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

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. A...

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Bibliographic Details
Published in:BMC nephrology 2018-05, Vol.19 (1), p.108-108, Article 108
Main Authors: Hirashio, Shuma, Satoh, Ayaka, Arima, Takahiro, Mandai, Kouichi, Awaya, Tadasuke, Oshima, Kumi, Hara, Shigeo, Masaki, Takao
Format: Article
Language:English
Subjects:
Antineoplastic Agents - therapeutic use
Bortezomib
Bortezomib - therapeutic use
C3 glomerulopathy
Cancer
Care and treatment
Case Report
Case studies
Chemotherapy
Complement
Complement C3 - metabolism
Complications and side effects
Dense deposit disease
Dosage and administration
Glomerulonephritis
Glomerulonephritis, Membranoproliferative - blood
Glomerulonephritis, Membranoproliferative - diagnosis
Glomerulonephritis, Membranoproliferative - drug therapy
Humans
Male
Middle Aged
Monoclonal gammopathies
Monoclonal gammopathy of renal significance
Monoclonal gammopathy of undetermined significance
Paraproteinemias - blood
Paraproteinemias - diagnosis
Paraproteinemias - drug therapy
Risk factors
Treatment Outcome
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Summary: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. 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. Rapid and efficient chemotherapy has the potential to limit renal damage in monoclonal gammopathy-associated DDD.
ISSN:1471-2369
1471-2369
DOI:10.1186/s12882-018-0905-6