C4A deficiency and elevated level of immune complexes: The mechanism behind increased susceptibility to systemic lupus erythematosus

Journal of Rheumatology

Volumn 29, Issue 11, 2002, Pages 2359-2366

  Traustadottir, Kristín H ^a   Sigfusson, Asbjörn ^a   Steinsson, Kristjan ^a   Erlendsson, Kristjan ^a  

^a LANDSPITALI UNIVERSITY HOSPITAL   (Iceland)

Author keywords

C4AQ0; Complement; Immune complex diseases; Immune complex handling; Systemic lupus erythematosus

Indexed keywords

COMPLEMENT COMPONENT C3; COMPLEMENT COMPONENT C4; COMPLEMENT COMPONENT C4A;

ARTICLE; CLINICAL ARTICLE; COMPLEMENT DEFICIENCY; CONTROLLED STUDY; DISEASE ACTIVITY; DISEASE PREDISPOSITION; ENZYME LINKED IMMUNOSORBENT ASSAY; ERYTHROCYTE; HUMAN; HUMAN CELL; IMMUNE COMPLEX DISEASE; OPSONIZATION; PATHOGENESIS; PRIORITY JOURNAL; SERUM; SYSTEMIC LUPUS ERYTHEMATOSUS;

ANTIGEN-ANTIBODY COMPLEX; COMPLEMENT C3; COMPLEMENT C4A; ERYTHROCYTES; HETEROZYGOTE; HUMANS; IMMUNOGLOBULIN G; LUPUS ERYTHEMATOSUS, SYSTEMIC; PROTEIN BINDING;

EID: 0036842157     PISSN: 0315162X     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (40)

1. Wilson WA, Perez MC, Armatis PE. Partial C4A deficiency is associated with susceptibility to systemic lupus erythematosus in black Americans. Arthritis Rheum 1988;31:1171-5.
2. Christiansen FTZ. Major histocompatibility complex (MHC) complement deficiency, ancestral haplotypes and systemic lupus erythematosus: C4 deficiency explains some but not all of the influence of the MHC. J Rheumatol 1991;18:1350-8.
3. Steinsson K, Arnason A, Erlendsson K, et al. A study of the major histocompatibility complex in a Caucasian family with multiple cases of systemic lupus erythematosus: Association with the C4AQ0 phenotype. J Rheumatol 1995;22:1862-6.
4. Dragon-Durey MA, Rougier N, Clauvel JP, et al. Lack of evidence of a specific role for C4A gene deficiency in determining disease susceptibility among C4-deficient patients with systemic lupus erythematosus. Clin Exp Immunol 2001;123:133-9.
5. Dunckley H, Gatenby PA, Hawkins B, et al. Deficiency of C4A is a genetic determinant of systemic lupus erythematosus in three ethnic groups. J Immunogenet 1987;14:209-18.
6. Steinsson K, Jonsdottir S, Arason GJ, et al. A study of the association of HLA DR, DQ, and complement C4 alleles with systemic lupus erythematosus in Iceland. Ann Rheum Dis 1998;57:503-5.
7. Kristjansdottir H, Bjarnadottir K, Hjalmarsdottir IB, et al. A study of C4AQ0 and MHC haplotypes in Icelandic multicase families with systemic lupus erythematosus. J Rheumatol 2000;27:2590-6.
8. Kishore N, Shah D, Skanes VM, Levine RP. The fluid phase binding of human C4 and its genetic variants, C4A3 and C4B1, to immunoglobulins. Mol Immunol 1988;25:811-9.
9. Schifferli JA, Steiger G, Paccaud J, et al. Difference in the biological properties of the two forms of the fourth component of human complement (C4). Clin Exp Immunol 1986;63:473-7.
10. Schifferli JA, Paccaud JP. Two isotypes of human C4, C4A and C4B, have different structure and function. Complement Inflamm 1989;6:19-26.
11. Law SKA, Dodds AW, Porter RR. A comparison of the properties of two classes, C4A and C4B, of the human complement C4. EMBO J 1984;3:1819-23.
12. Gatenby PA. Barbosa JE, Lachmann PJ. Differences between C4A and C4B in the handling of immune complexes: The enhancement of CR1 binding is more important than the inhibition of immunoprecipitation. Clin Exp Immunol 1990;79:158-63.
13. Reilly BD, Mold C. Quantitative analysis of C4Ab and C4Bb binding to the C3b/C4b receptor (CR1, CD35). Clin Exp Immunol 1997;110:310-6.
14. Finco O, Li S, Cuccia M, et al. Structural differences between the two human complement C4 isotypes affect the humoral immune response. J Exp Med 1992;175:537-43.
15. Prodeus AP, Goerg S, Shen LM, et al. A critical role for complement in maintenance of self-tolerance. Immunity 1998;9:721-31.
16. Schifferli JA, Ng YC, Peters DK. The role of complement and its receptor in the elimination of immune complexes. N Engl J Med 1986;315:488-95.
17. Carroll MC. The role of complement and complement receptors in induction and regulation of immunity. Annu Rev Immunol 1998;16:545-68.
18. Traustadottir KH, Steinsson K, Erlendsson K. C4AQ0 superimposed on a primary defect increases the susceptibility to systemic lupus erythematosus (SLE) in a family with association between C4AQ0 and SLE. J Rheumatol 1998;25:2118-25.
19. Tan EM, Cohen A, Fries J, et al. The revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982;25:1271-7.
20. Milford-Ward A. Proteins in plasma and body fluids. Electroimmunodiffusion. In: Thompson RA, editor. Techniques in Clinical Immunology. London: Blackwell Scientific; 1981:9.
21. Sim E, Cross SJ. Phenotyping of human complement component C4, a class-III HLA antigen. Biochem J 1986;239:763-7.
22. Moulds JM, Arnett FC, Giles CG, Hamilton RG. A novel immunoassay for the quantitation of human C4 gene products. Complement Inflamm 1990;7:95-101.
23. Moulds JM, Warner NB, Arnett FC. Complement component C4A and C4B levels in systemic lupus erythematosus: Quantitation in relation to C4 null status and disease activity. J Rheumatol 1993;20:443-7.
24. Rafnar BO, Traustadottir KH, Sigfusson A, et al. An enzyme based assay for the measurement of complement mediated binding of immune complexes to red blood cells. J Immunol Methods 1998;211:171-81.
25. Brandslund I, Siersted HC. Jensenius JC, Svehag SE. Detection and quantitation of immune complexes with a rapid polyethylene glycol precipitation complement consumption method (PEG-CC). Methods Enzymol 1981;74:551-71.
26. Arason GJ, D'Ambrogio MS, Vikingsdottir T, et al. Enzyme immunoassays for measuring complement-dependent prevention of immune precipitation and solubilization of preformed antigen-antibody complexes. J Immunol Methods 1999;223:37-46.
27. Lachmann PJ. Complement deficiency and the pathogenesis of autoimmune immune complex disease. Chem Immunol 1990;49:245-63.
28. Davies KA, Schifferli JA, Walport MJ. Complement deficiency and immune complex disease. Springer Semin Immunopathol 1994;15:397-416.
29. Davies KA, Peters AM, Beynon HL, Walport MJ. Immune complex processing in patients with systemic lupus erythematosus. In vivo imaging and clearance studies. J Clin Invest 1992;90:2075-83.
30. Davies KA, Erlendsson K. Beynon HL, et al. Splenic uptake of immune complexes in man is complement-dependent. J Immunol 1993;151:3866-73.
31. Walport MJ, Davies KA. Morley BJ, Botto M. Complement deficiency and autoimmunity. Ann NY Acad Sci 1997;815:267-81.
32. Klickstein LB, Barbashov SF, Liu T, et al. Complement receptor type 1 (CR1, CD35) is a receptor for C1q. Immunity 1997;7:345-55.
33. Tas SW, Klickstein LB, Barbashov SE, Nicholson-Weller A. C1q and C4b bind simultaneously to CR1 and additively support erythrocyte adhesion. J Immunol 1999;163:5056-63.
34. Schifferli JA, Peters DK. Complement, the immune-complex lattice, and the pathophysiology of complement-deficiency syndromes. Lancet 1983;2:957-9.
35. Whaley K, Ahmed AE. Control of immune complexes by the classical pathway. Behring Inst Mitt 1989;84:111-20.
36. Welch TR, Beischel L, Berry A, et al. The effect of null C4 alleles on complement function. Clin Immunol Immunopathol 1985;34:316-25.
37. Erlendsson K, Traustadóttir K, Freysdóttir J, Steinsson K, Jónsdóttir I, Valdimarson H. Reciprocal changes in complement activity and immune-complex levels during plasma infusion in a C2-deficient SLE patient. Lupus 1993;2:161-5.
38. Drake CG, Kotzin BL. Genetic and immunological mechanisms in the pathogenesis of systemic lupus erythematosus. Curr Opin Immunol 1992;4:733-40.
39. Briggs D, Stephens C, Vaughan R, et al. A molecular and serologic analysis of the major histocompatibility complex and complement component C4 in systemic sclerosis. Arthritis Rheum 1993;36:943-54.
40. Moriuchi J, Ichikawa Y, Takaya M, et al. Association of the complement allele C4AQ0 with primary Sjogren's syndrome in Japanese patients. Arthritis Rheum 1991;34:224-7.