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. 2014:2014:201568.
doi: 10.1155/2014/201568. Epub 2014 Feb 6.

Blockade of alternative complement pathway in dense deposit disease

Aurore Berthe-Aucejo  1 , Mathieu Sacquépée  2 , Marc Fila  3 , Michel Peuchmaur  4 , Emilia Perrier-Cornet  1 , Véronique Frémeaux-Bacchi  5 , Georges Deschênes  3
Affiliations

Blockade of alternative complement pathway in dense deposit disease

Aurore Berthe-Aucejo et al. Case Rep Nephrol. 2014.

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.

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Figures

Figure 1
Figure 1
(a) Type II membranoproliferative glomerulonephritis characterized by mesangial matrix and cellular increases is responsible for a lobular accentuation associated with a diffuse and intense staining of the peripheral basement membrane (periodic acid-Schiff [PAS], magnification [G]: ×200). (b) The diffuse and intense staining of the peripheral basement membrane indicates the presence of dense deposit material (PAS, G ×2000). (c) Immunofluorescence techniques show segmental pseudo linear and granular IgM deposits along the peripheral capillary wall (fluorescein isothiocyanate anti-IgM, G ×100).
Figure 2
Figure 2
Response to eculizumab therapy in dense deposit disease.
See this image and copyright information in PMC

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