C3 Glomerulopathy: The Asia Pacific Clinical Trial Landscape

Acute Glomerulonephritis (GN) is an inflammatory condition typically characterized by edema, hematuria, hypertension, and acute kidney injury. Glomerulonephritis can be broadly categorized as immune-complex glomerulonephritis (including infection-related glomerulonephritis, IgA nephropathy, lupus nephritis, and cryoglobulinemic glomerulonephritis), anti-neutrophil cytoplasmic antibodies associated glomerulonephritis, anti-glomerular basement membrane glomerulonephritis, C3 glomerulopathy, and monoclonal immunoglobulin-associated glomerulonephritis. Periods of exacerbation can occur in all forms of glomerulonephritis, but those with IgA nephropathy or C3 glomerulopathy are more likely to experience flare-ups of the disease. (1)

C3 Glomerulopathy (C3G) is a complex, rare complement-mediated renal disease. It is caused by uncontrolled activation of the complement alternative pathway (AP), degradation, or deposition, predominantly of the C3 fragments within the glomerulus leading to glomerular damage. Researchers have identified two major forms of C3G: C3 glomerulonephritis (C3GN) and dense deposit disease (DDD). All ages are affected by C3G, and the median age at diagnosis is 23. Haematuria, proteinuria, acute nephritic syndrome, and low levels of the complement C3 are common symptoms of C3G. Around half of those affected with C3G develop endstage renal disease (ESRD) within ten years of diagnosis, and occasionally, late comorbidity of decreased visual acuity. (2)