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Rope R, Kambham N, Arora N. A case report of paraproteinemia-associated pauci-immune glomerulonephritis - a new form of monoclonal gammopathy of renal significance?. Clin Nephrol Case Stud. 2017;5:48-53doi: 10.5414/CNCS109160.
Rope, R., Kambham, N., & Arora, N. (2017). A case report of paraproteinemia-associated pauci-immune glomerulonephritis - a new form of monoclonal gammopathy of renal significance?. Clinical nephrology. Case studies, 548-53. https://doi.org/10.5414/CNCS109160
Rope, Robert, et al. "A case report of paraproteinemia-associated pauci-immune glomerulonephritis - a new form of monoclonal gammopathy of renal significance?." Clinical nephrology. Case studies vol. 5 (2017): 48-53. doi: https://doi.org/10.5414/CNCS109160
Rope R, Kambham N, Arora N. A case report of paraproteinemia-associated pauci-immune glomerulonephritis - a new form of monoclonal gammopathy of renal significance?. Clin Nephrol Case Stud. 2017 Aug 17;5:48-53. doi: 10.5414/CNCS109160. eCollection 2017. PMID: 29043147; PMCID: PMC5642763.
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Clin Nephrol Case Stud. 2017 Aug 17;5:48-53. doi: 10.5414/CNCS109160. eCollection 2017.

A case report of paraproteinemia-associated pauci-immune glomerulonephritis - a new form of monoclonal gammopathy of renal significance?.

Clinical nephrology. Case studies

Robert Rope, Neeraja Kambham, Neiha Arora

Affiliations

  1. Division of Nephrology.
  2. Department of Pathology, Stanford University School of Medicine, Stanford, and.
  3. Department of Nephrology, Kaiser Permanente, Fremont, CA, USA.

PMID: 29043147 PMCID: PMC5642763 DOI: 10.5414/CNCS109160

Abstract

BACKGROUND: Renal disease associated with paraproteinemias is classically predicated upon pathologic paraprotein deposition in the kidney. However, growing evidence suggests that paraproteins may be able to systemically activate complement or neutrophils to drive renal damage. This may provide an alternative pathologic mechanism for renal injury in rare cases.

CASE REPORT: We report a case of a patient with crescentic pauci-immune glomerulonephritis presenting with rapidly progressive renal failure, polyarthropathy, and a purpuric rash in association with a monoclonal immunoglobulin G κ-light-chain producing multiple myeloma. Serum anti-neutrophil cytoplasmic antibodies were not detected. Kidney biopsy, including with Pronase digestion, did not reveal pathologic paraprotein deposition. Two previously published similar case reports are also discussed.

CONCLUSION: We propose a novel pathologic mechanism involving monoclonal proteins as a trigger for pauci-immune glomerulonephritis, potentially via complement dysregulation and/or neutrophil activation. This requires further epidemiologic and mechanistic study.

Keywords: monoclonal gammopathy of renal significance (MGRS); monoclonal gammopathy of unknown significance (MGUS); multiple myeloma; pauci-immune glomerulonephritis

References

  1. J Immunol. 1999 Oct 15;163(8):4590-6 - PubMed
  2. Mod Pathol. 2015 Jun;28(6):854-60 - PubMed
  3. Am J Kidney Dis. 2010 Nov;56(5):977-82 - PubMed
  4. Case Rep Nephrol Dial. 2015 Jun 04;5(2):124-9 - PubMed
  5. J Am Soc Nephrol. 2009 Feb;20(2):289-98 - PubMed
  6. Am J Pathol. 2007 Jan;170(1):52-64 - PubMed
  7. Mayo Clin Proc. 2013 Nov;88(11):1284-93 - PubMed
  8. Mod Rheumatol. 2010 Dec;20(6):632-6 - PubMed
  9. Clin Kidney J. 2015 Jun;8(3):343-50 - PubMed
  10. Joint Bone Spine. 2006 Dec;73(6):710-3 - PubMed
  11. World J Nephrol. 2015 May 6;4(2):169-84 - PubMed
  12. Mayo Clin Proc. 2009 Aug;84(8):685-93 - PubMed
  13. Clin Kidney J. 2013 Jun;6(3):327-329 - PubMed
  14. Kidney Int. 2013 Dec;84(6):1079-89 - PubMed
  15. Kidney Int. 2013 Jan;83(1):129-37 - PubMed
  16. J Am Soc Nephrol. 2014 Feb;25(2):225-31 - PubMed
  17. Clin J Am Soc Nephrol. 2013 Nov;8(11):2007-17 - PubMed
  18. Kidney Int. 2015 Apr;87(4):698-711 - PubMed
  19. N Engl J Med. 2006 Mar 30;354(13):1362-9 - PubMed
  20. Blood. 2012 Nov 22;120(22):4292-5 - PubMed

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