Skip to main content
SpringerLink
Log in

Complement deficiency and immune complex disease

  • Published:
Springer Seminars in Immunopathology Aims and scope Submit manuscript

Conclusions

Study of patients with complement deficiency has supplied important insights into the physiological importance of this component of the innate immune system. The most surprising finding to emerge from the study of such patients is the strong link between deficiencies of classical pathway proteins and susceptibility to SLE. This observation has stimulated many studies into the relationship between the complement system and processing mechanisms for immune complexes, which we have reviewed in this chapter. Although it is clear that complement deficiency is associated with several abnormalities of immune complex processing in vivo, the challenge still remains to provide a convincing link between these and the development of SLE.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

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

    PubMed  Google Scholar 

  2. Atkinson JP, Frank MM (1974) Studies on the in vivo effects of antibody. Interaction of IgM antibody and complement in the immune clearance and destruction of erythrocytes in man. J Clin Invest 54:339

    PubMed  Google Scholar 

  3. Balestrieri G, Tincani A, Migliorini P, Ferri C, Cattaneo R, Bombardiert S (1984) Inhibitory effect of IgM rheumatoid factor on immune complex solubilization capacity and inhibition of immune precipitation. Arthritis Rheum 27:1130

    PubMed  Google Scholar 

  4. Barbosa JE, Harrison RA, Backer PJ, Lachmann PJ (1992) An antipeptide antibody that recognizes a neoantigen in the CR1 stump remaining on E after proteolysis. Clin Exp Immunol 87:144

    PubMed  Google Scholar 

  5. Bartow TJ, Klickstein LB, Fearon DT (1989) Localization of monoclonal antibody epitopes on Cr1 by deletion mutagenesis. Complement Inflamm 6:312

    Google Scholar 

  6. Botto M, Walport MJ (1993) Heriditary deficiency of C3 in animals and humans. Int Rev Immunol (in press)

  7. Brai M, Misiano G, Maringhini S, Cutaja I, Hauptmann G (1988) Combined homozygous factor H and heterozygous C2 deficiency in an Italian family. J Clin Immunol 8:50

    PubMed  Google Scholar 

  8. Brown HC, Broom JC (1938) Studies in trypanosomiasis. II. Observations on the red cell adhesion test. Trans R Soc Trop Med Hyg 32:209

    Google Scholar 

  9. Christiansen FT, Dawkins RL, Uko G, McCluskey J, Kay PH, Zilko PJ (1983) Complement allotyping in SLE: association with C4A null. Aust N Z J Med 13:483

    PubMed  Google Scholar 

  10. Cohen JHM, Caudwell V, Levi-Strauss M, Bourgeois P, Kazatchkine MD (1989) Genetic analysis of CR1 expression on erythrocytes of patients with systemic lupus erythematosus. Arthritis Rheum 32:393

    PubMed  Google Scholar 

  11. Cosio FG, Shen XP, Hebert LA (1990) Immune complexes bind preferentially to specific subpopulations of human erythrocytes. Clin Immunol Immunopathol 55:337

    PubMed  Google Scholar 

  12. Couser WG, Salant DJ (1980) In-situ immune complex formation and glomerular injury. Kidney Int 17:1

    PubMed  Google Scholar 

  13. Daha MR, Hazevoet H, van Es LA, Cats A (1980) Stabilization of the classical pathway C3 convertase C4,2, by a factor F-4,2, isolated from the serum of patients with systemic lupus erythrematosus. Immunology 40:417

    PubMed  Google Scholar 

  14. Davies KA, Hird V, Stewart S, Sivolapenko GB, Jose P, Epenetos AA, Walport MJ (1990) A study of in vivo immune complex formation and clearance in man. J Immunol 144:4613

    PubMed  Google Scholar 

  15. Davies KA, Peters AM, Beynon HLC, Walport MJ (1992) Immune complex processing in systemic lupus erythematosus-in vivo imaging and clearance studies. J Clin Invest 90:2075

    PubMed  Google Scholar 

  16. Donaldson VH, Hess EV, McAdams AJ (1977) Lupus-erythematosus-like disease in three unrelated women with hereditary angioneurotic edema (letter). Ann Intern Med 86:312

    PubMed  Google Scholar 

  17. Duke HL, Wallace JM (1930) “Red-cell adhesion” in trypanosomiasis of man and animals. Parasitology 22:414

    Google Scholar 

  18. Dunckley H, Gatenby PA, Hawkins B, Naito S, Serjeantson SW (1987) Deficiency of C4A is a genetic determinant of systemic lupus erythematosus in three ethnic groups. J Immunogenet 14:209

    PubMed  Google Scholar 

  19. Edberg JC, Kujala GA, Taylor RP (1987) Rapid immune adherence reactivity of nascent, soluble antibody/DNA immune complexes in the circulation. J Immunol 139:1240

    PubMed  Google Scholar 

  20. Emlen W, Carl V, Burdick G (1992) Mechanism of transfer of immune complexes from red blood cell CR1 to monocytes. Clin Exp Immunol 89:8

    PubMed  Google Scholar 

  21. Fearon DT (1980) Identification of the membrane glycoprotein that is the C3b receptor of the human erythrocyte, polymorphonuclear leukocyte, B lymphocyte, and monocyte. J Exp Med 152:20

    PubMed  Google Scholar 

  22. Fielder AHL, Walport MJ, Batchelor JR, Rynes RI, Black CM, Dodi IA, Hughes GRV (1983) Family study of the major histocompatibility complex in patients with systemic lupus erythematosus: importance of null alleles of C4A and C4B in determining disease susceptibility. Br Med J 286:425

    Google Scholar 

  23. Font J, Herrero C, Bosch X, Cervera R, Ingelmo M, Mascaro JM (1990) Systemic lupus erythematosus in a patient with partial lipodystrophy. J Am Acad Dermatol 22:337

    PubMed  Google Scholar 

  24. Frank MM, Hamburger MI, Lawley TJ, Kimberly RP, Plotz PH (1979) Defecitve reticoloendothelial system Fc-receptor function in systemic lupus erythematosus. N Engl J Med 300:518

    PubMed  Google Scholar 

  25. Frank MM, Lawley TJ, Hamburger MI, Brown EJ (1983) Immunoglobulin G Fc receptormediated clearance in autoimmune diseases. Ann Intern Med 98:206

    PubMed  Google Scholar 

  26. Fries LF, Gaither TA, Hammer CH, Frank MM (1984) C3b covalently bound to IgG demonstrates a reduced rate of inactivation by factor H and I. J Exp Med 160:1640

    PubMed  Google Scholar 

  27. Haakenstad AO, Mannik M (1974) Saturation of the reticuloendothelial system with soluble immune complexes. J Immunol 112:1939

    PubMed  Google Scholar 

  28. Halma C, Daha MR, van Furth R, Camps JA, Evers Schouten JH, Pauwels EK, Lobatto S, van Es LA (1989) Elimination of soluble123I-labelled aggregates of human immunoglobulin G in humans; the effect of splenectomy. Clin Exp Immunol 77:62

    PubMed  Google Scholar 

  29. Hamburger MI, Lawley TJ, Kimberly RP, Plotz PH, Frank MM (1982) A serial study of splenic reticuloendothelial system Fc-receptor function in systemic lupus erythematosus. Arthritis Rheum 25:48

    PubMed  Google Scholar 

  30. Hammond A, Rudge AC, Loizou S, Bowcock SJ, Walport MJ (1989) Reduced numbers of complement receptor type 1 on erythrocytes are associated with increased levels of anticardiolipin antibodies. Arthritis Rheum 32:259

    PubMed  Google Scholar 

  31. Hartung K, Baur MP, Coldwey R, Fricke M, Kalden JR, Lakomek HJ, Peter HH, Schendel D, Schneider PM, Seuchter SA, Stangel W, Deicher HRG (1992) Major histocompatibility complex haplotypes and complement C4 alleles in systemic lupus erythematosus. J Clin Invest 90:1346

    PubMed  Google Scholar 

  32. Hauptmann G, Goetz J, Uring-Lambert B, Grosshans E (1986) Component deficiencies. 2. The fourth component. Prog Allergy 39:232

    PubMed  Google Scholar 

  33. Hebert LA, Cosio FG (1987) The erythrocyte-immune complex-glomerulonephritis man. Kidney Int 31:877

    PubMed  Google Scholar 

  34. Heidelberger M (1941) Quantitative chemical studies on complement or alexin. I. A method. J Exp Med 73:691

    Google Scholar 

  35. Hong K, Takata Y, Sayama K, Konozo H, Takeda J, Nakata Y, Kinoshita T, Inoue K (1984) Inhibition of immune precipitation by complement. Immunology 133:1464

    Google Scholar 

  36. Horstmann RD, Müller-Eberhard HJ (1985) Isolation of rabbit C3, Factor B, and Factor H and comparison of their properties with those of the human analog. J Immunol 134:1094

    PubMed  Google Scholar 

  37. Iida K, Mornaghi R, Nussenzweig V (1982) Complement receptor (CR1) deficiency in erythrocytes from patients with systemic lupus erythematosus. J Exp Med 155:1427

    PubMed  Google Scholar 

  38. Jasin HE (1979) Systemic lupus erythematosus, partial lipodystrophy and hypocomplementemia. J Rheumatol 6:43

    PubMed  Google Scholar 

  39. Johnson A, Harkin S, Steward MW, Whaley K (1987) The effect of immunoglobulin isotype and antibody affinity on complement-mediated inhibition of immune precipitation and solubilisation. Mol Immunol 24:1211

    PubMed  Google Scholar 

  40. Kazatchkine MD, Fearon DT (1990) Deficiencies of human C3 complement receptors type 1 (CR1, CD35) and type 2 (CR2, CD21). Immunodefic Rev 2:17

    PubMed  Google Scholar 

  41. Kimberly RP, Ralph P (1983) Endocytosis by the mononuclear phagocyte system and autoimmune disease. Am J Med 74:481

    PubMed  Google Scholar 

  42. Kimberly RP, Paris TM, Inman RD, McDougal JS (1983) Dynamics of mononuclear phagocyte system Fc receptor function in systemic lupus erythematosus. Relation to disease activity and circulating immune complexes. Clin Exp Immunol 51:261

    PubMed  Google Scholar 

  43. Kishore N, Shah D, Skanes VM, Levine RP (1988) The fluid-phase binding of human C4 and its genetic variants, C4A3 and C4B1, to immunogloublins. Mol Immunol 25:811

    PubMed  Google Scholar 

  44. Klopstock A, Schartz J, Bleiberg Y, Adam A, Szeinberg A, Schlomo J (1965) Herditary nature of the behaviour of erythrocytes in immune adherence — haemagglutination phenomenon. Vox Sang 10:177

    PubMed  Google Scholar 

  45. Kohler PF, Percy J, Campion WM, Smyth CJ (1974) Hereditary angioedema and “familial” lupus erythematosus in identical twin boys. Am J Med 56:406

    PubMed  Google Scholar 

  46. Lachmann PJ, Walport MJ (1987) Deficiency of the effector mechanisms of the immune response and autoimmunity. Ciba Found Symp 129:149

    PubMed  Google Scholar 

  47. Law SK, Dodds AW, Porter RR (1984) A comparison of the properties of two classes, C4A and C4B, of the human complement component C4. EMBO J 3:1819

    PubMed  Google Scholar 

  48. Levy M, Halbwachs Mecarelli L, Guber MC, Kohout G, Bensenouci A, Niaudet P, Hauptmann G, Lesavre P (1986) H deficiency in two borthers with atypical dense intramembranous deposit disease. Kidney Int 30:949

    PubMed  Google Scholar 

  49. Lobatto S, Daha MR, Voetman AA, Evers Schouten JH, Van Es AA, Pauwels EKJ, van Es LA (1987) Clearance of soluble aggregates of immunoglobulin G in healthy volunteers and chimpanzees. Clin Exp Immunol 68:133

    Google Scholar 

  50. Lobatto S, Daha MR, Breedveld FC, Pauwels EK, Evers Schouten JH, Voetman AA, Cats A, van Es LA (1988) Abnormal clearance of soluble aggregates of human immunoglobulin G in patients with systemic lupus erythematosus. Clin Exp Immunol 72:55

    PubMed  Google Scholar 

  51. Loos M, Heinz H-P (1986) Component deficiencies. 1. The first component: C1Q, C1R, C1s. Prog Allergy 39:212

    PubMed  Google Scholar 

  52. Lutz HU (1990) Erythrocyte clearance. In: Harris JR (ed) Blood cell biochemistry, erythroid cells. Plenum Press, New York, pp 81–120

    Google Scholar 

  53. Lutz HU, Stammler P, Fasler S, Ingold M, Fehr J (1992) Density separation of human red blood cells on self forming Percoll gradients: correlation with age. Biochim Biophys Acta 1116(1):1

    PubMed  Google Scholar 

  54. Madi N, Paccaud J-P, Steiger G, Schifferli JA (1989) Immune adherence of nascent hepatitis B surface antigen-antibody complexes in vivo in humans. Clin Exp Immunol 78:201

    PubMed  Google Scholar 

  55. Madi N, Paccaud JP, Steiger G, Schifferli JA (1991) Immune complex binding efficiency of erythrocyte complement receptor 1 (CR1). Clin Exp Immunol 84:9

    PubMed  Google Scholar 

  56. Medof ME, Prince GM (1983) Immune complex alterations occur on the human red blood cell membrane. Immunology 50:11

    PubMed  Google Scholar 

  57. Medof ME, Iida K, Mold C, Nussenzweig V (1982) Unique role of the complement receptor CR1 in the degradation of C3b associated with immune complexes. J Exp Med 156:1739

    PubMed  Google Scholar 

  58. Meyer O, Hauptmann G, Tappeiner G, Ochs HD, Mascart Lemone F (1985) Genetic deficiency of C4, C2 or C1q and lupus syndromes. Association with anti-Ro (SS-A) antibodies. Clin Exp Immunol 62:678

    PubMed  Google Scholar 

  59. Miller GW, Nussenzweig V (1975) A new complement function: solubilization of antigenantibody aggregates. Proc Natl Acad Sci USA 72:418

    PubMed  Google Scholar 

  60. Mitchell WS, Naama JK, Veitch J, Whaley K (1984) IgM-RF prevents complement mediated inhibition of immune precipitation. Immunology 52:445

    PubMed  Google Scholar 

  61. Miyakawa Y, Yamada A, Kosaka K, Tsuda F, Kosugi E, Mayumi M (1981) Defective immune-adherence (C3b) receptor on erythrocytes from patients with systemic lupus erythematosus. Lancet II:493

    Google Scholar 

  62. Moldenhauer F, David J, Fielder AHL, Lachmann PJ, Walport MJ (1987) Inherited deficiency of erythrocyte complement receptor type 1 does not cause susceptibility to systemic lupus erythematosus. Arthritis Rheum 30:961

    PubMed  Google Scholar 

  63. Moller NPH, Christiansen G (1983) Fc mediated immune precipitation. III. Visualisation by electron microscopy. Immunolgoy 48:469

    Google Scholar 

  64. Moller NP, Steengaard J (1979) Fc mediated immune precipitation. I. A new role of the Fc portion of IgG. Immunology 38:631

    PubMed  Google Scholar 

  65. Morgan CR, Sorenson RL, Lazarow A (1964) Further studies of an inhibitor of the two antibody immunoassay system. Diabetes 13:579

    PubMed  Google Scholar 

  66. Muller-Eberhard HJ, Kunkel HG (1961) Isolation of the thermolabile serum protein which precipitates gamma-globulin aggregates and participates in immune haemolysis. Proc Soc Exp Biol Med 106:291

    PubMed  Google Scholar 

  67. Naama JK, Hamilton AO, Yeung-Laiwah AC, Whaley K (1984) Prevention of immune precipitation by purified classical pathway components. Clin Exp Immunol 58:486

    PubMed  Google Scholar 

  68. Nelson DS (1963) Immune adherence. Adv Immunol 3:131

    Google Scholar 

  69. Nelson RA (1953) The immune adherence phenomenon: an immunologically specific reaction between micro-organisms and erythrocytes leading to enhanced phagocytosis. Science 118:733

    PubMed  Google Scholar 

  70. Nelson RA (1956) The immune adherence phenomenon. A hypotetical role of erythrocytes in defence against bacteria and viruses. Proc R Soc Med 49:55

    PubMed  Google Scholar 

  71. Ng YC, Peters DK, Walport MJ (1984) Monoclonal rheumatoid factor-Ig immune complexes. Poor fixation of opsonic C4 and C3 despite efficient complement activation. Arthritis Rheum 31:99

    Google Scholar 

  72. Ng YC, Schifferli JA, Walport MJ (1988) Immune complexes and erythrocyte CR1 (complement receptor type 1): effect of CR1 numbers on binding and release reactions. Clin Exp Immunol 71:481

    PubMed  Google Scholar 

  73. Nilsson UR, Nilsson B, Storm KE, Sjolin Forsberg G, Hallgren R (1992) Hereditary dysfunction of the third component of complement associated with a systemic lupus erythematosus-like syndrome and meningococcal meningitis. Arthritis Rheum 35:580

    PubMed  Google Scholar 

  74. Olsen ML, Goldstein R, Arnett FC, Duvic M, Pollack M, Reveille JD (1989) C4A gene deletion and HLA associations in black Americans with systemic lupus erythematosus. Immunogenetics 30:27

    PubMed  Google Scholar 

  75. Paccaud JP, Steiger G, Sjöholm AG, Spaeth PJ, Schifferli JA (1987) Tetanus toxoid-antitetanus toxoid complexes: a potential model to study the complement transport system for immune complex in humans. Clin Exp Immunol 69:468

    PubMed  Google Scholar 

  76. Paccaud JP, Carpentier J-L, Schifferli JA (1988) Direct evidence for the clustered nature of complement receptors type 1 on the erythrocyte membrane. J Immunol 141:3889

    PubMed  Google Scholar 

  77. Paccaud JP, Carpentier JL, Schifferli JA (1990) Exudation induces clustering of CR1 receptors at the surface of human polymorphonuclear leukocytes. Biochem Biophys Res Commun 172:1203

    PubMed  Google Scholar 

  78. Paccaud JP, Carpentier JL, Schifferli JA (1990) Difference in the clustering of complement receptor type 1 (CR1) on polmorphonuclear leukocytes and erythrocytes: effect on immune adherence. Eur J Immunol 20:283

    PubMed  Google Scholar 

  79. Paccaud JP, Schifferli JA, Baggiolini M (1990) NAP-1/IL-8 induces upregulation of CR1 receptors in human neutrophil leucocytes. Biochem Biophys Res Common 166:187

    Google Scholar 

  80. Pan CG, Strife CF, Ward MK, Spitzer RE, McAdams AJ (1992) Long-term follow-up of non-systemic lupus erythematosus glomerulonephritis in patients with hereditary angioedema: report of four cases. Am J Kidney Dis 19:526

    PubMed  Google Scholar 

  81. Parris TM, Kimberly RP, Imman RD, McDougal S, Gibofsky A, Christian C (1982) Defective Fc-receptor mediated function of the mononuclear phagocyte system in lupus nephritis. Ann Intern Med 97:526

    PubMed  Google Scholar 

  82. Pascual M, Lutz H, Stieger G, Schifferli JA (1993) Release of vesicles enriched in CR1 from human erythrocytes during ATP-depletion. J Immunol 151(1): 397

    PubMed  Google Scholar 

  83. Provost TT, Arnett FC, Reichlin M (1983) Homozygous C2 deficiency, lupus erythematosus and anti-Ro (SSA) antibodies. Arthritis Rheum 26:1279

    PubMed  Google Scholar 

  84. Quadri RA, Schifferli JA (1992) Overestimation of the number of complement receptor type 1 (CR1) on erythrocytes. Scand J Immunol 36:125

    PubMed  Google Scholar 

  85. Rasmussen JM, Teisner B, Jepsen HH, Svehag S-E, Knudsen F, Kirstein H, Buhl M (1988) Three cases of factor I deficiency: the effect of treatment with plasma. Clin Exp Immunol 74:131

    PubMed  Google Scholar 

  86. Reid KB (1989) Deficiency of the first component of human complement. Immunodefic Rev 1:247

    PubMed  Google Scholar 

  87. Ripoche J, Sim RB (1986) Loss of complement receptor type 1 (CR1) on ageing of erythrocytes. Studies of proteolytic release of the receptor. Biochem J 235:815

    PubMed  Google Scholar 

  88. Rodwell JD, Tang LH, Schumaker VN (1980) Antigen valence and Fc-localised secondary forces in antibody precipitation. Mol Immunol 17:1591

    PubMed  Google Scholar 

  89. Ross GD, Yount WJ, Walport MJ, Winfield JB, Parker CJ, Fuller CR, Taylor RP, Myones BL, Lachmann PJ (1985) Disease-associated loss of erythrocyte complement receptors (CR1, C3b-receptors) in patients with systemic lupus erythematosus and other diseases involving autoantiboides and/or complement activation. J Immunol 135:2005

    PubMed  Google Scholar 

  90. Ruddy S (1986) Component deficiencies. 3. The second component. Prog Allergy 39:250

    PubMed  Google Scholar 

  91. Sano Y, Nishimukai H, Kitamura H, Nagaki K, Inai S, Hamasaki Y, Maruyama I, Igata A (1981) Hereditary deficiency of the third component of complement in two sisters with systemic lupus erythematosus-like symptoms. Arthritis Rheum 24:1255

    PubMed  Google Scholar 

  92. Schifferli JA, Peters DK (1982) Complement-mediated inhibition of immune precipitation. Analysis by sucrose density gradient ultracentifugation. Clin Exp Immunol 47:563

    PubMed  Google Scholar 

  93. Schifferli JA, Taylor RP (1989) Physiological and pathological aspects of circulating immune complexes. Kidney Int 35:993

    PubMed  Google Scholar 

  94. Schifferli JA, Bartolotti SR, Peters DK (1980) Inhibition of immune precipitation by complement. Clin Exp Immunol 42:387

    PubMed  Google Scholar 

  95. Schifferli JA, Woo P, Peters DK (1982) Complement-mediated inhibition of immune precipitation. Role of the classical and alternative pathways. Clin Exp Immunol 47:555

    PubMed  Google Scholar 

  96. Schifferli JA, Steiger G, Hauptmann G, Spath PJ, Sjoholm AG (1985) Formation of soluble immune complexes by complement in sera of patients with various hypocomplementemic states. J Clin Invest 74:2127

    Google Scholar 

  97. Schifferli JA, Steiger G, Schapira M (1985) The role of C1, C1 inhibitor and C4 in modulating immune precipitation. Clin Exp Immunol 60:605

    PubMed  Google Scholar 

  98. Schifferli JA, Steiger G, Paccaud J-P (1986) Complement mediated inhibition of immune precipitation and solubilisation generate different concentrations of complement anaphylatoxins (C4a, C3a, C5a). Clin Exp Immunol 64:407

    PubMed  Google Scholar 

  99. Schifferli JA, Steiger G, Paccaud JP, Sjoholm AG, Hauptmann G (1986) Difference in the biological properties of the two forms of the fourth component of human complement (C4). Clin Exp Immunol 63:473

    PubMed  Google Scholar 

  100. Schifferli JA, Hauptmann G, Paccaud JP (1987) Complement-mediated adherence of immune complexes to human erythrocytes. Difference in the requirements for C4A and C4B. FEBS Lett 213:415

    PubMed  Google Scholar 

  101. Schifferli JA, Ng YC, Estreicher J, Walport MJ (1988) The clearance of tetanus toxoid/antitetanus toxoid immune complexes from the circulation of humans. Complement- and erythrocyte complement receptor 1-dependent mechanisms. J Immunol 140:899

    PubMed  Google Scholar 

  102. Schifferli JA, Ng YC, Paccaud J-P, Walport MJ (1989) The role of hypocomplementaemia and low erythrocyte complement receptor type 1 numbers in determining abnormal immune complex clearance in humans. Clin Exp Immunol 75:329

    PubMed  Google Scholar 

  103. Schur PH, Marcus-Bagley D, Awdeh Z, Yunis EJ, Alper CA (1990) The effect of ethnicity on major histocompatibility complex complement allotypes and extended haplotypes in patients with systemic lupus erythematosus. Arthritis Rheum 33:985

    PubMed  Google Scholar 

  104. Soeldner JS, Slone D (1986) Critical variables in the radioimmunoassay of serum insulin using the double antibody technique. Diabetes 14:771

    Google Scholar 

  105. Solal-Celigny P, Laviolette M, Herbert J, Atkins PC, Sirois M, Brun G, Lehner-Netsch G, Delage JM (1982) C3b inactivator deficiency with immune complex manifestations. Clin Exp Immunol 47:197

    PubMed  Google Scholar 

  106. Späth PJ, Pascual M, Meyer-Hänni L, Schaad UB, Schifferli JA (1988) Solubilization of immune precipitates by complement in the absence of properdin or factor D. FEBS Lett 234:131

    PubMed  Google Scholar 

  107. Taylor RP, Wright EL, Pocanic F (1989) Quantitative analyses of C3b capture and immune adherence of IgM antibody/dsDNA immune complexes. J Immunol 143:3626

    PubMed  Google Scholar 

  108. Taylor RP, Pocanic F, Reist C, Wright EL (1991) Complement-opsonized IgG antibody/ dsDNS immune complexes bind to CR1 clusters on isolated human erythrocytes. Clin Immunol Immunopathol 61:143

    PubMed  Google Scholar 

  109. Thompson RA, Winterborn MH (1981) Hypocomplementaemia due to a genetic deficiency of b1H globulin. Clin Exp Immunol 46:110

    PubMed  Google Scholar 

  110. Utiger RD, Parker ML, Daughaday WH (1962) Studies on human growth hormone. I. A radioimmunoassay for human growth hormone. J Clin Invest 41:254

    PubMed  Google Scholar 

  111. Valentijn RM, van Overhagen H, Hazevoet HM, Hermans J, Cats A, Daha MR, van Es LA (1985) The value of complement and immune complex determinations in monitoring disease activity in patients with systemic lupus erythematosus. Arthritis Rheum 28:904

    PubMed  Google Scholar 

  112. Van Dyne S, Holers VM, Lublin DM, Atkinson JP (1987) The polymorphism of the C4b/C4b receptor in the normal population and in patients with systemic lupus erythematosus. Clin Exp Immunol 68:570

    PubMed  Google Scholar 

  113. Varga L, Thiry E, Fust G (1988) BSA-anti-BSA immune complexes formed in the presence of serum do not bind to autologous red cells. Immunology 64:381

    PubMed  Google Scholar 

  114. Wallace JM, Wormall A (1931) Red cell adhesion in trypanosomiasis of man and other animals II. Some experiments on the mechanism of the reaction. Parasitology 23:346

    Google Scholar 

  115. Walport MJ, Peters AM, Elkon KB, Pusey C, Lavender JP, Hughes GRV (1985) The splenic extraction ratio of antibody-coated erythrocytes and its response to plasma exchange and pulse methylprednisolone. Clin Exp Immunol 60:465

    PubMed  Google Scholar 

  116. Walport MJ, Ross GD, Mackworth-Young C, Watson JV, Hogg N, Lachmann PJ (1985) Family studies of erythrocyte complement receptor type 1 levels: reduced levels in patients with SLE are acquired, not inherited. Clin Exp Immunol 59:547

    PubMed  Google Scholar 

  117. Walport MJ, Ng YC, Lachmann PJ (1987) Erythrocytes transfused into patients with SLE and haemolytic anemia lose complement receptor type 1 from their cell surface. Clin Exp Immunol 69:501

    PubMed  Google Scholar 

  118. Wilson JG, Wong WW, Schur PH, Fearon DT (1982) Mode of inheritance of decreased C3b receptors on erythrocytes of patients with systemic lupus erythematosus. N Engl J Med 307:981

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  120. Wilson JG, Wong WW, Murphy EE, III, Schur PH, Fearon DT (1987) Deficiency of the C3b/C4b receptor (CR1) of erythrocytes in systemic lupus erythematosus: analysis of the stability of the defect and of a restriction fragment length polymorphism of the CR1 gene. J Immunol 138:2708

    PubMed  Google Scholar 

  121. Wilson WA, Perez MC, Armatis PE (1988) Partial C4A deficiency is associated with susceptibility to systemic lupus erythematosus in black Americans. Arthritis Rheum 31:1171

    PubMed  Google Scholar 

  122. Wong WW, Farrell SA (1991) Proposed structure of the F′ allotype of human CR1. Loss of a C3 binding site may be associated with altered function. J Immunol 146:656

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Rheumatology Unit, Department of Medicine, Hammersmith Hospital, Du Cane Road, W12 ONN, London, UK

    Kevin A. Davies & Mark J. Walport

  2. Hôpital Cantonal Universitaire, 4, Geneva, Switzerland

    Jurg A. Schifferli

Authors
  1. Kevin A. Davies
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jurg A. Schifferli
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Mark J. Walport
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Davies, K.A., Schifferli, J.A. & Walport, M.J. Complement deficiency and immune complex disease. Springer Semin Immunopathol 15, 397–416 (1994). https://doi.org/10.1007/BF01837367

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01837367

Keywords

Search

Navigation