Table 2 Histo-pathology, genetic and autoantibody testing
C3GN1 | C3GN2 | C3GN3 | DDD1 | C3GN4 1st biopsy | C3GN4 2nd biopsy | DDD2 | C3GN5 1st biopsy | C3GN5 2nd biopsy | |
|---|---|---|---|---|---|---|---|---|---|
Light microscopy | |||||||||
Glomerula (n) | 20 | 15 | 3 | 8 | 25 | 13 | 11 | 24 | 19 |
Global sclerosis (%) | 5 | 7 | 0 | 0 | 56 | 38 | 9 | 4 | 16 |
Partial sclerosis (%) | 0 | 0 | 0 | 0 | 0 | 15 | 91 | 13 | 0 |
IF/TAa (%) | 5 | 10 | 0 | 5 | 5 | 25 | 30 | 10 | 10 |
Crescents (%) | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
Mesangial proliferation | pos | pos | pos | pos | pos | pos | neg | pos | pos |
Leukocyte infiltrationb (0–3) | 2 | 2 | 1 | 0 | 1 | 2 | 0 | 1 | 0 |
Immunofluorescencec | |||||||||
C3 (0–3) | 3 | 2 | 3 | 2 | 2 | 3 | 2 | 3 | 3 |
C4d (0–3) | NT | NT | 0 | NT | 0 | 0 | NT | NT | NT |
C5b-9 (0–3) | 2 | 1 | 2 | NT | 1 | 1 | 1 | NT | 2 |
IgA (0–3) | 1 | 1 | 0 | 1 | 0 | 0 | 1 | 0 | 0 |
IgG (0–3) | 1 | 0 | 1 | 1 | 0 | 0 | 0 | 1 | 2, κ light chain, mouse pos |
IgM (0–3) | 1 | 2 | 1 | 1 | 1 | 1 | 2 | 1 | 1 |
Electron microscopy | |||||||||
DDD deposits | neg | neg | neg | pos | neg | neg | pos | NT | neg |
Genetic analysis | |||||||||
CFH | NT | NT | neg | neg | neg | neg | neg | ||
CFI | NT | neg | neg | NT | HET c.1322A > G | neg | neg | ||
CFB | NT | NT | NT | NT | NT | neg | NT | ||
CFHR1–5 | NT | NT | neg | NT | HET del cfhr1, 3 | HET CFHR5 c.832G > A | neg | ||
MCP | NT | neg | neg | NT | neg | neg | neg | ||
C3 | NT | NT | HET c.2203C > T | neg | neg | neg | neg | ||
COL4A3 | NT | NT | NT | NT | HET c.4484A > G | NT | NT | ||
Autoantibodies | |||||||||
C3Nef | pos | pos | neg | neg | pos | neg | pos | ||
C3b | NT | pos | NT | NT | NT | NT | NT | ||
CFH | pos | pos | NT | NT | neg | neg | NT | ||
CFI | neg | NT | NT | NT | neg | NT | NT | ||