Occurrence of C3 nephritic factor and C4 nephritic factor in membranoproliferative glomerulonephritis (MPGN)

Ohi, Hiroyuki; Yasugi, Tadao

doi:10.1111/j.1365-2249.1994.tb06530.x

Cited by 51 publications

(49 citation statements)

References 20 publications

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“…The few laboratories routinely measuring C3Nef use different methods, predominantly hemolytic assays, and occassionally ELISA and surface plasmon resonance assay that detect various groups of C3Nefs [22,[33][34][35][36][37]. Results of the various measurements can also be influenced by the presence of C4 nephritic factor (C4Nef), anti-C1q autoantibodies or mutations in factor H 8 or factor I [10,[38][39][40]. Recent data suggest that a combination of different assays are likely to identify most C3Nefs, including occult cases [11,22,37].…”

Section: C3 Nephritic Factormentioning

confidence: 99%

“…in patients with glomerulonephritis [38,39]. Although the only study analyzing a cohort of 9 together with C3Nef [39], screening for C4Nef is currently not part of the routine diagnostics.…”

Section: C4 Nephritic Factormentioning

confidence: 99%

“…C4Nef can be measured by hemolytic assay or ELISA [39,47]. Problems with the specificity and reliability of the assay are similar to the C3Nef assay, as discussed above.…”

Section: C4 Nephritic Factormentioning

confidence: 99%

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Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome

et al. 2014

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The alternative pathway of complement is implicated in the pathogenesis of several renal diseases, such as atypical hemolytic uremic syndrome, dense deposit disease and other forms of C3 glomerulopathy. The underlying complement defects include genetic and/or acquired factors, the latter in the form of autoantibodies. Because the autoimmune forms require a specific treatment, in part different from that of the genetic forms, it is important to detect the autoantibodies as soon as possible and understand their characteristics. In this overview, we summarize the types of anti-complement autoantibodies detected in such diseases, i.e. autoantibodies to factor H, factor I, C3b, factor B and those against the C3 convertases (C3 nephritic factor and C4 nephritic factor). We draw attention to newly described autoantibodies and their characteristics, and highlight similarities and differences in the autoimmune forms of these diseases.

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Section: C3 Nephritic Factormentioning

confidence: 99%

Section: C4 Nephritic Factormentioning

confidence: 99%

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Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome

et al. 2014

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“…In patients with C3GN, paraffin immunofluorescence after protease digestion is strongly recommended to look for masked Igs (32). If negative, serum should be tested for C3 nephritic factor (C3nef) and C4 nephritic factor, which are autoantibodies that protect the alternative pathway C3 convertase C3bBb (C3nef) or the classic C3 convertase C4b2a (C4 nephritic factor), causing persistent activation of the complement system (50). The alternative pathway could also be activated by monoclonal proteins acting as autoantibodies against complement inhibitors, such as factor H (29,31).…”

Section: How Are Mgrs-associated Nephropathies Diagnosed?mentioning

confidence: 99%

A Patient with Abnormal Kidney Function and a Monoclonal Light Chain in the Urine

Leung

Nasr

2016

CJASN

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Monoclonal gammopathy is increasingly recognized as a cause of kidney injury. These renal conditions behave differently than ones without monoclonal gammopathy and require specific treatment. To avoid misdiagnosis, testing for paraprotein should be performed in addition to vasculitis and autoimmune diseases serologies in adults with unexplained AKI or proteinuria. Because the prevalence of monoclonal gammopathy is much more common than glomerular diseases, the nephrotoxicity of the monoclonal protein must be confirmed before cytotoxic therapy is initiated. This can only be done by a kidney biopsy. After a monoclonal gammopathy of renal significant is verified, the evaluation should then focus on the identification of the pathologic clone, because therapy is clone specific. We present this patient to illustrate the clinical presentation of a patient with renal dysfunction and a monoclonal gammopathy. This patient is also used to discuss the diagnostic process in detail when monoclonal gammopathy-associated renal disease is suspected.

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“…The detection of C3 NeF may be performed in several ways [82] . Further studies are needed to better correlate the presence of C3 NeF with the cause of the diseases and with treatment efficacy; (2) C4 NeF: The role of C4 NeF is still unclear, even if this autoantibody has been found in some patients affected by MPGN [83] ; (3)Antifactor H autoantibodies: Have been described …”

mentioning

confidence: 99%

Reclassification of membranoproliferative glomerulonephritis: Identification of a new GN: C3GN

Salvadori¹,

Rosso²

2016

WJN

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This review revises the reclassification of the membranoproliferative glomerulonephritis (MPGN) after the consensus conference that by 2015 reclassified all the glomerulonephritis basing on etiology and pathogenesis, instead of the histomorphological aspects. After reclassification, two types of MPGN are to date recognized: The immunocomplexes mediated MPGN and the complement mediated MPGN. The latter type is more extensively described in the review either because several of these entities are completely new or because the improved knowledge of the complement cascade allowed for new diagnostic and therapeutic approaches. Overall the complement mediated MPGN are related to acquired or genetic cause. The presence of circulating auto antibodies is the principal acquired cause. Genetic wide association studies and family studies allowed to recognize genetic mutations of different types as causes of the complement dysregulation. The complement cascade is a complex phenomenon and activating factors and regulating factors should be distinguished. Genetic mutations causing abnormalities either in activating or in regulating factors have been described. The diagnosis of the complement mediated MPGN requires a complete study of all these different complement factors. As a consequence, new therapeutic approaches are becoming available. Indeed, in addition to a nonspecific treatment and to the immunosuppression that has the aim to block the auto antibodies production, the specific inhibition of complement activation is relatively new and may act either blocking the C5 convertase or the C3 convertase. The drugs acting on C3 convertase are still in different phases of clinical development and might represent drugs for the future. Overall the authors consider that one of the principal problems in finding new types of drugs are both the rarity of the disease and the consequent poor interest in the marketing and the lack of large international cooperative studies.

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Occurrence of C3 nephritic factor and C4 nephritic factor in membranoproliferative glomerulonephritis (MPGN)

Cited by 51 publications

References 20 publications

Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome

Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome

A Patient with Abnormal Kidney Function and a Monoclonal Light Chain in the Urine

Reclassification of membranoproliferative glomerulonephritis: Identification of a new GN: C3GN

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