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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 J+Clin+Immunol
2016 ; 36
(5
): 517-27
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Testing the Activity of Complement Convertases in Serum/Plasma for Diagnosis of
C4NeF-Mediated C3 Glomerulonephritis
#MMPMID27146825
Blom AM
; Corvillo F
; Magda M
; Stasi?oj? G
; Nozal P
; Pérez-Valdivia MÁ
; Cabello-Chaves V
; Rodríguez de Córdoba S
; López-Trascasa M
; Okrój M
J Clin Immunol
2016[Jul]; 36
(5
): 517-27
PMID27146825
show ga
Autoantibodies termed C3-nephritic factor (C3NeF), which stabilize convertases of
the alternative complement pathway, often stimulate autoinflammatory diseases.
However, knowledge about analogous autoantibodies acting on the classical pathway
(C4NeF) is limited to a few reports, which indicate association with kidney
dysfunction, systemic lupus erythematous, and infections. C4NeF may appear
independently from C3NeF, but the lack of a routine diagnostic method predisposes
C4NeF for being an underestimated player in autoinflammatory episodes. We tested
the activity of classical convertases directly in serum/plasma to screen samples
from 13 patients with C3 glomerulopathies and identified one patient showing
significantly prolonged half-life of these enzymes. Observed effect was
reproduced by immunoglobulins purified from patient's plasma and additionally
confirmed on classical convertase built from purified components. Isolated
immunoglobulins protected classical convertases from both spontaneous and
inhibitor-driven decay but not from C4b proteolysis. The patient had a decreased
serum level of C3, elevated sC5b-9, and normal concentrations of factor B and C4.
Neither C3NeF nor other autoantibodies directed against alternative pathway
proteins (factor H, factor B, factor I, C3, and properdin) were found. Genetic
analysis showed no mutations in C3, CFB, CFH, CFI, MCP, THBD, and DGKE genes.
Renal biopsy revealed a membranoproliferative pattern with intense C3 deposits.
Our results underline the importance of C4NeF as an independent pathogenic factor
and a need for the implementation of routine examination of classical convertase
activity. Proposed method may enable robust inspection of such atypical cases.