Table 3.

Disorders to Consider in the Differential Diagnosis of C3G

DisorderGene(s)MOIClinical Features of This Disorder
Overlapping w/C3GDistinguishing from C3G
Post-infectious glomerulonephritis 1NAAcquiredHematuria, proteinuria, nephritic syndrome, edema, ↓ serum C3, C3 glomerular deposition, subepithelial hump-like depositsPost-infection (throat or skin) often due to Group A hemolytic streptococcus bacterium; ↓ levels of C3 resolve w/in 3 mos; glomerular codeposition of C3 & IgG
Immune-complex MPGN 2NAAcquiredHematuria, proteinuria, C3 deposits, subendothelial & subepithelial deposits, progressive diseaseImmune complex-mediated, often low complement C4 levels; codeposition of C3 & IgG/IgM/C1q/C4 on IF
Juvenile acute non-proliferative glomerulonephritis 3NAAcquiredMesangial cell proliferation, subepithelial deposits on EMC3 levels typically remaining in lower limits of nl
Familial lecithin-cholesterol acyltransferase deficiency 4 LCAT ARESRD, glomerular pattern of IF similar to dense deposit diseaseAbnl lipoprotein (lipoprotein X); corneal opacities; normochromic anemia; capillary endothelial damage; cross-striated & vacuole structures
Partial lipodystrophy 5Several genesAD/ARLoss of subcutaneous fat in upper half of the bodyNo renal disease; no dysregulation of complement alternative pathway
Age-related macular degeneration 6Many genesADDrusenNo renal disease
Malattia Leventinese & Doyne honeycomb retinal dystrophy 7 EFEMP1 ADDrusenNo renal disease

From: C3 Glomerulopathy

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