Eculizumab for dense deposit disease and C3 glomerulonephritis.

ABSTRACT:

Background and objectives

The principle defect in dense deposit disease and C3 glomerulonephritis is hyperactivity of the alternative complement pathway. Eculizumab, a monoclonal antibody that binds to C5 to prevent formation of the membrane attack complex, may prove beneficial.

Design, setting, participants, & measurements

In this open-label, proof of concept efficacy and safety study, six subjects with dense deposit disease or C3 glomerulonephritis were treated with eculizumab every other week for 1 year. All had proteinuria >1 g/d and/or AKI at enrollment. Subjects underwent biopsy before enrollment and repeat biopsy at the 1-year mark.

Results

The subjects included three patients with dense deposit disease (including one patient with recurrent dense deposit diseas

SUBMITTER: Bomback AS 

PROVIDER: S-EPMC3338285 | biostudies-literature | 2012 May

REPOSITORIES: biostudies-literature