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

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.

• Authors: Shuma Hirashio, Ayaka Satoh, Takahiro Arima, Kouichi Mandai, Tadasuke Awaya, Kumi Oshima, Shigeo Hara, Takao Masaki
• Journal: BMC nephrology (BMC Nephrol) Vol. 19 Issue 1 Pg. 108 (05 03 2018) ISSN: 1471-2369 [Electronic] England
• PMID: 29724182 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antineoplastic Agents
• Complement C3
• Bortezomib

Topics

• Antineoplastic Agents (therapeutic use)
• Bortezomib (therapeutic use)
• Complement C3 (metabolism)
• Glomerulonephritis, Membranoproliferative (blood, diagnosis, drug therapy)
• Humans
• Male
• Middle Aged
• Paraproteinemias (blood, diagnosis, drug therapy)
• Treatment Outcome