Light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits is associated with a high detection rate of the pathogenic plasma cell clone

Nasr, Samih H.; Larsen, Christopher P.; Sirac, Christophe; Theis, Jason D.; Domenger, Catherine; Chauvet, Sophie; Javaugue, Vincent; Hogan, Jonathan J.; Said, Samar M.; Dasari, Surendra; Vrana, Julie A.; McPhail, Ellen D.; Cornell, Lynn D.; Vilaine, Éve; Massy, Ziad A.; Boffa, J.J.; Buob, David; Toussaint, Stéphanie; Guincestre, Thomas; Touchard, Guy; D’Agati, Vivette D.; Leung, Nelson; Bridoux, Frank

doi:10.1016/j.kint.2019.10.025

Cited by 36 publications

(50 citation statements)

References 40 publications

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“…Rarely only heavy or light chain may be evident (8). Very recently a series of 17 patients with proliferative glomerulonephritis with deposition of monoclonal immunoglobulin light chain only (PGNMID-light chain) has been described (9). In this series, by immunofluorescence, deposits were composed of restricted light chain (kappa in 71% of cases) and C3, as in our patient.…”

Section: Discussionsupporting

confidence: 63%

Monoclonal Gammopathy of Renal Significance: Clinical and Histological Efficacy of a Bortezomib-Based Regimen

et al. 2020

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Monoclonal Gammopathy of Renal Significance (MGRS) is a group of heterogeneous disorders characterized by renal dysfunction secondary to the production of a monoclonal immunoglobulin by a nonmalignant B cell or plasma cell clone. We report the clinical and histological outcomes of two patients with biopsy-proven MGRS: one patient showed membranoproliferative glomerulonephritis with monoclonal k-light chain and C3 deposits, the second patient showed immunotactoid glomerulopathy. Both patients were treated with a 9-month chemotherapy protocol including bortezomib, cyclophosphamide, and dexamethasone. Renal biospy was repeated after 1 year. The estimated glomerular filtration rate (eGFR) increased from 22.5 (baseline) to 40 ml/min per 1.73 m2 after 12 months, then to 51.5 ml/min per 1.73 m2 after 24 months; proteinuria decreased from 4.85 (baseline) to 0.17 g/day after 12 months, then to 0.14 g/day after 24 months. Repeat renal biopsies showed a dramatic improvement of the glomerular proliferative lesions and near complete disappearance of the immune deposits. A bortezomib-based treatment proved very effective and was well-tolerated in the two patients presenting with clinically and histologically aggressive MGRS.

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Section: Discussionsupporting

confidence: 63%

Monoclonal Gammopathy of Renal Significance: Clinical and Histological Efficacy of a Bortezomib-Based Regimen

et al. 2020

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“…5,7,17 In contrast, PGNMID, also a renal-limited disease, is characterized by glomerular granular deposits of monotypic IgG (mostly IgG3κ), and occasionally IgM, IgA or monoclonal LC only. 15,[18][19][20][21] These deposits are predominantly subendothelial and mesangial, and uncommonly observed in subepithelial space. Due to overlapping features, it has been hypothesized that ITG and cryoglobulinemic glomerulonephritis could be variants of the same disease, and that low-level circulating cryoglobulins may be implicated in the pathophysiology of ITG.…”

Section: Discussionmentioning

confidence: 99%

Results of a nation-wide cohort study suggest favorable long-term outcomes of clone-targeted chemotherapy in immunotactoid glomerulopathy

Javaugue¹,

Dufour-Nourigat²,

Desport³

et al. 2021

Kidney International

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“…Case series suggests characterization by a high detection rate of pathogenic clonal disorder and response to anti-myeloma agents. Recurrence post kidney transplantation is extremely common 4 .…”

Section: Discussionmentioning

confidence: 99%

“…reported and there is increasing evidence that the different kidney biopsy findings in multiple myeloma can have prognostic implications [4,5].…”

Section: Several Pathologic Manifestations Of Myeloma Related Kidney mentioning

confidence: 99%

Kidney Injury in Multiple Myeloma: A Kidney Biopsy Teaching Case

2021

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Light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits is associated with a high detection rate of the pathogenic plasma cell clone

Cited by 36 publications

References 40 publications

Monoclonal Gammopathy of Renal Significance: Clinical and Histological Efficacy of a Bortezomib-Based Regimen

Monoclonal Gammopathy of Renal Significance: Clinical and Histological Efficacy of a Bortezomib-Based Regimen

Results of a nation-wide cohort study suggest favorable long-term outcomes of clone-targeted chemotherapy in immunotactoid glomerulopathy

Kidney Injury in Multiple Myeloma: A Kidney Biopsy Teaching Case

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