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Case Reports in Nephrology
Volume 2014, Article ID 201568, 4 pages
http://dx.doi.org/10.1155/2014/201568
Case Report

Blockade of Alternative Complement Pathway in Dense Deposit Disease

1Service de Pharmacie, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, France
2Service de Néphrologie, Centre Hospitalier Territorial de Nouvelle Calédonie, Gaston Bourret, BP J5, 98849 Nouméa, New Caledonia
3Service de Néphrologie Pédiatrique, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, France
4Laboratoire d’Anatomopathologie, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, France
5Laboratoire d’Immunologie, Hôpital Européen Georges-Pompidou, 20-40 rue Leblanc, 75015 Paris, France

Received 31 October 2013; Accepted 26 December 2013; Published 6 February 2014

Academic Editors: R. Enríquez, H. Matsukura, and K. Nozu

Copyright © 2014 Aurore Berthe-Aucejo et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

A patient aged 17 with dense deposit disease associated with complement activation, circulating C3 Nef, and Factor H mutation presented with nephrotic syndrome and hypertension. Steroid therapy, plasma exchange, and rituximab failed to improve proteinuria and hypertension despite a normalization of the circulating sC5b9 complex. Eculizumab, a monoclonal antibody directed against C5, was used to block the terminal product of the complement cascade. The dose was adapted to achieve a CH50 below 10%, but proteinuria and blood pressure were not improved after 3 months of treatment.