C3 glomerulopathy: A new complement-based entity

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 makes 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: Alternative complement pathway; C3 glomerulopathy; C3 nephritic factor; Dense-deposit disease; Eculizumab; Enfermedad por depósitos densos; Factor H; Factor I; Factor nefrítico C3; Glomerulopatía C3; Vía alternativa del complemento.