C3 glomerulopathy

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.

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.

The disease is heterogeneous and there’s a broad range of clinicopathological findings, triggering events,  complement abnormalities, and renal outcomes.

Eculizumab is approved as a specific blocker of the fifth component of complement and is used to treat patients with C3 glomerulopathy.

Process the frequently recurs after kidney transplantation.

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