Blockade of Alternative Complement Pathway in Dense Deposit Disease

oleh: Aurore Berthe-Aucejo, Mathieu Sacquépée, Marc Fila, Michel Peuchmaur, Emilia Perrier-Cornet, Véronique Frémeaux-Bacchi, Georges Deschênes

Format: Article
Diterbitkan: Hindawi Limited 2014-01-01

Deskripsi

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.