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Vol. 214. Issue 5.
Pages 266-274 (June - July 2014)
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Vol. 214. Issue 5.
Pages 266-274 (June - July 2014)
Clinical up-date
C3 glomerulopathy: A new complement-based entity
Glomerulopatía C3: una nueva entidad basada en el complemento
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1001
A. de Lorenzo
Corresponding author
doctorberto@hotmail.com

Corresponding author.
, S. Tallón, B. Hernández-Sevillano, G. de Arriba
Servicio de Nefrología, Hospital Universitario de Guadalajara, Departamento de Medicina, Universidad de Alcalá, Alcalá de Henares, Madrid, Spain
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Table 1. New classification of membranoproliferative glomerulonephritis.
Table 2. Case series of C3 glomerulopathy: characteristics, treatment and evolution.
Table 3. Eculizumab: dosage and method of administration.
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Abstract

C3 glomerulopathy is a new, recently described entity that has changed the perspective, treatment and classification of a number of glomerular diseases. It encompasses 2 similar but clearly differentiated pathologies—the dense-deposit disease and C3 glomerulonephritis itself. The alternative complement pathway plays a fundamental role in its pathogenesis and, specifically, the mutations and defects in its regulatory factors (mainly factor H and factor I), as well as the presence of acquired autoantibodies (C3 nephritic factor), which generates an unbridled activation of the system, and ultimately, a deposit of its products at the glomerular level. Its poor prognosis and onset in young populations make the detailed study of new therapeutic alternatives for this disease essential. Recently eculizumab, an anti-C5 antibody, has demonstrated effectiveness in the treatment of these patients.

Keywords:
C3 glomerulopathy
Dense-deposit disease
Alternative complement pathway
Factor H
Factor I
C3 nephritic factor
Eculizumab
Resumen

La glomerulopatía C3 es una nueva entidad descrita recientemente que ha cambiado la visión, el tratamiento y la clasificación de algunas enfermedades glomerulares. Engloba 2 patologías similares pero claramente diferenciadas: la enfermedad por depósitos densos y la glomerulonefritis C3 propiamente dicha. La vía alternativa del complemento juega un papel fundamental en su patogenia, y en concreto las mutaciones o defectos en sus factores reguladores (fundamentalmente factor H y factor I), así como la presencia de autoanticuerpos adquiridos (factor nefrítico C3) que generan una activación desenfrenada del sistema, y en último término un depósito de sus productos a nivel glomerular. Su mal pronóstico y la aparición en población joven hacen preciso el estudio de nuevas alternativas terapéuticas. Recientemente eculizumab, un anticuerpo anti C5, ha demostrado efectividad en el tratamiento de estos pacientes.

Palabras clave:
Glomerulopatía C3
Enfermedad por depósitos densos
Vía alternativa del complemento
Factor H
Factor I
Factor nefrítico C3
Eculizumab

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