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C3 glomerulopathy: a new classification

Nature Reviews Nephrology volume 6, pages 494–499 (2010)Cite this article

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Abstract

Several distinct pathological patterns of glomerular inflammation are associated with abnormal regulation of the complement system, specifically, with dysregulation of the alternative pathway of the complement system. However, these conditions share the pathological finding of complement C3 (C3) deposited within the glomerulus in the absence of substantial immunoglobulin. This finding has alerted us and others to the possible presence of genetic and acquired complement dysregulation in individual patients. This article summarizes our current understanding of the relationship between dysregulation of the complement system and glomerular inflammation. Here, we suggest that glomerular pathologies that are characterized by the isolated deposition of C3 could usefully be classified by the term C3 glomerulopathy. In our view, this classification would alert the pathologist and nephrologist to the importance of screening for acquired and genetic abnormalities in complement regulation. In the future, it could help to identify individuals who might benefit from therapeutic inhibition of the complement system.

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Figure 1: Examples of renal pathology in dense deposit disease.
Figure 2: Examples of renal pathology in C3 glomerulonephritis.
Figure 3: Hypothetical mechanisms through which dysregulation of the complement system results in MPGN1.
Figure 4: Pathogenesis of dense deposit disease.

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Authors and Affiliations

  1. Département de Nephrologie, INSERM UMR643, CHU de Nantes, 1 place Alexis-Ricordeau, Nantes, 44000, France

    Fadi Fakhouri

  2. Service d'Immunologie Biologique, Hôpital Européen Georges Pompidou, 14 rue Leblanc, Paris, 75015, France

    Véronique Frémeaux-Bacchi

  3. INSERM U845, Hôpital Necker, 149 rue de Sèvres, Paris, 75015, France

    Laure-Hélène Noël

  4. Division of Immunology and Inflammation, Centre for Complement and Inflammation Research (CCIR), Faculty of Medicine, Imperial College, Hammersmith Hospital Campus, Du Cane Road, London, W12 0NN, UK

    H. Terence Cook & Matthew C. Pickering

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Correspondence to Matthew C. Pickering.

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Fakhouri, F., Frémeaux-Bacchi, V., Noël, LH. et al. C3 glomerulopathy: a new classification. Nat Rev Nephrol 6, 494–499 (2010). https://doi.org/10.1038/nrneph.2010.85

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