Proliferative glomerulonephritis with monoclonal immunoglobulin deposits: a nephrologist perspective

Author:

Bridoux Frank1,Javaugue Vincent1,Nasr Samih H2,Leung Nelson3

Affiliation:

1. Department of Nephrology, Centre de Référence Maladies Rares « Amylose AL et autres maladies par dépôts d’immunoglobulines monoclonales », Centre Hospitalier Universitaire et Université de Poitiers, Poitiers, and CNRS UMR 7276-INSERM 1262, Limoges, France

2. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA

3. Division of Nephrology and Hypertension and Division of Hematology, Mayo Clinic, Rochester, MN, USA

Abstract

Abstract Proliferative glomerulonephritis (GN) with monoclonal immunoglobulin deposits (PGNMIDs) is a recently described entity among the spectrum of monoclonal gammopathy of renal significance (MGRS). The disease is renal limited and manifests with chronic glomerular disease, altered renal function and albuminuria, sometimes in the nephrotic range. Acute nephritic syndrome is rare. PGNMID occurs mostly in the sixth decade, but it may affect young adults. Histologically, PGNMID is characterized predominantly by membranoproliferative GN and less frequently by diffuse endocapillary GN, mesangioproliferative GN or atypical membranous GN. Immunofluorescence and electron microscopic studies are the cornerstone of diagnosis, showing granular deposits involving glomeruli only, and composed of monotypic immunoglobulin G (IgG), with a single heavy chain subclass (most commonly IgG3) and light chain (LC) restriction (usually κ), admixed with complement deposits. PGNMID variants with monotypic LC-only, IgA or IgM deposits are uncommon. Ultrastructurally, deposits are amorphous with predominant subendothelial and mesangial distribution. PGNMID should be distinguished from type 1 cryoglobulinemic GN and immunotactoid GN, which share some common pathological features. Contrary to other MGRS lesions, the rate of detection of the nephrotoxic monoclonal Ig in the serum or urine, and of an abnormal bone marrow B-cell clone, is only ∼30%. Renal prognosis is poor, with progression to end-stage renal disease in 25% of patients within 30 months and frequent early recurrence on the renal allograft. The pathophysiology of PGNMID is unclear and its treatment remains challenging. However, recent studies indicate that clone-targeted chemotherapy may significantly improve renal outcomes, opening future perspectives for the management of this rare disease.

Publisher

Oxford University Press (OUP)

Subject

Transplantation,Nephrology

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