2024年11月5日 · C3 glomerulopathy (C3G) is a rare glomerular disease stemming from dysregulation of the complement system's alternative pathway, resulting in excessive C3 protein deposits in the kidney’s glomeruli.

2007年7月20日 · C3 glomerulopathy (C3G) is a complex ultra-rare complement-mediated renal disease caused by uncontrolled activation of the complement alternative pathway (AP) in the fluid phase (as opposed to cell surface) that is rarely inherited in a simple mendelian fashion. C3G affects individuals of all ages, with a median age at diagnosis of 23 years.

2014年8月12日 · The term C3G includes dense deposit disease (DDD) and C3 glomerulonephritis (C3GN); these are two patterns of damage and inflammation in the glomeruli. In other words, the damage and inflammation in the kidney tissue in DDD looks different from that in C3GN when seen under a microscope.

2025年1月30日 · There is a further subdivision of complement 3 glomerulopathy into C3 glomerulonephritis (C3GN) and dense deposit disease (DDD). In this classification, dense deposit disease essentially replaced MPGN type II. MPGN type I and III now fall into C3 glomerulonephritis or immune complex glomerulonephritis.

2024年7月10日 · Immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) and complement 3 glomerulopathy (C3G) are rare, complement-mediated kidney diseases, previously classified under the group of kidney disorders termed membranoproliferative glomerulonephritis (MPGN) type 1, type 2, and type 3.

2024年11月18日 · C3G, C3 glomerulopathy; IC-MPGN, immune-complex membranoproliferative glomerulonephritis. Pegcetacoplan is a pegylated cyclic peptide that inhibits selectively C3 and C3b, thereby blocking both C3- and C5-convertases.

In this review, we provide an overview of C3G, as well as summarizing the evidence for current treatments and detailing the clinical trials that are currently underway. C3 glomerulopathy (C3G) is an ultra-rare cause of glomerular kidney disease, driven by dysregulation of the alternative pathway of the complement system.

1 天前 · C3G shares features with conditions such as IgA nephropathy (IgAN), immune complex–mediated membranoproliferative glomerulonephritis (IC-MPGN), and lupus nephritis (LN). Key distinctions include ...

1 天前 · The hallmark of C3G is predominant C3 deposition in kidney biopsy samples with minimal or no immunoglobulin deposits. This distinct immunofluorescence finding is essential for accurate diagnosis and differentiates C3G from immune complex–mediated diseases such as lupus nephritis and membranoproliferative glomerulonephritis.

2025年1月8日 · The pathogenesis of C3G centers on the dysregulation of the alternative complement pathway, often due to autoantibodies known as C3, C4, or C5 nephritic factors, or genetic mutations in key complement regulatory proteins.