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Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice
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Complement and glomerular disease: new insights

Matthew Pickering1, H Terence Cook

  • 1Centre for Complement & Inflammation Research, Division of Immunology and Inflammation, Department of Medicine, Imperial College London, Hammersmith Campus, Du Cane Road, London W12 0NN, UK.

Current Opinion in Nephrology and Hypertension
|March 23, 2011
PubMed

Insights

Complement dysregulation is linked to glomerular diseases like C3 glomerulopathy and atypical hemolytic uremic syndrome (aHUS). C5 inhibition shows promise for aHUS, but its role in C3 glomerulopathies requires further study.

Area of Science:

  • Nephrology
  • Immunology
  • Genetics

Background:

  • Complement dysregulation is closely associated with glomerular pathology.
  • C3 glomerulopathy, including dense deposit disease (DDD) and CFHR5 nephropathy, is characterized by isolated C3 deposition.
  • Atypical hemolytic uremic syndrome (aHUS) involves genetic defects in complement regulation.

Purpose of the Study:

  • To summarize recent advancements in understanding complement dysregulation in glomerular diseases.
  • To review the role of C3 and C5 in the pathogenesis of C3 glomerulopathies and aHUS.
  • To discuss therapeutic strategies targeting the complement system.

Main Methods:

  • Review of recent scientific literature on complement-related glomerular diseases.
  • Analysis of genetic and molecular mechanisms underlying C3 glomerulopathy and aHUS.
  • Evaluation of clinical data and animal models for complement-targeted therapies.

Main Results:

  • C3 glomerulopathy is defined by isolated C3 deposition, with DDD and CFHR5 nephropathy as key examples.
  • Systemic C3 dysregulation underlies familial DDD, while CFHR5 plays a role in kidney complement regulation.
  • Animal models show C5 activation is critical in aHUS-related renal thrombotic microangiopathy and pauci-immune glomerulonephritis.

Conclusions:

  • Eculizumab (anti-C5 antibody) is an effective therapy for aHUS.
  • The therapeutic role of C5 inhibition in C3 glomerulopathies remains an important area for future research.
Abstract

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