Novel insights to local complement activity in C3 glomerulopathy

C3 glomerulopathy is an ultra-rare glomerular disease driven by dysregulation of the alternative pathway of complement. It affects about 1-2 persons per million, half of whom progress to end-stage renal failure within ten years of diagnosis. The defining feature required to diagnose C3 glomerulopathy is deposition of complement component C3 in the renal glomerulus on kidney biopsy as resolved by immunofluorescence.