Abstract
There are known and theoretical defects in the immune response system which might make an individual susceptible to the development of nephritis. Certainly the various deficiencies of immunoglobulins, B and T lymphocytes are well known, and are clinically characterized by recurrent infections. They are not known to be associated with renal diseases, other than perhaps urinary tract infections. Increasing interest has focused lately on inherited, isolated deficiencies of components of the complement system. While first recognized in otherwise normal individuals (reviewed in Jersild et al., 1976; Carpenter et al., 1977; Schur, 1977, 1978; Agnello, 1978), there have recently been numerous reports of their association with disease.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Agnello, V., 1978, Complement dificiency states, Medicine 57:1.
Carpenter, C. B., Raum, D., Glass, D., and Schur, P. H., 1977, Ordering of genes for HLA antigens and complement components on the human sixth chromosome, in: HLA and Malignancy (G. P. Murphy, ed.), pp. 9–20, Liss, New York.
Daniels, C. A., Borsos, T., Rapp, H. J., Synderman, R., and Notkins, A. L., 1969, Neutralization of sensitized virus by purified components of complement, Proc. Natl. Acad. Sci. USA 65:528.
Day, N. K., Geiger, H., McLean, R., Michael, A., and Good, R. A., 1973, C2 deficiency: Development of lupus erythematosus, J. Clin. Invest. 52:1601.
Day, N. K., L’Esperance, P. L. L., Good, R. A., Michael, A. F., Hansen, J. A., Dupont, B., and Jersild, C., 1975, Hereditary C2 deficiency: Genetic studies and association with the HL-A system, J. Exp. Med. 141:1464.
Day, N. K., Rubinstein, P., Case, D., Hansen, J. A., Good, R. A., Walker, M. E., Tulchin, N., Dupont, B., and Jersild, C., 1976, Linkage of gene for C2 deficiency and the major histocompatibility complex (MHC) in man: Family study of a further case, Vox Sang. 31:96.
Einstein, L. P., Alper, C. A., Bloch, K. J., Herrin, J. T., Rosen, F. S., David, J. R., and Colten, H. R., 1975, Biosynthetic defect in monocytes from human beings with genetic deficiency of the second component of complement, N. Engl. J. Med. 292:1169.
Friend, P. S., Handwerger, B. S., Kim, Y., Michael, A. F., and Yunis, E. J., 1975, C2 deficiency in man, genetic relationship to a mixed lymphocyte reaction determinant (7a*), Immunogenetics 2:569.
Fu, S. M., Stern, R., Kunkel, H. G., Dupont, B., Hansen, J. A., Day, N. K., Good, R. A., Jersild, C., and Fotino, M., 1975a, LD-7a association with C2 deficiency in five of six families, in: Histocompatibility Testing (F. Kissmeyer-Nielsen, ed.), pp. 933–936, Munksgaard, Copenhag
Fu, S. M., Stern, R., Kunkel, H. G., Dupont, B., Hansen, J. A., Day, N. K., Good, R. A., Jersild, C., and Fotino, M., 1975b, Mixed lymphocyte culture determinants and C2 deficiency in four families, J. Exp. Med. 142:495.
Gelfand, E. W., Clarkson, J. E., and Minta, J. O., 1975, Selective deficiency of the second component of complement in a patient with anaphylactoid purpura, Clin. Immunol. Immunopathol. 4:269.
Gibson, D. J., Glass, D., Carpenter, C. B., and Schur, P. H., 1976, Hereditary C2 deficiency: Diagnosis and HLA gene complex associations,J. Immunol. 116:1065.
Glass, D., Raum, D., Gibson, D., Stillman, J. S., and Schur, P. H., 1976, Inherited deficiency of the second component of complement: Rheumatic disease associations,J. Clin. Invest. 58:853.
Hauptmann, G., Tongio, M. M., Gross-Wilde, H., and Mayer, S., 1977, Linkage between C2 deficiency and the HLA-A10, B18, Dw2/Bf S haplotype in a French family, Immunogenetics 4:447.
Jersild, C., Rubinstein, P., and Day, N. K., 1976, The HLA system and inherited deficiencies of the complement system, Transplant. Rev. 32:43.
Kim, Y., Friend, P. S., Dresner, I. G., Yunis, E. J., and Michael, A. F., 1977, Inherited deficiency of The second component of complement (C2) with membranoproliferative glomerulonephritis, Am. J. Med. 62:765.
Kohler, P. F., Percy, J., Campion, W. M., and Smyth, C., 1976, Hereditary angioedema and ‘familial’ lupus erythematosus in identical twin boys, Am. J. Med. 56:406.
Mannik, M., Arond, W. P., Hall, A. P., and Gilliland, B. C., 1971, Studies on a n t i g e n-a n t i b o dy complexes. I. Elimination of soluble complexes from rabbit circulation, J. Exp. Med. 133:713.
Ochs, H., Rosenfield, S. I., Thomas, E. D., Giblet, E. R., Alper, C. A., Dupont, B., Schaller, J. G., Gilliland, B. C., Hansen, J. A., and Wedgewood, R. J., 1977, Linkage between the gene or genes controlling synthesis of The fourth component of complement and the major histocompatibility complex, N. Engl. J. Med. 296:470.
Opelz, G., and Glovsky, M. M., 1976, HLA antigen studies in a family with C2 deficiency,J. Immunogenet. 3:303.
Osterland, C. K., Espinoza, L., Parker, L. P., and Schur, P. H., 1975, Inherited C2 deficiency and systemic lupus erythematosus: Studies on a family, Ann. Intern. Med. 82:323.
Pickering, R. J., Michael, A. F., Herdman, R. C., Good, R. A., and Gewurz, H., 1971, The complement system in chronic glomerulonephritis in three newly associated aberrations, J. Pediatr. 78:30.
Raum, D., Glass, D., Carpenter, C. B., Alper, C. A., and Schur, P. H., 1976, The chromosomal o r d e r of genes controlling the major histocompatibility complex, properdin factor B, and deficiency of the second component of complement, J. Clin. Invest. 58:1240.
Rittner, C., Hauptmann, G., Gross-Wilde, H., Grosshans, E., Tongio, M. M., and Mayer, S., 1975, Linkage between HLA (major histocompatibility complex) and genes controlling the synthesis of The fourth component of complement, in: Histocompatibility Testing (F. Kissmeyer-Nielsen, ed.), pp. 945–953, Munksgaard, Copenhagen.
Roberts, J. L., Schwartz, M. M., and Lewis, E. J., 1978, Hereditary C2 deficiency and systemic lupus erythematosus associated with severe glomerulonephritis, Clin. Exp. Immunol. 31:328.
Schur, P. H., 1977, Complement testing in the diagnosis of immune and autoimmune diseases, Am. J. Clin. Pathol. 68:647.
Schur, P. H., 1978, Genetics of complement deficiencies associated with lupus-like syndromes, Arthritis Rheum. 21:5153.
Sissons, J. G. P., West, R. J., Fallow, J., Williams, D. G., Boucher, B. J., Amos, N., and Peters, D. K., 1976, The complement abnormalities of lipodystrophy, N. Engl. J. Med. 294:461.
Stratton, F., 1974, cited by Lachmann, P. J., Genetic deficiencies of the complement system, Boll. 1st. Sieroter. Milan. 53(Suppl. I): 195.
Wild, J. H., Zvaifler, N. J., Mauuller-Eberhard, H. J., and Wilson, C. B., 1976, C3 metabolism in a patient with deficiency of The second component of complement (C2) and discoid lupus erythematosus, Clin. Exp. Immunol. 24:238.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1983 Springer-Verlag US
About this chapter
Cite this chapter
Schur, P.H. (1983). Genetic Defects of the Complement Pathways: Relationship to HLA and Disease. In: Cummings, N.B., Michael, A.F., Wilson, C.B. (eds) Immune Mechanisms in Renal Disease. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-4625-8_35
Download citation
DOI: https://doi.org/10.1007/978-1-4613-4625-8_35
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4613-4627-2
Online ISBN: 978-1-4613-4625-8
eBook Packages: Springer Book Archive