Complement and Mesangiocapillary Glomerulonephritis Role of Complement Deficiency in the Pathogenesis of Nephritis

112 cases of MPGN, whose diagnosis was made on light microscopy, were reviewed. Histological examination showed 66 cases of ‘classical’ MPGN, 33 of MPGN with a lobular pattern and 13 of MPGN with epithelial crescents. In 11 patients dense intramembranous deposits were observed. On immunofluorescence (95 cases) 62 patients showed deposits of C3 together with immunoglobulins, 20 had a predominant deposition of C3 and in 13 C3 alone was present. At the moment of biopsy 57 patients had nephrotic syndrome, 43 hypertension, 43 impaired renal function and 65 hypocomplementaemia. In 23 cases, one or more episodes of macroscopic haematuria occurred. The actuarial survival was 70% after 10 years and 50% after 20 years from onset. At last observation 25 patients were dead or on haemodialysis, 22 had impaired renal function, 62 had normal renal function and 2 were in complete remission. The histological variety with epithelial crescents had a significantly worse outcome. The presence of dense deposits or of any specific immunofluorescence pattern had no prognostic significance. Nephrotic syndrome, renal function impairment and hypertension indicated a poor prognosis : however, macroscopic haematuria or hypocomplementaemia did not influence the outcome.

Section: Introductionmentioning

confidence: 99%

The Prognostic Value of Some Clinical and Histological Parameters in Membranoproliferative Glomerulonephritis (MPGN)

Belgiojoso

Tarantino

Colasanti

et al. 1977

“…Many authors have suggested that in this glomerulonephritis there is a strong evidence of an involvement of the alternate pathway [3,17].…”

Section: Discussionmentioning

confidence: 79%

“…This chronic nephritis occurs with greater frequency in children than in adults and is often accompanied by hypocomplementemia [2,13,21], The complement profile in the 'classical MPGN' and in 'lobular glomerulonephritis', which probably represents the same clinicopathological entity [5], often gives evidence of an alternate path way mechanism, activated by a circulating factor designated Cs nephritic factor (C;. NeF) [20,17], Many previous studies concern pediatric patients and only a few publi cations describe investigations in adults [11,8], The present study was under- …”

mentioning

confidence: 99%

Membranoproliferative Glomerulonephritis: Correlations between Immunological and Histological Findings

Zucchelli¹,

M²,

Cagnoli³

et al. 1976

34 adult patients with membranoproliferative glomerulonephritis are reviewed; the diagnosis was based on histological studies, by light and electron microscopy. Hypocomplementemia was detected in 67.6% of the cases; the C₃ depression was associated with normal early complement components (82.5%) and low levels of C₃ PA (65.2%). In the serum of ten hypocomplementemic patients the presence of circulating factor, which is able to break down the normal C₃in vitro, was found. Immunofluorescence deposits of C₃ were present in 100% of the cases, IgM in 92, IgG in 86, IgA in 30, C_1q in 53 and C₄ in 46% of the cases. Only in a few cases there was a correlation between serum complement profile and the immunofluorescence pattern. Hypocomplementemic patients with ‘classical’ pathway activation seemed to have a worse clinical course than the others. The results of humoral and cellular immunity studies do not permit to draw any clear conclusion.

“…In addition, complement may reduce the capacity of immune complexes to lodge on and damage vascular endothelium or cell membranes, by its recently described ability to solubilize them and thus alter their configuration [9], Genetically determined deficiencies of individual com plement components have been described and some of these are associated with the development of immune complex disease, including glomerulonephritis [10][11][12]. These family studies support the hypothesis that a bal anced, functional complement system is an essential part of normal immunity.…”

Section: Discussionmentioning

confidence: 99%

Complement and Glomerular Disease – A Natural History Study

Np¹,

Eyres²,

Ej³

et al. 1981

Over a 5-year period we have performed sequential measurements of a range of complement components in 127 patients. Each had a well-characterised glomerular lesion and there was no evidence of an underlying connective tissue disorder. In 17 patients with varied histopathology, who did not have C3 nephritic factor, there was a persisting complement defect which was present during remission in the patients we were able to study. The finding of such defects is consistent with the thesis that primary complement system abnormalities predispose to the development of glomerular lesions. Interestingly, these abnormalities did not influence the prognosis of our patients. In 12 other cases without C3 nephritic factor, complement levels were below the normal range when the glomerular lesion was active but returned to it in remission; these were secondary changes. We showed by discriminant analysis that some circulating complement component levels, assessed in relation to each other and without reference to a statisticallly derived ‘normal’ range, discriminated between histological subgroups and had prognostic significance as well. These patterns could not be distinguished until the data were stored and analysed by computer.