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Immunology of the Connective Tissue Diseases pp 119–145Cite as

The Complement System and Connective Tissue Disease

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Part of the book series: Immunology and Medicine Series ((IMME,volume 22))

Abstract

Complement comprises a complex system of proteins incorporating a triggered enzyme cascade, regulatory proteins and complement receptors. The activities of complement include: promotion of the inflammatory response; opsonization of pathogens; opsonization and clearance of immune complexes; target cell lysis; and the development of antibody responses. These diverse activities, bridging the inflammatory and adaptive immune systems, are compatible with an important role for the complement system in the pathogenesis of the connective tissue diseases. At the heart of these disorders is immunologically generated inflammation, and complement participates both in the induction of disease and in the expression of tissue injury. Thus complement can operate within the disease process at both the inducer and the effector stages.

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References

  1. Law SK, Levine RP. Interaction between the third complement protein and cell-surface macromolecules. Proc Natl Acad Sci USA. 1977; 74: 2701–2705.

    PubMed  CAS  Google Scholar 

  2. Panburn MK, Muller-Eberhard HJ. The C3 convertase of the alternative pathway of human complement. Enzymic properties of the bimolecular proteinase. Biochem J. 1986; 235: 723–730.

    Google Scholar 

  3. Burton DR, Boyd J, Brampton AD, Easterbrook-Smith ED, Emanuel EJ, Novotny J, Rademacher TW, van Schravendij MR, Sterberg MJ, Dwek RA. The Clq receptor site on immunoglobulin G. Nature. 1980; 288: 338–344.

    PubMed  CAS  Google Scholar 

  4. Schumaker VN, Zavodsky P, Poon PH. Activation of the first component of complement. Ann Rev Immunol. 1987; 5: 21–42.

    CAS  Google Scholar 

  5. Law SK, Fearon DT, Levine RP. Action of the C3b-inactivator on cell bound C3b. J Immunol. 1979; 122: 7262–7265.

    Google Scholar 

  6. Muller-Eberhard HJ. Molecular organisation and function of the complement system. Ann Rev Biochem. 1988; 57: 321–347.

    CAS  Google Scholar 

  7. Lublin DM, Atkinson JP. Decay-acceleration factor: Biochemistry, molecular biology and function. Ann Rev Immunol. 1979; 7: 35–58.

    Google Scholar 

  8. Liszewski MK, Post TW, Atkinson JP. Membrane cofactor protein (MCP or CD46): newest member of the regulators of complement activation gene cluster. Ann Rev Immunol. 1991; 9: 431–455.

    CAS  Google Scholar 

  9. Ahearn JM, Fearon DT. Structure and function of the complement receptors, CR1 (CD35) and CR2 (CD21). Adv Immunol. 1989; 46: 183–219.

    PubMed  CAS  Google Scholar 

  10. Nagaki K, Iida K, Inai S. The inactivator of the first component of human complement (C1INA). The complex formation with the activated first component of human complement (C1) or with its subcomponents. Int Arch Allergy Appl Immunol. 1974; 46: 935–948.

    PubMed  CAS  Google Scholar 

  11. Podack ER, Muller-Eberhard HJ. Isolation of human S-protein, an inhibitor of the membrane attack complex of complement. J Biol Biochem. 1979; 254: 9908–9914.

    Google Scholar 

  12. Davies A, Simmons DL, Hale G, Harrison RA, Tighe H, Lachmann PJ, Waldmann H. CD 262, an Ly-6-like protein expressed in human lymphoid cells, regulates the action of the complement membrane attack complex on homologous cells. J Exp Med. 1989; 170: 637–654.

    PubMed  CAS  Google Scholar 

  13. Schonermark S, Rauterberg EW, Shin ML, Loke S, Roelke D, Hansch GM. Homologous species restriction in lysis of human erythrocytes: a membrane derived protein with C8-binding capacity functions as an inhibitor. J Immunol. 1986; 136: 1772–1776.

    PubMed  CAS  Google Scholar 

  14. Halperin JA, Nicholson-Weller A. Paroxysmal nocturnal haemoglobinuria. A complement-mediated disease. Complement Inflamm. 1989; 6: 65–72.

    PubMed  CAS  Google Scholar 

  15. Reid KBM, Bentley DR, Campbell RD, Chung LP, Sim RB, Kristensen T, Tack BF. Complement system proteins which interact with C3b or C4b. Immunol Today. 1986; 7: 230–233.

    CAS  Google Scholar 

  16. Fearon DT. Cellular receptors for fragments of the third component of complement. Immunol Today. 1984; 5: 105–110.

    CAS  Google Scholar 

  17. Wong WW, Cahill JM, Rosen MD, Kennedy CA, Bonaccio ET, Morris MJ, Wilson JG, Klickstein LB, Fearon DT. Structure of the human CR1 gene. Molecular basis of the structural and quantitative polymorphisms and identification of a new CR1-like allele. J Exp Med. 1989; 169: 847–863.

    PubMed  CAS  Google Scholar 

  18. Fingeroth JD, Weiss JJ, Tedder TF, Strominger JL, Biro PA, Fearon DT. Epstein-Barr virus receptor on human B lymphocytes is the C3d receptor, CR2. Proc Natl Acad Sci (USA). 1984; 81: 4510–4514.

    CAS  Google Scholar 

  19. Heyman B, Wiersma EJ, Kinoshita T. In vivo inhibition of the antibody response by a complement receptor specific monoclonal antibody. J Exp Med. 1990; 172: 665–668.

    PubMed  CAS  Google Scholar 

  20. Papamichail M, Gutierrez C, Embling P, Johnson P, Holborow EJ, Pepys MB. Complement dependence of localisation of aggregated IgG in germinal centres. Scand J Immunol. 1975; 4: 343–347.

    PubMed  CAS  Google Scholar 

  21. Klaus GGB, Humphrey JH. The generation of memory cells. The role of C3 in the generation of B memory cells. Immunology. 1977; 33: 31–40.

    PubMed  CAS  Google Scholar 

  22. Ross GD, Medof ME. Membrane complement receptors specific for bound fragments of C3. Adv Immunol. 1985; 37: 217–267.

    PubMed  CAS  Google Scholar 

  23. Doershuk CM, Winn RK, Coxson HO, Harlan JM. CD18-dependent and independent mechanisms of neutrophil emigration in the pulmonary and systemic microcirculation of rabbits. J Immunol. 1990; 144: 2327–2333.

    CAS  Google Scholar 

  24. Lachmann PJ, Walport MJ. Genetic deficiency diseases of the complement system. In: Ross GD, ed. Immunobiology of the Complement System. New York, Academic Press, 1986: 237–261.

    Google Scholar 

  25. Sellar GC, Cockburn D, Reid KBM. Localisation of the gene encoding the A chain, B chain and C chain of human Clq to 1p34.1 to 1p36 3. Immunogenetics. 1992; 35: 214–216.

    PubMed  CAS  Google Scholar 

  26. Steinsson K, McClean RH, Merrow M, Rothfield NF, Weinstein A. Selective complete Clq deficiency associated with systemic lupus erythematosus. J Rheumatol. 1983; 10: 590–594.

    PubMed  CAS  Google Scholar 

  27. Thompson RA, Haeney M, Reid KBM, Davies JG, White RHR, Cameron AH. A genetic defect of the Clq component of complement associated with childhood (immune complex) nephritis. N Engl J Med. 1980; 303: 22–24.

    PubMed  CAS  Google Scholar 

  28. McAdam R, Groundis D, Reid KBM. A homozygous point mutation results in a stop codon in the B-chain of a Clq-deficient individual. Immunogenetics. 1988; 27: 259–264.

    PubMed  CAS  Google Scholar 

  29. Tosi M, Duponche C, Meo T, Julier C. Complete cDNA sequence of human Cls and close physical linkage of the homologous genes Cls and Clr. Biochem. 1987; 26: 8516–8524.

    CAS  Google Scholar 

  30. Baur MP, Neugebauer M, Deppe H, Sigmund M, Luton T, Mayr WR, Albert ED. Population analysis on the basis of deduced phenotypes from random families. In: Albert ED, Baur MP, Mayr WR, eds. Histocompatibility Testing. Berlin, Heidelberg: Springer-Verlag; 1984: 333–341.

    Google Scholar 

  31. Dunckley H, Gatenby PA, Hawkins B, Naito S, Serjeantson SW. Deficiency of C4A is genetic determinant of systemic lupus erythematosus in three ethnic groups. J Immunogenetics. 1987; 14: 209–218.

    CAS  Google Scholar 

  32. Batchelor JR, Fielder AHL, Walport MJ, David J, Lord DK, Davey N, Dodi IA, Malesit ID, Wanachiwanin W, Bernstein R. Family study of the major histocompatibility complex in HLA DR3 negative patients with systemic lupus erythematosus. Clin Exp Immunol. 1987; 70: 364–371.

    PubMed  CAS  Google Scholar 

  33. Schifferli JA, Steiger G, Paccaud J-P, Sjoholm AJ, Hauptmann G. Difference in the biological properties of the two forms of the fourth component of human complement. Clin Exp Immunol. 1986; 63: 473–477.

    PubMed  CAS  Google Scholar 

  34. Christiansen FT, McCluskey J, Dawkins RL, Kay PH, Uko G, Zilko PJ. Complement allotyping in SLE: association with C4A null. Aust and NZ J Med. 1983; 13: 483–488.

    CAS  Google Scholar 

  35. Jacob CO, McDevitt HO. Tumour necrosis factor in murine autoimmune lupus nephritis. Nature. 1990; 331: 356–357.

    Google Scholar 

  36. Jacob CO, Fronek Z, Lewis GD, Koo M, Hansen JA, McDevitt HO. Heritable major histocompatibility complex class-II associated differences in the production of tumour necrosis factor a: relevance to genetic predisposition to systemic lupus erythematosus. Proc Natl Acad Sci USA. 1990; 87: 1233–1237.

    PubMed  CAS  Google Scholar 

  37. Carroll MC, Palsdottir A, Belt KT, Porter RR. Deletion of complement C4 and steroid 21hydroxylase genes in the HLA class III region. EMBO J. 1985; 4: 2547–2552.

    PubMed  CAS  Google Scholar 

  38. Mauff G, Alper CA, Awdeh ZL, Batchelor JR, Bertrams J, Brunn-Petersen G, Dawkins RL, Demant P, Edwards J, Grosse-Wilde H, Hauptmann G, Klouda P, Lamm L, Mollenhaur E, Nerl E, Olaison B, O’Neil GJ, Rittner C, Roos M, Skanes V, Teisberg P, Wells L. Statement of the nomenclature of C4 allotypes. Immunobiology. 1983; 164: 184–191.

    PubMed  CAS  Google Scholar 

  39. Hauptmann G, Tappeiner G, Schifferli JA. Inherited deficiency of the fourth component of human complement. Immunodef Rev. 1988; 1: 3–22.

    PubMed  CAS  Google Scholar 

  40. Braun L, Schneider NL, Giles CM, Bertrams J, Rittner C. Null alleles of human complement C4: evidence for pseudogenes at the C4A locus and for gene conversion at the C4B locus. J Exp Med. 1990; 171: 129–140.

    PubMed  CAS  Google Scholar 

  41. Agnello V. Complement deficiency states. Medicine. 1978; 57: 1–23.

    PubMed  CAS  Google Scholar 

  42. Glass D, Baum D, Gibson D, Stillman JS, Schur PH. Inherited deficiency of the second component of complement. J Clin Invest. 1976; 58: 853–861.

    PubMed  CAS  Google Scholar 

  43. Johnson CA, Densen P, Hurford RK, Colten HR, Westel RA. Type 1 human complement C2 deficiency - a 28 base pair gene deletion causes skipping of exon 6 during RNA splicing. J Biol Chem. 1992; 267: 9347–9353.

    PubMed  CAS  Google Scholar 

  44. Agostini A. Inherited Cl inhibitor deficiency. Complement. 1989; 6: 112–118.

    Google Scholar 

  45. Massa MC, Connolly MD. An association between C1-esterase inhibitor deficiency and lupus erythematosus. Report of two cases and review of the literature. J Am Acad Dermatol. 1982; 7: 255–264.

    PubMed  CAS  Google Scholar 

  46. Botto M, Fong KY, So AK, Rudge A, Walport MJ. Molecular basis of hereditary C3 deficiency. J Clin Invest. 1990; 86: 1158–1163.

    PubMed  CAS  Google Scholar 

  47. Botto M, Fong KY, So AK, Barlow R, Routier R, Morley BJ, Walport MJ. Homozygous hereditary C3 deficiency due to a partial gene deletion. Proc Natl Acad Sci USA. 1992; 89: 4957–4961.

    PubMed  CAS  Google Scholar 

  48. Thompson RA, Winterborn MH. Hypocomplementaemia due to genetic deficiency of ß1H globulin. Clin Exp Immunol. 1981; 46: 110–119.

    PubMed  CAS  Google Scholar 

  49. Fijen CAP, Kuijper EJ, Hannema AJ, Sjoholm AG, van Putten JPM. Complement deficiencies in patients over ten years old with meningococcal disease with uncommon serotypes. Lancet. 1989; 2: 585–588.

    PubMed  CAS  Google Scholar 

  50. Petersen BH, Lee Ti, Snyderman R, Brooks GF. Neisseria meningitidis and neisseria gonorrhoeae bacteraemia associated with C6, C7 and C8 deficiency. Ann Int Med. 1979; 90: 917–920.

    PubMed  CAS  Google Scholar 

  51. Trapp RG, Mooney E, Coleman TH, Forristal J, Herman JH. Hereditary complement (C6) deficiency associated with systemic lupus erythematosus, Sjogren’s syndrome and hyperthyroidism. J Rheumatol. 1987; 14: 1030–1033.

    PubMed  CAS  Google Scholar 

  52. Wilson JG, Wong W, Schur PH, Fearon DT. Mode of inheritance of decreased C3b receptors on erythrocytes of patients with systemic lupus erythematosus. N Engl J Med. 1982; 307: 981–986.

    PubMed  CAS  Google Scholar 

  53. Wilson JG, Murphy EE, Wong WW, Klickstein LB, Weis JH, Fearon DT. Identification of a restriction fragment length polymorphism by CR1 cDNA that correlates with the number of CR1 on erythrocytes. J Exp Med. 1986; 164: 50–59.

    PubMed  CAS  Google Scholar 

  54. Miykawa Y, Yamada A, Kosaka K, Tsuda F, Kosugie E, Mayumi M. Defective immune adherence (C3b) receptor on erythrocytes of patients with systemic lupus erythematosus. Lancet. 1981; 2: 493–497.

    Google Scholar 

  55. Walport MJ, Lachmann PJ. Erythrocyte complement receptor type 1, immune complexes and the rheumatic diseases. Arth Rheum. 1988; 31: 153–158.

    CAS  Google Scholar 

  56. Spitzer RE, Vallota EH, Forristal J, Sudora E, Stitzel A, Davis NC, West CD. Serum C’3 lytic system in patients with glomerulonephritis. Science. 1969; 164: 436–437.

    PubMed  CAS  Google Scholar 

  57. Sissons JGP, West RJ, Fallows J, Williams DG, Boucher BJ, Amos N, Peters DK. The complement abnormalities of partial lipodystrophy. N Engl J Med. 1976; 294: 461–465.

    PubMed  CAS  Google Scholar 

  58. Daha MR, Hazevoet HM, Van Es LA, Cats A. Stabilisation of the classical pathway C3 convertase C42 by a factor F-42, isolated from serum of patients with systemic lupus erythematosus. Immunology. 1980; 40: 417–424.

    PubMed  CAS  Google Scholar 

  59. Agnello V, Koffler D, Eisenberg JW, Winchester RJ, Kunkel HG. Clq precipitins in the sera of patients with systemic lupus erythematosus and other hypocomplementaemic states: characterisation of high and low molecular weight types. J Exp Med. 1971; 134: 228S.

    Google Scholar 

  60. Wener MH, Uwatako S, Mannik M. Antibodies to the collagen-like region of Clq in sera of patients with rheumatic diseases. Arthritis Rheum. 1989; 32: 544–551.

    PubMed  CAS  Google Scholar 

  61. Wisnieski JJ, Naff GB. Serum IgG antibodies to Clq in hypocomplementemic urticarial vasculitis syndrome. Arthritis Rheum. 1989; 32: 1119–27.

    PubMed  CAS  Google Scholar 

  62. Siegert CE, Daha MR, Halma C, van der Voort EA, Breedveld FC. IgG and IgA autoantibodies to Clq in systemic and renal diseases. Clin Exp Rheumatol. 1992; 10: 19–23.

    PubMed  CAS  Google Scholar 

  63. Schifferli JA, Ng YC, Peters DK. The role of complement and its receptor in the elimination of immune complexes. New Engl J Med. 1986; 315: 488–495.

    PubMed  CAS  Google Scholar 

  64. Moller NPH. Fc-mediated immune precipitation. I. A new role of the Fc portion of IgG. Immunology. 1979; 38: 631–640.

    PubMed  CAS  Google Scholar 

  65. Schifferli JA, Woo P, Peters DK. Complement-mediated inhibition of immune precipitation. I. Role of classical and alternative pathways. Clin Exp Immunol. 1982; 47: 555–562.

    PubMed  CAS  Google Scholar 

  66. Siegel I, Liu TL, Gleicher N. The red-cell immune system. Lancet. 1981; 2: 556–559.

    PubMed  CAS  Google Scholar 

  67. Cornacoff JB, Hebert LA, Smead WL, Vaneman LE, Birmingham DJ, Waxman FJ. Primate erythrocyte immune-complex clearing mechanism. J Clin Invest. 1983; 71: 236–247.

    PubMed  CAS  Google Scholar 

  68. Waxman FJ, Hebert LA, Cornacoff JB, Vaneman ME, Smead WL, Kraut EH, Birmingham DJ, Taguiam JM. Complement depletion accelerates the clearance of immune complexes from the circulation of primates. J Clin Invest. 1984; 74: 1329–1340.

    PubMed  CAS  Google Scholar 

  69. Schifferli JA, Ng YC, Paccaud JP, Walport MJ. The role of hypocomplementaemia and low erythrocyte complement receptor type 1 numbers in determining abnormal immune complex clearance in humans. Clin Exp Immunol. 1989; 75: 329–335.

    PubMed  CAS  Google Scholar 

  70. Theofilopoulos AN, Dixon FJ. The biology and detection of immune complexes. Adv Immunol. 1979; 28: 89–220.

    PubMed  CAS  Google Scholar 

  71. Uwatako S, Mannik M. Low molecular weight Clq-binding immunoglobulin G in patients with systemic lupus erythematosus consists of autoantibodies to the collagen-like region of Clq. J Clin Invest. 1988; 82: 816–824.

    Google Scholar 

  72. Aguado MT, Lambris JD, Tsokos GC, Burger R, Bitter-Suermann D, Tamerius JD, Dixon FJ, Theofilopoulos AN. Monoclonal antibodies against complement 3 neoantigens for detection of immune complexes and complement activation. Relationship between immune complex levels, state of C3, and numbers of receptors for C3b. J Clin Invest. 1985; 76: 1418–1426.

    PubMed  CAS  Google Scholar 

  73. Meltzer M, Franklin EC, Elias K, McCluskey RT, Cooper N. Cryoglobulinaemia - a clinical and laboratory study. II. Cryoglobulins with rheumatoid factor activity. Am J Med. 1966; 40: 837–851.

    PubMed  CAS  Google Scholar 

  74. Alpert E, Isselbacher KJ, Schur PH. The pathogenesis of arthritis associated with viral hepatitis. N Engl J Med. 1971; 285: 185–190.

    PubMed  CAS  Google Scholar 

  75. Michalak T. Immune complexes of Hepatitis B surface antigen in the pathogenesis of polyarteritis nodosa. A study of seven necropsy cases. Am J Pathol. 1978; 90: 619–632.

    PubMed  CAS  Google Scholar 

  76. Cohen R, Conn DL, Illstup DM. Clinical features, prognosis, and response to treatment in polyarteritis. Mayo Clin Proc. 1980; 55: 146–154.

    PubMed  CAS  Google Scholar 

  77. Garcia-Fuentes M, Chantier C, Williams DG. Cryoglobulinaemia in Henoch-Schonlein purpura. Br Med J. 1977; 2: 163–169.

    PubMed  CAS  Google Scholar 

  78. Shlomchik M, Mascelli M, Shan H, Radic MZ, Pisetsky D, Marshak-Rothstein A, Weigert A. Anti-DNA antibodies from autoimmune mice arise by clonal expression and somatic mutation. J Exp Med. 1990; 171: 265–297.

    PubMed  CAS  Google Scholar 

  79. Van Es JH, Meyling FHJG, Van De Akker WRM, Aanstoot H, Derksen RHWM, Logtenberg T. Somatic mutations in the variable regions of a human IgG anti-doublestranded DNA autoantibody suggest a role for antigen in the introduction of systemic lupus erythematosus. J Exp Med. 1991; 173: 461–470.

    PubMed  Google Scholar 

  80. Lachmann PJ, Walport MJ. Deficiency of the effector mechanisms of the immune response and autoimmunity. In: Whelan J, ed. Ciba Foundation Symposium 129: Autoimmunity and autoimmune diseases. Chichester: Wiley; 1987: 149–171.

    Google Scholar 

  81. Schifferli JA, Ng YC, Estreicher J, Walport MJ. The clearance of tetanus toxoid/anti-tetanus toxoid immune complexes from the circulation of humans. Complement and erythrocyte complement receptor 1-independent mechanisms. J Immunol. 1988; 140: 899–904.

    PubMed  CAS  Google Scholar 

  82. Davies KA, Peters AM, Beynon HLC, Walport MJ. Immune complex processing in patients with systemic lupus erythematosus - in vivo imaging and clearance studies. J Clin Invest. 1992; 90: 2075–2083.

    PubMed  CAS  Google Scholar 

  83. Perlmutter DH, Strunk RC, Goldberger G, Cole FS. Regulation of complement protein C2 and factor B by interleukin-1 and gamma interferon acting on transfected L cells. Mol Immunol. 1986; 23: 1263–1266.

    PubMed  CAS  Google Scholar 

  84. Heurkens AH, Breedveld FC, Keur CV, Brand R, Daha MR. Degradation of aggregates of activated C3 (C3b) by monocytes of patients with rheumatoid arthritis is related to vasculitis. Clin Exp Immunol. 1990; 80: 177–180.

    PubMed  CAS  Google Scholar 

  85. O’Sullivan MM, Amos N, Bedwell A, Williams BD. Complement mediated inhibition of immune precipitation in rheumatoid vasculitis. Rheumatol Int. 1990; 10: 159–163.

    PubMed  Google Scholar 

  86. Natvig JB, Randen I, Thompson K. Probing of the rheumatoid factor (RF) gene repertoire in rheumatoid arthritis (RA) by hybridoma clones. Clin Exp Immunol. 1990; 8 (Suppl 5): 75–80.

    Google Scholar 

  87. Gause A, Kuppers R, Mierau I. A somatically mutated Vic IV gene encoding a human rheumatoid factor light chain. Clin Exp Immunol. 1992; 88: 430–434.

    PubMed  CAS  Google Scholar 

  88. Mageed RA, Kirwan JR, Holborow EJ. Localisation of circulating immune complexes from patients with rheumatoid arthritis in murine spleen germinal centres. Scand J Immunol. 1991; 34: 323–331.

    PubMed  CAS  Google Scholar 

  89. Lloyd W, Schur PH. Immune complexes, complement, and anti-DNA in exacerbations of systemic lupus erythematosus (SLE). Medicine Baltimore. 1981; 60: 208–217.

    PubMed  CAS  Google Scholar 

  90. Cameron JS, Lessof MH, Ogg CS, Williams BD, Williams DG. Disease activity in the nephritis of systemic lupus erythematosus in relation to serum complement concentrations. DNA-binding capacity and precipitating anti-DNA antibody. Clin Exp Immunol. 1976; 25: 418–427.

    PubMed  CAS  Google Scholar 

  91. Alper CA, Rosen FS, Watson L. Studies of the in vivo behaviour of human C3 in normal subjects and patients. J Clin Invest. 1967; 46: 2021–2034.

    PubMed  CAS  Google Scholar 

  92. Lockshin MD, Qamar T, Levy RA, Druzin ML. Pregnancy in systemic lupus erythematosus. Clin Exp Rheumatol. 1989; 7 (Suppl 3): 5195–197.

    Google Scholar 

  93. Kovacsovics T, Tschopp J, Kress A, Isliker H. Antibody-independent activation of Cl, the first component of complement by cardiolipin. J Immunol. 1985; 135: 2695–2700.

    PubMed  CAS  Google Scholar 

  94. Hugli TE. Biochemistry and biology of anaphylatoxins. Complement. 1986; 3: 111–127.

    PubMed  CAS  Google Scholar 

  95. Biesecker G, Lavin L, Ziskind M, Koffler D. Cutaneous localisation of the membrane attack complex in discoid and systemic lupus erythematosus. N Engl J Med. 1982; 306: 264–270.

    PubMed  CAS  Google Scholar 

  96. Dahlback K, Lofberg H, Dahlback B. Vitronectin colocalises with Ig deposits and C9 neoantigen in discoid lupus erythematosus and dermatitis herpetiformis, but not in bullous pemphigoid. Br J Dermatol. 1989; 120: 725–733.

    PubMed  CAS  Google Scholar 

  97. Krishnan C, Kaplan MH. Immunopathologic studies of systemic lupus erythematosus. II. Anti-nuclear reaction of gamma-globulin eluted from homogenates and isolated glomeruli of kidneys from patients with lupus nephritis. J Clin Invest. 1967; 46: 569–580.

    PubMed  CAS  Google Scholar 

  98. Biesecker G, Katz S, Koffler D. Renal localisation of the membrane attack complex in systemic lupus erythematosus. J Exp Med. 1981; 154: 1779–1794.

    PubMed  CAS  Google Scholar 

  99. Sims PJ, Wiedmer T. The response of human platelets to activated components of the complement system. Immunology Today. 1991; 12: 338–342.

    PubMed  CAS  Google Scholar 

  100. Peltier AP, Vial MC, de Seze S. Etudes sur le méchanisme de la baisse du taux de complément dans le liquide synovial au cours de la polyarthrite rheumatoide. 1. Variations respectives des taux de C’1, C’2, et C’3 et du taux de complément total. Pathologie et Biologie. 1970; 18: 959–967.

    PubMed  CAS  Google Scholar 

  101. Brodeur JP, Ruddy S, Schwartz LB, Mosley G. Synovial fluid levels of complement to SCSb-9 and fragment Bb are elevated in patients with rheumatoid arthritis. Arthritis Rheum. 1991; 34: 1531–1537.

    PubMed  CAS  Google Scholar 

  102. Mellbye OJ, Fprre O, Mollnes TE, Kvarnes L. Immunopathology of subcutaneous rheumatoid nodules. Ann Rheum Dis. 1991; 50: 909–912.

    PubMed  CAS  Google Scholar 

  103. Kissel JT, Mendel JR, Rammahon KW. Microvascular deposits of complement membrane attack complex in dermatomyositis. N Engl J Med. 1986; 314: 329–334.

    PubMed  CAS  Google Scholar 

  104. Colten HR. Biosynthesis of complement. Adv Immunol. 1976; 22: 67–118.

    PubMed  CAS  Google Scholar 

  105. Colten HR, Strunk RC, Cole FS, Perlmutter DH. Regulation of complement protein biosynthesis in mononuclear phagocytes. In: Biochemistry of Macrophages. London: Pitman; 1986; 141–154.

    Google Scholar 

  106. Moffat BJ, Lappin D, Birnie GD, Whaley K. Complement biosynthesis in human synovial tissue. Clin Exp Immunol. 1989; 78: 54–60.

    PubMed  CAS  Google Scholar 

  107. Ruddy S, Colten HR. Rheumatoid arthritis. Biosynthesis of complement proteins by synovial tissues. N Engl J Med. 1974; 290: 1284–1288.

    PubMed  CAS  Google Scholar 

  108. Firestein GS, Paine MM, Littman BH. Gene expression (collagenase, tissue inhibitor of metalloproteinases, complement, and HLA-DR) in rheumatoid arthritis and osteoarthritis synovium. Quantitative analysis and effect of intraarticular corticosteroids. Arthritis Rheum. 1991; 34: 1094–1105.

    PubMed  CAS  Google Scholar 

  109. Katz Y, Strunk RC. Synovial fibroblast-like cells synthesise seven proteins of the complement system. Arthritis Rheum. 1988; 31: 1365–1371.

    PubMed  CAS  Google Scholar 

  110. Cochrane CGH, Muller-Eberhard HG, Aikon BS. Depletion of plasma complement in vivo by a protein of cobra venom: its effect on various immunologic reactions. J Immunol. 1970; 105: 55–64.

    PubMed  CAS  Google Scholar 

  111. Klickstein LB, Barlow TJ, Miletic V, Robson LD, Smith JA, Fearon DT. Identification of distinct Cab and C4b recognition sites in the human C5b/C4b receptor (CR1, CD35) by deletion mutagenesis. J Exp Med. 1988; 168: 1699–1717.

    PubMed  CAS  Google Scholar 

  112. Weisman HF, Barlow T, Leppo MK, Marsh HC, Carson GR, Concino MF, Boyle MP, Roux KH, Weisfeldt ML, Fearon DT. Soluble human complement receptor type 1: in vivo inhibitor of the complement suppressing post-ischaemic myocardial inflammation and necrosis. Science. 1990; 249: 146–151.

    PubMed  CAS  Google Scholar 

  113. Mulligan MS, Yeh CG, Rudolph AR, Ward PA. Protective effects of soluble CR1 in complement-and neutrophil-mediated tissue injury. J Immunol. 1992; 148: 1479–1485.

    PubMed  CAS  Google Scholar 

  114. Pruitt SK, Baldwin WM, Marsh HC, Lin SS, Yeh CG, Bollinger RR. The effect of soluble complement receptor type 1 on hyperacute allograft rejection. Transplantation. 1991; 50: 868–873.

    Google Scholar 

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  1. Guy’s Hospital, London, UK

    G. S. Panayi (ARC Professor of Rheumatology) (ARC Professor of Rheumatology)

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© 1994 Springer Science+Business Media Dordrecht

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Vyse, T.J., Walport, M.J. (1994). The Complement System and Connective Tissue Disease. In: Panayi, G.S. (eds) Immunology of the Connective Tissue Diseases. Immunology and Medicine Series, vol 22. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-1432-5_7

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  • DOI: https://doi.org/10.1007/978-94-011-1432-5_7

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-4627-5

  • Online ISBN: 978-94-011-1432-5

  • eBook Packages: Springer Book Archive

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