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Kidney Int Rep. 2021 May 04;6(8):2059-2065. doi: 10.1016/j.ekir.2021.04.026. eCollection 2021 Aug.

A Fresh Perspective on Monoclonal Gammopathies of Renal Significance.

Kidney international reports

Pierre Aucouturier, Vivette D D'Agati, Pierre Ronco

Affiliations

  1. Hôpital St-Antoine, Département d'Immunologie Biologique, AP-HP, and Sorbonne Université / Inserm UMRS 938, Paris, France.
  2. Department of Pathology and Cell Biology, Columbia University Medical Center, New York, New York, USA.
  3. Sorbonne Université / Inserm UMRS 1155, Paris, France; Service de Néphrologie Centre Hospitalier du Mans, Le Mans, France.

PMID: 34386655 PMCID: PMC8343799 DOI: 10.1016/j.ekir.2021.04.026

Abstract

Monoclonal gammopathies of renal significance (MGRS) encompass a remarkable variety of kidney diseases that result from intrinsic nephrotoxic properties of certain monoclonal Igs or their subunits. Effective disease-modifying treatments rely on the targeting of a malignant B-cell clone that may be demonstrable but often is quite hypothetical. Hence, convincing arguments for the genuine monoclonal character of the causative mono-isotypic Ig tissue deposits is needed for design of appropriate treatment strategies. The purpose of this article was to critically analyze distinct situations of suspected MGRS that occur in the practice of pathologists, nephrologists, hematologists, and immunologists. A particular focus of interest is the group of conditions known as proliferative glomerulonephritis with mono-isotypic immunoglobulin deposits (PGNMIDs), which illustrates the difficulties and ambiguities surrounding a definitive assignment of MGRS status.

© 2021 International Society of Nephrology. Published by Elsevier Inc.

Keywords: IgG3; PGNMID; glomerulopathy; immunoglobulin isotypes; immunoglobulin variable regions; monoclonal gammopathy; monotypic deposits

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