C3 glomerulopathy

Abstract

The role of the complement system in pathogenesis of glomerular kidney diseases has been suspected and anticipated since long time. Recent developments in immunology and genetics, as well as accumulation and review of significant amount of clinical data allowed revision of the current classification and introduction of a new category - C3 glomerulopathy. It consists of two diseases - dense deposits disease and C3 glomerulonephritis. Pathogenesis is common - dysregulation of the alternative pathway of the complement due to genetic or acquired causes. Immunofluorescent study of renal biopsy is diagnostic - presence of isolated or predominant deposition of C3, independent of the other findings. The two diseases are distinguished by electron microscopy - typical intramembrane-ous electron dense deposits are consistent with dense deposits disease or if they are not present, the case is classified as C3 glomerulonephritis. C3 glomerulopathy is ultra-rare disease, but with severe clinical course and leads to end-stage renal disease in short time. There is a high rate of recurrence of the disease in the graft after kidney transplantation. Unfortunately, treatment is nonspecific and currently there are no therapeutic options with proved efficacy.