus Erythematosus Immune Complex Systemic Lupus Erythematosus Patient Lupus Nephritis Complement Component 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Alper, C. A. and Rosen, F. L., 1967, Studies of the in vivo behavior of human C3 in normal subjects and patients, J. Clin. Invest. 46:2021–2034.PubMedCrossRefGoogle Scholar
  2. Alper, C. A., and Rosen, F. S., 1976, Genetics of the complement system, Adv. Hum. Genet. 7:141–188.PubMedGoogle Scholar
  3. Appel, A. E., Sablay, L. B., Golden, R. A., Barlond, P., Grazel, A. I., Bank, N., 1978, The effect of normalization of serum complement and anti-DNA antibody on the course of lupus erythematous, Am. J. Med. 64:274 Massachusetts 283PubMedCrossRefGoogle Scholar
  4. Arroyaue, C. M., Taylor, D. G., Gallup, P., 1978, Screening test for complement activation by counterim-munoelectrophoresis, Am. J. Clin. Pathol. 69:440.Google Scholar
  5. Atkinson, J. P., Gorman, J. C., Curd, J., Hyla, J. F., Deegan, M. J., Keren, D. F., Abdou, N. I., and Walker, S. E., 1981, Cold dependent activation of complement in discrepancy between clinical and laboratory parameters, Arthritis Rheum. 24:592–601.PubMedCrossRefGoogle Scholar
  6. Blomgren, S. E., Condemi, J. J., Bignal, M. C., and Vaughan, J. H., 1969, Antinuclear antibody induced by procainamide. A prospective study, N. Engl. J. Med. 281:64–66.PubMedCrossRefGoogle Scholar
  7. Caldwell, J. R., Ruddy, S., Schur, P. H., 1972, Acquired CI inhibitor deficiency in lymphosarcoma, Clin. Immunol. Immunopathol. 1:39.CrossRefGoogle Scholar
  8. Cameron, J. S., Lessof, M. H., Ogg, C. S., Williams, B. D., and Williams, D. G., 1976, Disease activity in the nephritis of systemic lupus erythematosus in relation to serum complement concentrations, Clin. Exp. Immunol. 25:418–427.PubMedGoogle Scholar
  9. Cochrane, C. G., and Koffler, D., 1973, Immune complex disease in experimental animals and man, Adv. Immunol. 16:186–261.Google Scholar
  10. Cohen, R. D., Conn, D. L., and Ilstrup, D. M., 1980, Clinical features, prognosis, and responses to treatment in polyarteritis, Mayo Clin. Proc. 55:146–155.PubMedGoogle Scholar
  11. Cornocoff, J. B., Herbert, L. H., Smead, W. L., VanAman, M. E., Birmingham, D. J., and Waxman, F. J., 1983, Primate erythrocyte-immune complex-clearing mechanism, J. Clin. Invest. 71:236–247.CrossRefGoogle Scholar
  12. Dawkins, R. L., Christiansen, F. T., Kay, P. H., Garlepp, M., McCluskey, J., Hollingsworth, P. N., and Silko, P. J., 1983, Disease associations with complotypes, supratypes and haplotypes, Immunol. Rev. 70:5–22.CrossRefGoogle Scholar
  13. Fearon, D. T., 1981, Complement as a mediator of inflammation, Clin. Immunol. Allergy 1:225–242.Google Scholar
  14. Fearon, D. T., and Wong, W. W., 1983, Complement ligand-receptor interactions that mediate biological responses, Ann. Rev. Imuno.. 1:243–271.CrossRefGoogle Scholar
  15. Franco, A. E., and Schur, P. H., 1971, Hypocomplementemia in rheumatoid arthritis, Arthritis Rheum. 14:231–238.PubMedCrossRefGoogle Scholar
  16. Frank, M. M., and Atkinson, J. P., 1975, Complement in clinical medicine, DM 1975 (Jan.):1–154.Google Scholar
  17. George, D., and Glass, D., 1983, Quantitation of complement proteins in rheumatic disease, Clin. Rheum. Dis. 9:177–198.PubMedGoogle Scholar
  18. Gilliam, J. N., Cheatom, D. E., Hurd, E. R., Stastny, P., and Ziff, M., 1974, Immunoglobulin in clinically uninvolved skin in systemic lupus erythematosus, J. Clin. Invest. 53:1434–1440.PubMedCrossRefGoogle Scholar
  19. Glass, D., Fearon, D. T., and Austen, K. F., 1983, Inherited abnormalities of the complement system, in:Metabolic Basis of Inherited Disease, 5th ed. ( J. B. Stanbury and J. B. Wyngaarden, eds.), McGraw- Hill, New York, pp. 1934–1955.Google Scholar
  20. Gough, W., Lightfoot, R. W., and Christian, C. L., 1974, Cryoglobulins and complement in immune complex disease, Arthritis Rheum. 17:497.Google Scholar
  21. Hadler, N. M., Gerwin, R. D., Frank, M. M., Whitaker, J. N., Baker, M., and Decker, J. L., 1973, The fourth component of complement in the cerebrospinal fluid in systemic lupus erythematosus, Arthritis Rheum. 16:507–521.PubMedCrossRefGoogle Scholar
  22. Hecht, B., Siegel, N., Adler, M., Kashgarian, M., and Hayslett, J. P., 1976, Prognostic indices in lupus erythematosus, Medicine 55:163–164.PubMedCrossRefGoogle Scholar
  23. Hobart, M. J., and Lachman, P. J., 1976, Allotypes of complement components in man, Transplant. Rev. 32:26–42.PubMedGoogle Scholar
  24. Hunder, G. G., McDuffie, F. C., and Hepper, N. G., 1972, Pleural fluid complement in systemic lupus erythematosus and rheumatoid arthritis, Ann. Intern. Med. 76:357–363.PubMedGoogle Scholar
  25. Hunder, G. G., Mullen, B. J., and McDuffie, F. C., 1974, Complement in pericardial fluid of lupus erythematosus, Ann. Intern. Med. 80:453–548.PubMedGoogle Scholar
  26. Jarrett, M. P., Sablay, L. B., Walter, L., Barland, P., and Grayzel, A. L., 1981, The effect of continuous normalization of serum hemolytic complement on the course of lupus nephritis. A five year prospect study, Am. J. Med. 70:1067–1072.PubMedCrossRefGoogle Scholar
  27. Koffler, D., and Bieseker, G., 1983, Immunopathogenesis of tissue injury, in:The Clinical Management of Systemic Lupus Erythematosus ( P. H. Schur, ed.), Grune & Stratton, New York, p. 29–47.Google Scholar
  28. Lange, K., Ores, R., Shauss, W., and Wachstein, M., 1965, Steroid therapy of systemic lupus erythematosus based on immunological considerations, Arthritis Rheum. 8:244–258.PubMedCrossRefGoogle Scholar
  29. Levinsky, R. I., Cameron, J. S., and Soothill, J. F., 1977, Serum immune complexes and disease activity in lupus nephritis, Lancet 1:564–567.PubMedCrossRefGoogle Scholar
  30. Lewis, E. J., Carpenter, C. B., and Schur, P. H., 1971, Serum complement components levels in human glomerulonephritis, Ann. Intern. Med. 75:555–560.PubMedGoogle Scholar
  31. Lightfoot, R. W., and Hughes, G. R., 1976, Significance of persisting serologic abnormalities in SLE, Arthritis Rheum. 19:837–843.PubMedCrossRefGoogle Scholar
  32. Lloyd, W., and Schur, P. H., 1981, Immune complexes, complement and anti-DNA in exacerbations of systemic lupus erythematosus (SLE), Medicine 60:208 - 217.PubMedCrossRefGoogle Scholar
  33. Miniter, M. F., Stollar, B. D, and Agnello, V., 1979, Reassessment of the clinical significance of native DNA antibodies in systemic lupus erythematosus, Arthritis Rheum. 22:959–968.PubMedCrossRefGoogle Scholar
  34. Muller-Eberhard, H. J., 1974, The complement system and nephritis, Adv. Nephrol. 4:3–14.Google Scholar
  35. Nydegger, U. E., Lambert, P. H., Gerber, H., and Miescher, P. A., 1974, Circulating immune complexes in the serum in systemic lupus erythematosus and in carriers of hepatitis B antigen, J. Clin. Invest. 54:297 - 309.PubMedCrossRefGoogle Scholar
  36. O’Neill, G. J., Yang, S. Y., Tegoli, J., Berger, R., and Dupont, B., 1978, Chido and Rodgers blood groups are distinct antigenic components of human complement C4, Nature 273:668–670.PubMedCrossRefGoogle Scholar
  37. Pekin, T. J., Jr., and Zvaifler, N. J., 1970, Synovial fluid findings in systemic lupus erythematosus, Arthritis Rheum. 13:777–785.PubMedCrossRefGoogle Scholar
  38. Petz, L. D., Sharp, G. C., Cooper, N. R., and Irvin, W. S., 1971, Serum and cerebral spinal fluid complement and serum auto-antibodies in systemic lupus erythematosus, Medicine 50:259–275.PubMedCrossRefGoogle Scholar
  39. Provost, I. I., and Tomasi, T. B., Jr., 1973, Evidence for complement activation via the alternate pathway in skin diseases, herpes gestationis, systemic lupus erythematosus, and bullous pemphigoid, J. Clin. Invest. 52:1779–1787.PubMedCrossRefGoogle Scholar
  40. Pussel, B. A., Lockwood, C. M., Scott, D. M., Pinching, A. J., and Peters, D. K., 1978, Value of immune complex assays in diagnosis and management, Lancet 2:359–364.CrossRefGoogle Scholar
  41. Rosen, F. S., and Austen, K. F., 1969, The “neurotic edema” (hereditary angioedema), N. Engl. J. Med. 280:1356–1357.PubMedCrossRefGoogle Scholar
  42. Rothfield, N., Ross, A., Minta, J. O., and Lepow, I. H., 1972, Glomerular and dermal deposition of properdin in systemic lupus erythematosus, N. Engl. J. Med. 287:681–685.PubMedCrossRefGoogle Scholar
  43. Ruddy, S., and Austen, K. F., 1970, The complement system in rheumatoid synovitis. I. An analysis of complement component activities in rheumatoid synovial fluids, Arthritis Rheum. 13:713 - 725.PubMedCrossRefGoogle Scholar
  44. Ruddy, S., Carpenter, C. B., Chin, K. W., Krostmann, J. N., Sorter, N. A., Gotze, O., Muller-Eberhad, H. J., and Austen, K. F., 1975, Human complement metabolism: An analysis of 144 studies, Medicine 54:165–178.Google Scholar
  45. Schur, P. H., 1972, Human gamma G subclasses, Prog. Clin. Immunol. 1:71–104.PubMedGoogle Scholar
  46. Schur, P. H., 1975, Complement in lupus, Clin. Rheum. Dis. 1:519–543.Google Scholar
  47. Schur, P. H., 1977, Complement testing in the diagnosis of immune and autoimmune diseases, Am. J. Clin. Pathol. 68:647–659.PubMedGoogle Scholar
  48. Schur, P. H., 1983, Complement studies of sera and other biologic fluids, Hum. Pathol. 14:338–342.PubMedCrossRefGoogle Scholar
  49. Schur, P. H., and Austen, K. F., 1968, Complement in human disease, Annu. Rev. Med. 10:1–34.CrossRefGoogle Scholar
  50. Silverstein, A. M., 1960, Essential hypocomplementemia:Report of a case, Blood 16:1338–1341.PubMedGoogle Scholar
  51. Sliwinski, A. J., and Zvaifler, N. J., 1972, Decreased synthesis of the third component in hypocomplementemic systemic lupus erythematosus, Clin. Exp. Immunol. 11:21–29.PubMedGoogle Scholar
  52. Soter, N. A., 1976, Clinical presentations and mechanisms of necrotizing angiitis, J. Invest. Dermatol. 67:354–359.PubMedCrossRefGoogle Scholar
  53. Stastny, P., and Ziff, M., 1969, Cold-insoluble complexes and complement levels in systemic lupus erythematosus, N. Engl. J. Med. 280:1376–1381.PubMedCrossRefGoogle Scholar
  54. Tan, E. M., and Kunkel, H. G., 1966, An immunofluorescent study of the skin lesions in systemic lupus erythematosus, Arthritis Rheum. 9:37–46.PubMedCrossRefGoogle Scholar
  55. Vaughan, J. H., Bayles, T. B., and Savour, C. B., 1951, The response of serum gammaglobulin level and complement titer to adrenocorticotrophic hormone therapy in lupus erythematosus disseminatus, J. Lab. Clin. Med. 37:698–702.PubMedGoogle Scholar
  56. Verroust, P. J., Wilson, C. B., Cooper, N. R., Edginton, T. S., and Dixon, F. J., 1974, Glomerular complement components in human glomerulonephritis, J. Clin. Invest. 53:77–84.PubMedCrossRefGoogle Scholar
  57. Wilson, J. G., Wong, W. W., Schur, P. H., and Fearon, D. T., 1982, Mode of inheritance of decreased C3b receptors on erythrocytes of patients with systemic lupus erythematosus, N. Engl. J. Med. 307:981–986.PubMedCrossRefGoogle Scholar

Copyright information

© Plenum Publishing Corporation 1985

Authors and Affiliations

  • Peter H. Schur
    • 1
  1. 1.Division of Rheumatology and ImmunologyBrigham and Women’s Hospital, Harvard Medical SchoolBostonUSA

Personalised recommendations