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2018 ; 19
(1
): 108
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Favorable effect of bortezomib in dense deposit disease associated with
monoclonal gammopathy: a case report
#MMPMID29724182
Hirashio S
; Satoh A
; Arima T
; Mandai K
; Awaya T
; Oshima K
; Hara S
; Masaki T
BMC Nephrol
2018[May]; 19
(1
): 108
PMID29724182
show ga
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.