Hypocomplementaemia caused by C3 nephritic factors (C3 NeF): Clinical findings and the coincidence of C3 NeF type II with anti-C1q autoantibodies

Journal of Internal Medicine

Volumn 242, Issue 6, 1997, Pages 455-464

  Skattum, L ^a   Martensson U ^a   Sjoholm A G ^a  

^a LUND UNIVERSITY   (Sweden)

Author keywords

Autoimmunity; Complement 3 nephritic factors; Glomerulonephritis meningococcal infections

Indexed keywords

ALTERNATIVE COMPLEMENT PATHWAY C3 C5 CONVERTASE; AUTOANTIBODY; CLASSICAL COMPLEMENT PATHWAY C3 C5 CONVERTASE; COMPLEMENT COMPONENT C1Q; COMPLEMENT COMPONENT C3; COMPLEMENT COMPONENT C4; COMPLEMENT COMPONENT C5; NEPHRITIC FACTOR; PROPERDIN;

ADOLESCENT; ADULT; ANAPHYLACTOID PURPURA; ANTIBODY SPECIFICITY; ARTICLE; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; DISEASE ASSOCIATION; HUMAN; HYPOCOMPLEMENTEMIA; LIPODYSTROPHY; MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS; MENINGOCOCCOSIS; PRIORITY JOURNAL; SYSTEMIC LUPUS ERYTHEMATOSUS;

EID: 0031455761     PISSN: 09546820     EISSN: None     Source Type: Journal    
DOI: 10.1111/j.1365-2796.1997.tb00018.x     Document Type: Article

References (41)

1. Ross SC, Densen P. Complement deficiency states and infection: Epidemiology, pathogenesis and consequences of neisserial and other infections in an immune deficiency. Medicine 1984; 63: 243-73.
2. Sjöholm AG. Inherited complement deficiency states: implications for immunity and immunological disease. APMIS 1990; 98: 861-74.
3. Figueroa JE, Densen P. Infectious diseases associated with complement deficiencies, Clin Microbiol Rev 1991; 4: 359-95.
4. Davis AE, Ziegler JB, Gelfand EW, Rose FJ, Alper CA. Heterogenity of NeF and its identification as an immunoglobulin. Proc Natl Acad Sci USA 1977; 74: 3980-83.
5. Scott DM. Amos N. Sissons JGP, Lachmann PJ, Peters DK. The immunoglobulin nature of nephritic factor (NeF). Clin Exp Immunol 1978; 32: 12-24.
6. Daha MR, Austen KF, Fearon DT. Heterogeneity, polypeptide chain composition and antigenic reactivity of C3NeF. J Immunol 1978; 120: 1389-94.
7. Daha MR, Fearon DT, Austen KF. C3 nephritic factor (C3NeF): stabilization of fluid phase and cell-bound alternative pathway convertase. J Immunol 1976; 116: 1-7.
8. Weiler JM, Daha MR, Austen KF. Fearon DT. Control of the amplification convertase of complement by the plasma protein β1H. Proc Natl Acad Sci USA 1976; 73: 3268-72.
9. Ohi H, Watanabe S, Fujita T, Yasugi T. Significance of C3 nephritic factor (C3NeF) in non-hypocomplementaemic serum with membranoproliferative glomerulonephritis. Clin Exp Immunol 1992; 89: 479-84.
10. Charlesworth JA, Williams DG, Sherington E, Lachmann PJ, Peters DK. Metabolic studies of the third component of complement and the glycine-rich b glycoprotein in patients with hypocomplementemia. J Clin Invest 1974; 53: 1578-87.
11. Peters DK, Martin A, Weinstein A. Cameron JS. Barratt TM. Ogg CS, Lachmann PJ. Complement studies in membranoproliferative glomerulonephritis. Clin Exp Immunol 1972; 11: 311-20.
12. Sissons JGP, West RJ, Fallows J. Williams DG, Boucher BJ, Amos N, Peters DK. The complement abnormalities of lipodystrophy. N Engl J Med 1976; 294: 461-5.
13. Walport MJ, Davies KA, Botto M, Naughton MA, Isenberg DA, Biasi D et al. C3 nephritis factor and SLE. Report of four cases and review of the literature. QJ Med 1994; 87: 609-15.
14. Karstorp A. C3 activator and hypocomplementia in a 'healthy' woman. Br Med J 1976: i: 501-2.
15. Gewurz AT, Imherr SM, Strauss S, Gewurz H, Mold C. C3 nephritic factor and hypocomplementemia in a clinically healthy individual. Clin Exp Immunol 1983; 54: 253-8.
16. Tedesco F, Tovo PA, Tamaro G, Basaglia M, Perticarari S, Villa MA. Selective C3 deficiency due to C3 nephritic factor in an apparently healthy girl. La Ricerca Clin Lab 1985; 15: 323.
17. Ng YC, Peters DK. C3 nephritic factor (C3NeF): dissociation of cell-bound and fluid phase stabilization of alternative pathway convertase. Clin Exp Immunol 1986; 65: 450-57.
18. Mollnes TE, Ng YC, Peters DK, Lea T. Tschopp J, Harboe M. Effect of nephritic factor on C3 and on the terminal pathway of complement in vivo and in vitro. Clin Exp Immunol 1986; 65: 73-9.
19. Clardy CW, Forristal J, Strife CF, West CD. A properdin dependent nephritic factor slowly activating C3, C5 and C9 in membrano-proliferative glomerulonephritis. types I and III. Clin Immunol Immunopathol 1989; 50: 333-47.
20. Tanuma Y, Ohi H, Hatano M. Two types of C3 nephritic factor: properdin-dependent C3NeF and properdin-independent C3NeF. Clin Immunol Immunopathol 1990; 56: 226-38.
21. Sjöholm AG. Mårtensson U, Sturfelt G. Serial analysis of autoantibody responses to the collagen-like region of C1q, collagen type II, and double-stranded DNA in patients with systemic lupus erythematosus. J Rheumatol 1997; 24: 871-8.
22. Mårtensson U, Sjöholm AG, Sturfelt G, Truedsson L, Laurell A-B. Western blot analysis of human IgG reactive with the collagenous portion of CIq: evidence of distinct binding specificities. Scand J Immunol 1992; 35: 735-44.
23. Strife CF, Leahy AE, West CD. Antibody to a cryptic, solid phase C1q antigen in membranoproliferative nephritis. Kidney Int 1989; 35: 836-42.
24. Strife CF, Prada AL, Clardy CW, Jackson E, Forristal J. Autoantibody to complement neoantigens in membranoproliferative glomerulonephritis. J Pediatr 1990; 116: 98-102.
25. Sturfelt G, Johnson U, Sjöholm AG. Sequential studies of complement activation in systemic lupus erythematosus. Scand J Rheumatol 1985; 14: 184-96.
26. Sjöholm AG. Complement components and complement activation in acute poststreptococcal glomerulonephritis. Int Archs Allergy Appl Immunol 1979; 58: 274-84.
27. Rother U. A new screening test for nephritis factor based on a stable cell bound convertase on sheep erythrocytes. J Immunol Methods 1982; 51: 101-7.
28. López-Trascasa M. Marín MA, Fontán G. Nephritic factor determination. A comparison between two methods. J Immunol Methods 1987; 98: 77-82.
29. Johnson U, Truedsson L, Gustavii B. Complement components in 100 newborns and their mothers determined by electroimmunoassay. Acta Pathal Microbiol Immunol Scand (C) 1983; 91: 147-50.
30. Cooper NR. Laboratory investigation of complement proteins and complement receptors. Baillieres Clin Immunol Allergy 1988; 2: 263-93.
31. Seino J, van der Wall Bake WL, van Es LA, Daha MR. A novel ELISA assay for the detection of C3 nephritic factor. J Immunol Methods 1993; 159: 221-7.
32. Nordin Fredrikson G. Westberg J, Kuijper EJ, Tijssen CC, Sjöholm AG, Uhlén M, Truedsson L. Molecular characterization of properdin deficiency type III: dysfunction due to a single point mutation in exon 9 of the structural gene causing a tyrosine to aspartic acid interchange. J Immunol 1996; 157: 3666-71.
33. Voller A, Bidwell DE, Bartlett A. Enzyme immunoassays in diagnostic medicine. Bull WHO 1976; 53: 55-65.
34. Hogäsen K, Jansen JH, Mollnes TE, Hovdenes J, Harboe M. Hereditary porcine membranoproliferative glomerulonephritis type Il is caused by factor H deficiency. J Clin Invest 1995; 95: 1054-61.
35. Nilsson UR, Nilsson B, Storm K-E, Sjölin-Forsberg G, Hällgren R. Hereditary dysfunction of the third component of complement associated with a systemic lupus erythematosus-like syndrome and meningococcal. Arthritis Rheum 1992; 35: 580-86.
36. Linshaw MA, Stapleton FB, Cuppage FE, Forristal J, West CD, Schreiber RD, Wilson CB. Hypocomplementemic glomerulonephritis in an infant and mother. Am J Nephrol 1987; 7: 470-77.
37. Meri S, Koistinen V, Miettinen A, Törnroth T. Seppälä IJT. Activation of the alternative pathway of complement by monoclonal λ light chains in membranoproliferative glomerulonephritis. J Exp Med 1992; 175: 939-50.
38. Halbwachs L,Leveillé M, Lesavre PH, Wattel S, Leibowitch J. Nephritic factor of the classical pathway of complement. lmmunoglobulin G autoantibody directed against the classical pathway C3 convertase enzyme. J Clin Invest 1980; 65: 249-56.
39. Martin A, Lachmann PJ. Halbwachs L, Hobart MJ. Haemolytic diffusion plate assays for factors B and D of the alternative pathway of complement activation. Immunochemistry 1976; 13: 317-24.
40. Fremeaux-Bacchi V, Weiss L, Brun P, Kazatchkine MD. Selective disappearance of C 3 NeF IgG autoantibody in plasma of a patient with membranoproliferative glomerulonephritis following renal transplantation. Nephrol Dial Transplant 1994; 9: 811-14.
41. Spitzer RE, Stitzel AE, Tsokos GC. Autoantibody to the alternative pathway C3/C5 convertase and its anti-idiotypic response. J Immunol 1992; 148: 137-41.