Complement component 3 glomerulopathy

Complement component 3 glomerulopathy (C3G) is a rare, complement-mediated renal disorder affecting the glomeruli across pediatric and adult populations It is caused by acquired or genetic dysregulation of the alternative complement pathway, resulting in excessive complement activation in the circulation and within the glomerular microenvironment Pathophysiologically, the disease is defined on renal biopsy by prominent, dominant deposition of complement component 3 (C3) with absent or minimal im...

It is caused by acquired or genetic dysregulation of the alternative complement pathway, resulting in excessive complement activation in the circulation and within the glomerular microenvironment Clinically, affected patients typically present with hematuria, proteinuria, nephritic or nephrotic syndrome, and progressive decline in kidney function that frequently advances to end-stage renal disease

Pathophysiologically, the disease is defined on renal biopsy by prominent, dominant deposition of complement component 3 (C3) with absent or minimal immunoglobulin staining, comprising two major histological subtypes: C3 glomerulonephritis and dense deposit disease