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2017•Kidney International ReportsOpen access

Long-term Eculizumab Therapy in a Child With Refractory Immune Complex–Mediated Membranoproliferative Glomerulonephritis

Rahul Chanchlani, Paul Scott Thorner, Seetha Radhakrishnan, Diane Hébert, Valérie S. Langlois, Steven Arora, David S. Barth, Daniel C. Cattran, Michael Kirschfink, Christoph P. B. Licht

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Abstract

There have been recent developments in the understanding of the pathogenesis of membranoproliferative glomerulonephritis (MPGN) supporting a prominent role for the complement alternative pathway (AP).1,2 MPGN due to AP dysregulation has been further classified into dense-deposit disease (DDD) and C3 glomerulonephritis (C3GN), and grouped together as C3 glomerulopathy (C3G). This entity includes all glomerular lesions that are characterized by predominant C3 accumulation with minimum or scant Ig deposition and highlights the pathogenetic contribution of complement.

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There have been recent developments in the understanding of the pathogenesis of membranoproliferative glomerulonephritis (MPGN) supporting a prominent role for the complement alternative pathway (AP).1,2 MPGN due to AP dysregulation has been further classified into dense-deposit disease (DDD) and C3 glomerulonephritis (C3GN), and grouped together as C3 glomerulopathy (C3G). This entity includes all glomerular lesions that are characterized by predominant C3 accumulation with minimum or scant Ig deposition and highlights the pathogenetic contribution of complement.

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Available abstract

There have been recent developments in the understanding of the pathogenesis of membranoproliferative glomerulonephritis (MPGN) supporting a prominent role for the complement alternative pathway (AP).1,2 MPGN due to AP dysregulation has been further classified into dense-deposit disease (DDD) and C3 glomerulonephritis (C3GN), and grouped together as C3 glomerulopathy (C3G). This entity includes all glomerular lesions that are characterized by predominant C3 accumulation with minimum or scant Ig deposition and highlights the pathogenetic contribution of complement.

Key concepts: Membranoproliferative glomerulonephritis, Glomerulopathy, Medicine, Eculizumab, Alternative complement pathway, Atypical hemolytic uremic syndrome, Complement system, Glomerulonephritis

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Long-term Eculizumab Therapy in a Child With Refractory Immune Complex–Mediated Membranoproliferative Glomerulonephritis — Research Paper | ScholarLens