Biological significance of the C3 nephritic factor in membranoproliferative glomerulonephritis.
Serum levels of the C3 nephritic factor (C3NeF), an IgG autoantibody directed against the C3bBb convertase of the alternative complement pathway, and of eight complement components (C1q, C4, C3, C3d, C5, C9, fB and properdin) were measured in 109 serum samples from 27 patients with idiopathic membranoproliferative glomerulonephritis (MPGN) (type I, 20 cases, and type II, 7 cases) and 14 patients with secondary MPGN. Correlations between the concentrations of C3NeF, serum complement levels and progression of the renal damage were studied during the course of the disease in 14 patients with C3NeF activity. The results showed that (1) C3NeF activity was more frequent in patients with type II MPGN than in patients with type I disease; nevertheless there was a high incidence of this splitting activity in patients with secondary MPGN, (2) high levels of the complement components were present in patients with MPGN, (3) low levels of C3 occurred frequently in type II disease and in secondary MPGN, (4) there was no correlation between C3, fB and C3NeF levels, (5) the presence of C3NeF was associated with a more rapid deterioration of renal function. Longitudinal studies showed that serum levels of C3NeF were not satisfactory for monitoring the clinical course of the illness and, in this respect, are similar to the levels of other autoantibodies in patients with autoimmune disease. As MPGN is a clinical syndrome with various pathogeneses, we suggest that the autoantibody, C3NeF, should be considered only as a marker of some forms of MPGN.
D001323:Autoantibodies / Q000032:analysis; D003176:Complement C3 / Q000378:metabolism; D003178:Complement C3 Nephritic Factor / Q000097:blood*; D003169:Complement Inactivator Proteins / Q000097:blood*; D003165:Complement System Proteins / Q000378:metabolism; D005921:Glomerulonephritis / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007677:Kidney Function Tests; D012044:Regression Analysis
D001323:Autoantibodies; D003176:Complement C3; D003178:Complement C3 Nephritic Factor; D003169:Complement Inactivator Proteins; D007074:Immunoglobulin G; D003165:Complement System Proteins