Monoclonal Gammopathy of Renal Significance: Clinical and Histological Efficacy of a Bortezomib-Based Regimen.
C3 glomerulonephritis (C3GN)
Immunotactoid glomerulonephritis
bortezomib
glomerulonephritis
monoclonal gammopathy of renal significance (MGRS)
Journal
Frontiers in medicine
ISSN: 2296-858X
Titre abrégé: Front Med (Lausanne)
Pays: Switzerland
ID NLM: 101648047
Informations de publication
Date de publication:
2020
2020
Historique:
received:
25
07
2020
accepted:
23
11
2020
entrez:
4
1
2021
pubmed:
5
1
2021
medline:
5
1
2021
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 33392216
doi: 10.3389/fmed.2020.587345
pmc: PMC7772466
doi:
Types de publication
Case Reports
Langues
eng
Pagination
587345Informations de copyright
Copyright © 2020 Quattrocchio, Barreca, Vaccarino, Del Vecchio, De Simone, Fenoglio, Ferro, Pagliaro, Pini, Manes and Roccatello.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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