Table 2.
Pathological characteristics of patients with atypical anti-GBM disease.
| Characteristic | Total patients (N = 60) |
|---|---|
| Immunofluorescence | |
| IgG linear deposition, n (%) | 60 (100.0) |
| Intensity (scale 0~4+) | 1.0 (1.0, 1.5) |
| Location (GBM/aTBM/Bowman's capsules), n (%) | 60/37/5 (100.0/61.7/8.3) |
| IgG subclass (n = 59) | |
| IgG1/IgG2/IgG3/IgG4, n (%) | 27/21/7/11 (45.8/35.6/11.9/18.6) |
| IgA deposit, n (%) | 27 (45.0) |
| IgM deposit, n (%) | 33 (55.0) |
| C3 deposit, n (%) | 39 (65.0) |
| C1q deposit, n (%) | 10 (16.7) |
| bFRA deposit, n (%) | 20 (33.3) |
| Albumin deposit, n (%) | 38 (63.3) |
| Light microscopy | |
| Number of glomeruli | 25.0 (19.3, 36.0) |
| Crescent formation, n (%) | 25 (41.7) |
| Percentage of crescents, % | 27.3 (0.0, 49.7) |
| cTA/IF, n (%) | 58 (96.7) |
| Electron microscopy | |
| Electric dense deposit, n (%) | 33 (55.9) (n = 59) |
| Combined dGN, n (%) | 35 (58.3) |
| eIgAN (including fHSP-GN), n (%) | 12 (20.0) |
| gMN, n (%) | 8 (13.3) |
| hMPGN, n (%) | 6 (10.0) |
| iAAV, n (%) | 4 (6.7) |
| jFSGS, n (%) | 3 (5.0) |
| kTBMN n (%) | 1 (1.7) |
| lTMA, n (%) | 1 (1.7) |
a
TBM, tubular basement membrane;
b
FRA, fibrinogen-fibrin related antigens;
c
TA/IF, tubular atrophy and interstitial fibrosis;
d
GN, glomerulonephritis;
e
IgAN, IgA nephropathy;
f
HSP-GN, Henoch–Schönlein purpura glomerulonephritis;
g
MN, membranous nephropathy;
h
MPGN, membranoproliferative glomerulonephritis;
i
AAV, ANCA-associated vasculitis;
j
FSGS, focal segmental glomerulosclerosis;
k
TBMN, thin basement membrane nephropathy;
l
TMA, thrombotic microangiopathy.