Table 2.
Kidney biopsy findings of EXT1/EXT2-associated MN
| Case | Glomeruli/Sclerosed | Mesangial or Endocapillary Hypercellularity | Interstitial Inflammation/IFTA | Arteries | Immunofluorescence Microscopy | Electron Microscopy Deposits SE/SU/ME | Tubuloreticular Inclusion |
|---|---|---|---|---|---|---|---|
| 1 | 10/0 | Not present | 0/0 | Moderate sclerosis | IgG (3+) C1q (1+) C3 (3+) | +/−/+ | + |
| 2 | 22/0 | Not present, two small crescents | 0/0 | Normal | IgG (3+) IgA (3+) IgM (1+) C1q (2+) C3 (3+) | +/−/+ | + |
| 3 | 8/2 | Not present | 0/0 | Normal | IgG (2+) IgM (1+) C3 (1+) | +/−/− | – |
| 4 | 38/0 | Not present | 0/0 | Normal | IgG (3+) IgM (1+) C3 (2+) | +/−/+ | + |
| 5 | 19/1 | Not present | 0/0 | Normal | IgG (3+) IgA (1+) IgM (1+) C1q (1+) C3 (3+) | +/+/+ | − |
| 6 | 7/0 | Not present | 0/0 | Normal | IgG (3+) IgA (2+), C1q (1+) C3 (2+) | +/+/+ | + |
| 7 | 64/0 | Not present | 0/0 | Normal | IgG (3+) C3 (1+) | +/+/+ | + |
| 8 | 23/1 | Not present | 0/10 | Normal | IgG (3+) IgA (2+) IgM (1+) C1q (2+) C3 (3+) | +/+/+ | + |
| 9 | 26/6 | Not present | 0/0 | Normal | IgG (3+) IgM (1+) C3 (2+) | +/−/+ | − |
| 10 | 48/4 | Not present | 0/10 | Normal | IgG (3+) IgA (2+) IgM (1+) C1q (1+) C3 (2+) | +/−/+ | + |
| 11 | 29/5 | Not present | 0/0 | Normal | IgG (2+) IgM (1+) C3 (3+) | +/−/+ | − |
| 12 | 54/18 | Not present | 10/30 | Mild sclerosis | IgG (3+) IgM (1+) C1q (1+) C3 (3+) | +/−/a | + |
| 13 | 18/1 | Not present | 0/0 | Normal | IgG (3+) IgA (2+) IgM (1+) C1q (2+) C3 (3+) | +/−/+ | + |
| 14 | 12/0 | Not present, three small crescents | 0/20 | Normal | IgG (3+) IgM (1+) C1q (2+) C3 (3+) | +/−/+a | + |
| 15 | 12/2 | Not present | 25/25 | Normal | IgG (3+) IgM (1+) C1q (1+) C3 (3+) | +/−/+a | + |
| 16 | 23/1 | Not present | 0/0 | Normal | IgG (3+) C3 (3+) | +/−/+ | + |
| 17 | 17/0 | Not present | 0/0 | Normal | IgG (3+) IgA (2+) C1q (1+) C3 (3+) | +/−/+ | + |
| 18 | 4/0 | Not present | 0/0 | Normal | IgG (3+) IgM (2+) C1q (1+) C3 (3+) | +/+/+ | + |
| 19 | 14/0 | Not present | 0/0 | Normal | IgG (2+) C3 (2+) | +/−/- | − |
| 20 | 18/2 | Not present | 0/0 | Normal | IgG (3+) IgA (2+) IgM (1+) C1q (2+) C3 (3+) | +/−/+ | + |
| 21 | 20/1 | Not present | 0/0 | Normal | IgG (3+) IgA (1+) IgM (1+) C1q (2+) C3 (3+) | +/−/+ | + |
| 22 | 17/9 | Not present | 0/80 | Severe sclerosis | IgG (3+) IgM (2+) C1q (1+) C3 (3+) | +/+/+ | + |
| 23 | 14/0 | Present | 10/30 | Normal | IgG (3+) IgA (3+) IgM (3+) C1q (2+) C3 (3+) | +/+/+ | + |
| 24 | 19/2 | Not present | 0/0 | Normal | IgG (3+) IgA (3+) IgM (3+) C1q (2+) C3 (3+) | +/+/+a | + |
| 25 | 27/4 | Present | 0/10 | Moderate | IgG (3+) IgM (1+) C1q (2+) C3 (3+) | +/+/+ | + |
| 26 | 16/1 | Not present | 0/25 | Normal | IgG (3+) IgM (1+) C1q (2+) C3 (2+) | +/−/+ | + |
IFTA, interstitial fibrosis and tubular atrophy; SE, subepithelial; SU, subendothelial; ME, mesangial.
a
Tubular basement membrane deposits.