WCN25-3918 C3GN ASSOCIATED WITH DELETIONS IN CHFR1 AND CHFR3 GENES

C3GN is a rare disorder that typically results from abnormality in alternative pathway of complement system. It is characterized by deposition of predominant C3 component of complement with or without small amount of immunoglobulin in mesangial subepithelial and subendothelial glomerular areas. Pathogenetic mutation occurs in C3 , complement factor B, complement factor H, complement factor I and CFHR5. Autoantibodies against C3 nephrotic factor, C5 nephritic factor, anti CHF antibodies and anti complement factor B autoantibodies are also associated with this.