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Non-Randall Glomerulonephritis with Non-Organized Monoclonal Ig Deposits

  • Pierre RoncoEmail author
  • Alexandre Karras
  • Emmanuelle Plaisier
Part of the Current Clinical Pathology book series (CCPATH)

Abstract

Plasma cell dyscrasias can induce a variety of kidney diseases either with organized deposits - AL amyloidosis, type-1 cryoglobulin, and immunotactoid glomerulonephritis, or with non-organized deposits of light or heavy chains characteristic of LCDD and HCDD, respectively. In addition to these well-defined diseases, a recently described entity has emerged, characterized by non-Randall-type, non-organized glomerular deposition of complete IgG. The term proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) was proposed, as most of the cases reported in the initial series shared pathologic features with membranoproliferative glomerulonephritis (MPGN). Nevertheless, cellular proliferation is sometimes minimal and the glomerulopathy can then be categorized as atypical membranous nephropathy (MN). Diagnosis relies on immunofluorescence examination demonstrating light chain isotype restriction, mostly kappa (70–80 %), associated with gamma heavy chain subclass restriction, and on electron microscopy showing lack of organization of the granular electron-dense deposits. Interestingly, the IgG subclass seems to influence the glomerular pattern as most MPGN are associated with IgG3, while the majority of MN is associated with IgG1. Less than a third of all patients have evidence of dysproteinemia by serum and urine examination, while hypocomplementemia is observed in 30 % of them, despite negative testing for cryoglobulin. Renal prognosis is poor, leading to dialysis in 20 % of cases, although it can be improved by treatment of the underlying hemopathy which should be carefully searched for. Several reports suggest that rituximab, a B-cell depleting agent, could be a promising treatment in patients without overt malignancy, including those with post-kidney transplant recurrence of the disease. Several cases of GN with isolated renal C3 deposits and circulating monoclonal gammopathy have been recently reported. These cases might represent an unusual complication of plasma cell dyscrasia, related to complement activation through an autoantibody activity of the monoclonal Ig against a complement alternative pathway regulator protein such as complement factor H.

Keywords

Membranoproliferative glomerulonephritis Monoclonal component Membranous nephropathy Anti-CD20 antibody Ig subclass 

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Pierre Ronco
    • 1
    • 2
    • 3
    Email author
  • Alexandre Karras
    • 4
  • Emmanuelle Plaisier
    • 1
    • 2
  1. 1.Sorbonne Universités, UPMC Univ Paris 06, UMR_S 1155ParisFrance
  2. 2.Department of Nephrology and DialysisAP-HP, Hôpital TenonParisFrance
  3. 3.UMR_S 1155, Batiment Recherche, Hôpital TenonParisFrance
  4. 4.Department of NephrologyAP-HP, University Paris Descartes, Hôpital Européen Georges PompidouParisFrance

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