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Results for “Complement C3 Nephritic Factor”

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At least 145 records · Page 8Linked to original sources
PubMed1994

Selective disappearance of C3NeF IgG autoantibody in the plasma of a patient with membranoproliferative glomerulonephritis following renal transplantation.

The serum of patients with membranoproliferative glomerulonephritis (MPGN) often exhibits C3 nephritic factor activity (C3NeF) associated with autoantibodies directed against the C3bBb alternative complement pathway C3 convertase. In the present study, we have sequentially assessed C3NeF activity in the purified IgG fraction of the serum of a patient with MPGN type II and end-stage renal disease every month for 1 year following renal transplantation and bilateral nephrectomy. C3NeF activity in the patient's purified serum IgG decreased with time from the first month after transplantation and became undetectable after forty-five weeks. At that time, plasma levels of C3 and factor B had returned to normal values. The disappearance of C3NeF activity in patient's IgG was selective in that no change in activity of two other autoantibodies and of one antibody against a non-self antigen was observed with time, indicating that the decrease in C3NeF activity was not related to a non-specific effect of immunosuppressive therapy. Loss of C3NeF activity was not related to the generation of anti-idiotypic antibodies against C3NeF. This study documents for the first time the selective disappearance of C3NeF activity following renal transplantation and bilateral nephrectomy. The results suggest that the autoantibody represents an antigen-driven expansion of self reactive B cell clones in response to a specific disease process in the kidney of patients with MPGN.

Adult↗
PubMed1987

Novel C3 nephritic factor activity in the glomerulonephritis of staphylococcal endocarditis.

A case of glomerulonephritis complicating staphylococcal endocarditis is presented. Hypocomplementaemia and a C3-activating factor in the serum suggested that the patient might have mesangiocapillary glomerulonephritis in association with C3-nephritic factor. Renal biopsy showed that this was not so and further examination of the serum factor showed that it differed from classical C3-nephritic factor because it was not an immunoglobulin. It is postulated that complement activation and glomerulonephritis in staphylococcal endocarditis may be the direct result of a bacterial product. A substance in the serum which activates C3 should be confirmed to be an immunoglobulin before the presence of classical C3-nephritic factor is assumed.

Complement Activation↗
PubMed1982

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.

Autoantibodies↗
PubMed1988

Screening for complement deficiencies in patients surviving from epidemic meningococcal disease.

Sera from 132 patients surviving from meningococcal disease during the high-endemic period 1 January 1980 to 31 December 1984 on the Faroe Islands were screened for deficiencies of the complement (C) system by measuring hemolytic complement function in serum. Samples from 12 patients with reduced C function were further investigated by immunochemical quantification of individual C proteins. One patients was identified with C3-deficiency (C3-concentration 6% of normal) due to the presence of C3 nephritic factor (C3NeF) in serum. In addition, eight patients had minor aberrations in one or several complement proteins. It is concluded, that C deficiency is not an important risk factor during epidemics of meningococcal disease. The C3NeF activity could not be absorbed with Neisseria meningitidis group B, type 15, indicating absence of crossreactivity between neisserial antigens and C3NeF.

Complement C3 Nephritic Factor↗
PubMed1990

[Chronic urticaria and acquired complement deficiency due to a nephritic factor (C3NeF)].

Several studies have suggested that complement activation processes are frequently involved in the pathogenesis of urticaria. We report clinical evolution and studies of complement-mediated functions in a 47-year-old previously described patient presenting with chronic urticaria, in whom we found persistent low complement hemolytic activity (65-75% of normal values), depressed levels of third complement component (C3, between 55% and 65%) and of factor B (between 60% and 75%), together with C1, C4, C2, C5, C5b neoantigen and fluid phase terminal complement (SC5b-9) complex within the normal range, pointing to activation of the alternative pathway. A circulating low affinity C3 nephritic factor (C3NeF), known to enhance cleavage of human serum C3, was detected. The urticarial lesions, which were initially pruritic and persisted for less than 24 hours, became subsequently fixed and burning, and were accompanied by fever and arthralgia. Skin biopsy specimens showed moderate leukocytoclastic vasculitis. Response to varied treatment regimens, including antihistamines and colchicine, was poor. Therapy with oral corticosteroids produced some improvement. The association of chronic urticaria with C3NeF without clinical and biological signs of membranoproliferative glomerulonephritis and partial lipodystrophy has not to our knowledge been reported before. This observation raises the question of a possible role of C3NeF in the pathogenesis of urticaria.

Chronic Disease↗
PubMed1979

Further evidence for the antibody nature of C3 nephritic factor (C3NeF).

C3 nephritic factor (C3NeF), found in the sera of some patients with membranoproliferative glomerulonephritis, has been shown to be composed of two heavy and two light chains, like IgG; in addition it shares antigenic determinants with IgG. Purified C3NeF binds to the amplification convertase of complement, C3b,Bb, and thereby prevents decay of its C3-cleaving potential. The capability of C3NeF to bind to C3b,Bb was used as a means for purifying C3NeF to homogeneity. The investigation described in this report suggests that binding of C3NeF to C3b,Bb occurs via the Fab portion of the molecule. Pepsin treatment of eight C3NeF preparations resulted in an average loss of 76% of C3NeF functional activity. Papain treatment induced a loss of approximately 90%. The decrease in functional activity could be attributed to the accelerated rate of dissociation of 125I-F(ab')2 and 125I-Fab fragments from stabilized cell-bound C3b,Bb. The dissociation rate of 125I-F(ab')2 from C3b,Bb was comparable with the decay of the functional activity of C3b,Bb stabilized by F(ab')2 or Fab fragments of C3NeF. Although these results suggest that the stabilizing activity of C3NeF is mediated by the Fab portion of the molecule, it was found that the Fc portion also contributes to its functional activity.

Antibodies↗
PubMed1979

Renal transplantation for patients with type I and type II membranoproliferative glomerulonephritis: serial complement and nephritic factor measurements and the problem of recurrence of disease.

Fourteen patients with membranoproliferative glomerulonephritis as their original kidney disease received 16 renal allografts. All 14 patients are alive, 11 currently have functioning allografts, and one graft was lost to recurrence of membranoproliferative glomerulonephritis. Originally depressed serum complement (C3) concentrations returned to normal soon after transplantation in those patients with no clinical evidence of recurrence. Two patients with type II membranoproliferative glomerulonephritis had recurrence of disease. Nephritic factor (C3NeF) was high in both these patients before they received their transplants and was absent soon thereafter. However, abnormally high levels were again detected in their course. The one recurrence of type I membranoproliferative glomerulonephritis was associated with depressed C3, Clq, C4 and factor B but without C3NeF activity. Despite warnings of "high risks/ and "high mortality" associated with renal transplants in patients with membranoproliferative glomerulonephritis, we, because of these results and a review of the literature, continue to recommend renal transplants from both living related (LRD) and cadaver (CAD) donors in otherwise suitable patients who have renal failure due to membranoproliferative glomerulonephritis.

Adolescent↗
PubMed1998

Partial lipodystrophy, mesangiocapillary glomerulonephritis, and complement dysregulation. An autoimmune phenomenon.

Partial lypodistrophy (PLD) is a rare disease in which, there is loss of fat usually from the upper part of the body. The disease is frequently associated with mesangiocapillary (membranoproliferative) glomerulonephritis Type II (MCGN II). In the early 1970s, it was noticed that MCGN II and/or PLD was sometimes associated with dysfunction of the complement system as reported in several case descriptions and studies. Subsequently, an IgG autoantibody was detected-C3 nephritic factor (C3NeF). The target of this autoantibody is the alternative pathway C3 convertase-C3bBb. There are sporadic case reports that linked PLD, MCGNII, and C3NeF with autoimmune diseases. This association may be more than a coincidence. The complement deficiency may lead to perturbation of the immune system, which may trigger some of the autoimmune diseases. This article will be focused on the association among PLD, MCGN II and C3NeF.

Autoimmune Diseases↗