title: Blockade of alternative complement pathway in dense deposit disease.
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.
authors.affiliation: Service de Pharmacie, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, France.
authors.forename: Aurore
authors.identifier: 0000-0002-9457-6996
authors.initials: A
authors.lastname: Berthe-Aucejo
authors.affiliation: Service de Néphrologie, Centre Hospitalier Territorial de Nouvelle Calédonie, Gaston Bourret, BP J5, 98849 Nouméa, New Caledonia.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Sacquépée
authors.affiliation: Service de Néphrologie Pédiatrique, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Fila
authors.affiliation: Laboratoire d'Anatomopathologie, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Peuchmaur
authors.affiliation: Service de Pharmacie, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, France.
authors.forename: Emilia
authors.initials: E
authors.lastname: Perrier-Cornet
authors.affiliation: Laboratoire d'Immunologie, Hôpital Européen Georges-Pompidou, 20-40 rue Leblanc, 75015 Paris, France.
authors.forename: Véronique
authors.initials: V
authors.lastname: Frémeaux-Bacchi
authors.affiliation: Service de Néphrologie Pédiatrique, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, France.
authors.forename: Georges
authors.initials: G
authors.lastname: Deschênes