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Use of eculizumab in crescentic IgA nephropathy: Proof of principle and conundrum?

Lookup NU author(s): Professor Tim Goodship



This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).


© The Author 2015. Published by Oxford University Press on behalf of ERA-EDTA. IgA nephropathy (IgAN) is characterized by a variable clinical course and multifaceted pathophysiology. There is substantial evidence to suggest that complement activation plays a pivotal role in the pathogenesis of the disease. Therefore, complement inhibition using the humanized anti-C5 monoclonal antibody eculizumab could be a rational treatment. We report here a 16-year-old male with the vasculitic form of IgAN who failed to respond to aggressive conventional therapy including high-dose steroids, cyclophosphamide and plasma exchange and whowas treated with fourweekly doses of 900 mg eculizumab followed by a single dose of 1200 mg. He responded rapidly to this treatment and has had a stable creatinine around 150 μmol/L (1.67 mg/dL) for >6 months. However, proteinuria was unabated on maximal conventional anti-proteinuric treatment, and a repeat renal biopsy 11 months after presentation revealed severe chronic changes. We believe this case provides proof of principle that complement inhibition may be beneficial in IgAN but also that development of chronicity may be independent of complement.

Publication metadata

Author(s): Ring T, Pedersen BB, Salkus G, Goodship THJ

Publication type: Article

Publication status: Published

Journal: Clinical Kidney Journal

Year: 2015

Volume: 8

Issue: 5

Pages: 489-491

Print publication date: 01/10/2015

Online publication date: 27/08/2015

Acceptance date: 27/07/2015

Date deposited: 18/07/2017

ISSN (print): 2048-8505

ISSN (electronic): 2048-8513

Publisher: Oxford University Press


DOI: 10.1093/ckj/sfv076


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