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C3 glomerulonephritis associated with monoclonal gammopathy: a case series

Ladan Zand1, Andrea Kattah, Fernando C Fervenza

  • 1Department of Internal Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN 55905, USA.

American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation
|April 30, 2013
PubMed

Insights

C3 glomerulonephritis (C3 GN) is linked to monoclonal gammopathy, especially in older adults. Treating the underlying blood disorder can improve kidney function and reduce proteinuria and hematuria in C3 GN patients.

Area of Science:

  • Nephrology
  • Hematology
  • Immunology

Background:

  • C3 glomerulonephritis (C3 GN) is characterized by complement factor deposition in glomeruli, stemming from alternative complement pathway dysregulation.
  • This dysregulation can arise from mutations or functional inhibition of complement-regulating proteins, sometimes linked to monoclonal gammopathy.

Purpose of the Study:

  • To investigate the association between C3 GN and monoclonal gammopathy.
  • To describe the clinical, hematologic, and renal characteristics of patients with C3 GN and monoclonal gammopathy.
  • To evaluate treatment approaches and outcomes for this patient cohort.

Main Methods:

  • A case series of 32 patients with C3 GN at Mayo Clinic.
  • Analysis of clinical features, bone marrow and kidney biopsies, and complement pathway abnormalities.
  • Review of treatment strategies and patient follow-up.

Main Results:

  • Ten of 32 C3 GN patients (31%) had evidence of monoclonal immunoglobulin.
  • The mean age of these patients was 54.5 years.
  • Kidney biopsies showed membranoproliferative GN with C3 deposition; 7 of 9 tested had alternative complement pathway abnormalities. Treatment targeting underlying hematologic conditions led to improved kidney function and reduced proteinuria/hematuria.

Conclusions:

  • C3 GN is associated with monoclonal gammopathy, particularly in older individuals.
  • Targeting the underlying hematologic malignancy is a crucial therapeutic strategy for C3 GN.
  • Further studies are needed to elucidate the direct activation of the alternative pathway by monoclonal immunoglobulins.
Abstract

Keywords:
C3 Glomerulonephritis (C3 GN)membranoproliferative glomerulonephritis (MPGN)monoclonal gammopathymonoclonal gammopathy of undetermined significance (MGUS)

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