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Published in: BMC Nephrology 1/2018

Open Access 01-12-2018 | Case report

Favorable effect of bortezomib in dense deposit disease associated with monoclonal gammopathy: a case report

Authors: Shuma Hirashio, Ayaka Satoh, Takahiro Arima, Kouichi Mandai, Tadasuke Awaya, Kumi Oshima, Shigeo Hara, Takao Masaki

Published in: BMC Nephrology | Issue 1/2018

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Abstract

Background

Complement component 3 (C3) glomerulopathy, which includes dense deposit disease (DDD) and C3 glomerulonephritis, is caused by dysregulation of the alternative complement pathway. In most cases, C3 glomerulopathy manifests pathologically with membranoproliferative glomerulonephritis-like features. An association between C3 glomerulopathy and monoclonal gammopathy was recently reported in several cases, raising the possibility that C3 glomerulopathy is the underlying pathological process in monoclonal gammopathy of renal significance.

Case presentation

We herein report a case of monoclonal gammopathy-induced DDD that improved histologically and clinically with chemotherapy including bortezomib. Our case is the first in which treatment response can be linked to the histological response. Potential pathological insights are also discussed.

Conclusions

Rapid and efficient chemotherapy has the potential to limit renal damage in monoclonal gammopathy-associated DDD.
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Metadata
Title
Favorable effect of bortezomib in dense deposit disease associated with monoclonal gammopathy: a case report
Authors
Shuma Hirashio
Ayaka Satoh
Takahiro Arima
Kouichi Mandai
Tadasuke Awaya
Kumi Oshima
Shigeo Hara
Takao Masaki
Publication date
01-12-2018
Publisher
BioMed Central
Published in
BMC Nephrology / Issue 1/2018
Electronic ISSN: 1471-2369
DOI
https://doi.org/10.1186/s12882-018-0905-6

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