A rare disease characterized by activation of the alternative complement cascade.
Associated with deposition of the third component of complement without any immunoglobulin deposits in the glomeruli of the kidney.
Characterized by accumulation of complement factors in the glomeruli due to overactivation and abnormal regulation of the alternative pathway of complement.
The abnormal control of the alternative pathway of complement may be due to acquire or a genetic abnormalities of the complement regulatory proteins.
Characterized on kidney biopsy by deposition of complement by-products the result of over activation of the alternative complement pathway.
The renal histology reveals C3 glomerulopathy by light microscopic findings is frequently of a member membranoproliferative glomerulonephritis pattern.
The process is an immune complex one, with complement component C3 indicating complement dysregulation.
Renal biopsy demonstrates strong C3 deposition.
Many patients with C3 glomerular deposits have defects in regulatory proteins, inhibitors or accelerators of the alternative complement pathway.
When C3 deposits are scattered in the mesangial regions and subepithelial or subendothelial locations, disease is called C3 glomerulonephritis.
Immune complex membranoproliferative glomerulonephritis is characterized by glomerular deposition of both immunoglobulins and complement by-products.
Most cases or secondary to infection, auto immunity, or monotypic immunoglobulin deposition.
The disease is heterogeneous and there’s a broad range of clinicopathological findings, triggering events, complement abnormalities, and renal outcomes.
Within 10 years after diagnosis, up to 50% of patients have progression to advanced kidney failure that leads to dialysis or transplantation.
Mycophenolate mofetil has led to partial remissions with high incident of recurrence, up to 70%, after the kidney translation despite its use as a component of post transplantion immunosuppression.
Recurrent C3 glomerulopathy leads to allograft loss in up to 60% of grafts.
Eculizumab is approved as a specific blocker of the fifth component of complement and is used to treat patients with C3 glomerulopathy hey solo incident of response (30%) or no response.
Process the frequently recurs after kidney transplantation.
Pegcetacoplan results in a significantly greater reduction in proteinuria than placebo among patients with a C3 glomerulopathy or primary immune complex membrano proliferative glomerulonephritis.
Pegcetacoplan binds C3 and its activation fragment C3b thereby regulating C3 cleavage, and generation of downstream complement factors: it inhibits complement activation through the classical, lectin, and alternative complement pathways.