Posted in Histopathology, Nephropathology

C3 Glomerulopathy- A new entity based on immunofluorescence

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 C3CFBCFHCFI 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.

C3 Glomerulopathy- A new entity based on immunofluorescence
C3 glomerulopathy showing characteristic findings. Full article

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.

C3 Glomerulopathy- A new entity based on immunofluorescence
Spectrum and differentials of C3 glomerulopathy. Full article.

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