• Figure 1.

    Alternative complement pathway dysregulation in C3 glomerulopathy

  • 1.

    Iatropoulos P, et al.. Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome. Mol Immunol 2016; 71:131142. doi: 10.1016/j.molimm.2016.01.010

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2.

    Smith RJH, et al.. C3 glomerulopathy—understanding a rare complement-driven renal disease. Nat Rev Nephrol 2019; 15:129143. doi: 10.1038/s41581-018-0107-2

  • 3.

    Servais A, et al.. Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int 2012; 82:454464. doi: 10.1038/ki.2012.63

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4.

    Angelo JR, et al.. Allograft failure in kidney transplant recipients with membranoproliferative glomerulonephritis. Am J Kidney Dis 2011; 57:291299. doi: 10.1053/j.ajkd.2010.09.021

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5.

    Smith RJ, et al.. New approaches to the treatment of dense deposit disease. J Am Soc Nephrol 2007; 18:24472456. doi: 10.1681/ASN.2007030356

  • 6.

    Fakhouri F, et al.. Practical management of C3 glomerulopathy and Ig-mediated MPGN: Facts and uncertainties. Kidney Int 2020; 98:11351148. doi: 10.1016/j.kint.2020.05.053

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7.

    Rabasco C, et al.. Effectiveness of mycophenolate mofetil in C3 glomerulonephritis. Kidney Int 2015; 88:11531160. doi: 10.1038/ki.2015.227

  • 8.

    Avasare RS, et al.. Mycophenolate mofetil in combination with steroids for treatment of C3 glomerulopathy: A case series. Clin J Am Soc Nephrol 2018; 13:406413. doi: 10.2215/CJN.09080817

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9.

    Ravindran A, et al.. C3 glomerulopathy: Ten years’ experience at Mayo Clinic. Mayo Clin Proc 2018; 93:9911008. doi: 10.1016/j.mayocp.2018.05.019

  • 10.

    Floege J, et al.. Chapter 8: Complement-associated glomerulonephritis. KDIGO Clinical Practice Guidelines on Glomerular Diseases. Public draft review. June 2020. https://kdigo.org/wp-content/uploads/2017/02/KDIGO-GN-GL-Public-Review-Draft_1-June-2020.pdf

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11.

    Bomback AS, et al.. Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol 2012; 7:748756. doi: 10.2215/CJN.12901211

  • 12.

    Ruggenenti P, et al.. C5 convertase blockade in membranoproliferative glomerulonephritis: A single-arm clinical trial. Am J Kidney Dis 2019; 74:224238. doi: 10.1053/j.ajkd.2018.12.046

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13.

    Galvan MD, et al.. Evaluation of urine complement biomarker in C3G following complement alternative pathway inhibition with ACH-4471 (SA-PO424). ASN Kidney Week 2018. https://www.asn-online.org/education/kidneyweek/2018/program-abstract.aspx?controlId=3021605

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14.

    Wong EKS, et al.. LNP023: A novel oral complement alternative pathway factor B inhibitor safely and effectively reduces proteinuria in C3 glomerulopathy (SU-OR39). ASN Kidney Week 2020. https://www.asn-online.org/education/kidneyweek/2020/program-abstract.aspx?controlId=3442221

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15.

    Dixon BP, et al.. C3 inhibition with APL-2 targets the underlying disease process of C3G complement hyperactivity and improves proteinuria (FR-PO906). ASN Kidney Week 2019. https://www.asn-online.org/education/kidneyweek/2019/program-abstract.aspx?controlId=3231183

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 16.

    Jayne DRW, et al.. Avacopan for the treatment of ANCA-associated vasculitis. N Engl J Med 2021; 384:599609. doi: 10.1056/NEJMoa2023386

  • 17.

    ChemoCentryx Press Release: ChemoCentryx and VFMCRP Provide Topline Results from ACCOLADE Trial of Avacopan in C3 Glomerulopathy Including Improved Estimated Glomerular Filtration Rate (eGFR). December 21, 2020. https://ir.chemocentryx.com/news-releases/news-release-details/chemocentryx-and-vfmcrp-provide-topline-results-accolade-trial

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18.

    Bomback AS, et al.. C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy. Kidney Int 2018; 93:977985. doi: 10.1016/j.kint.2017.10.022

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19.

    Caravaca-Fontán F, et al.. Validation of a histologic scoring index for C3 glomerulopathy. Am J Kidney Dis [published online ahead of print December 22, 2020]. doi: 10.1053/j.ajkd.2020.11.011; https://www.ajkd.org/article/S0272-6386(20)31160-4/fulltext

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20.

    Martín B, Smith RJH. C3 glomerulopathy. In GeneReviews (University of Seattle, WA) 2007 (updated 2018). https://www.ncbi.nlm.nih.gov/books/NBK1425/

C3 Glomerulopathy: Update on Pathogenesis and Treatment

Shikha Wadhwani Shikha Wadhwani, MD, MS, is Assistant Professor of Medicine, Division of Nephrology and Hypertension, Northwestern University Feinberg School of Medicine, Chicago, IL. Samir V. Parikh, MD, FASN, is Associate Professor of Medicine, Division of Nephrology, The Ohio State University Medical Center, Columbus, OH.

Search for other papers by Shikha Wadhwani in
Current site
Google Scholar
PubMed
Close
and
Samir V. Parikh Shikha Wadhwani, MD, MS, is Assistant Professor of Medicine, Division of Nephrology and Hypertension, Northwestern University Feinberg School of Medicine, Chicago, IL. Samir V. Parikh, MD, FASN, is Associate Professor of Medicine, Division of Nephrology, The Ohio State University Medical Center, Columbus, OH.

Search for other papers by Samir V. Parikh in
Current site
Google Scholar
PubMed
Close
Restricted access
Save