DEFINITION : C3 glomerulopathy is a recently defined entity that encompasses a group of kidney diseases caused by abnormal control of complement activation with deposition of complement component C3 in glomeruli leading to variable glomerular inflammation.
TYPES: It is important to understand that, C3 glomerulopathy involves a spectrum of disorders.
1. DENSE DEPOSIT DISEASES
2. C3 GLOMERULONEPHRITIS
3. RARE CONDITIONS SUCH AS Complement factor H -R5 deficiency
4. OTHERS – Genetic variants in the C3, CFB, CFH, CFI and CFHR1–CFHR5 genes are potentially causal; both rare and common variants can coexist and are associated with susceptibility to disease.
Complement Factor H is isajor negative regular of alternative complement pathway.
DIFFERENTIAL DIAGNOSIS OF C3 GLOMERULOPATHY
1. Post- infectious glomerulonephritis- 30 % have C3 deposits alone. Hence clinical history can differentiate between the two.
2. Para-protein associated glomerulonephritis. Complement dysregulation may be caused due to paraproteins. Hence differentiation based on renal biopsy and Immunofluorescence alone is not possible.
3. Membranoproliferative glomerulonephritis
4. Cresentic glomerulonephritis.
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