Table 1.
Demographic and clinical characteristics of patients with atypical anti-GBM disease.
| Characteristic | Total patients (N = 60) |
|---|---|
| Demography | |
| Male/female | 42/18 (2.3/1) |
| Age, year | 51.7 ± 15.6 |
| Clinical feature | |
| Interval from onset to diagnosis, month | 2.1 (1.1, 6.9) |
| Smoking, n (%) | 32 (53.3) |
| Prodromal infection, n (%) | 8 (13.3) |
| Hemoptysis, n (%) | 3 (5.0) |
| aAKD and bAKI, n (%) | 27 (45.0) |
| Oliguria/anuria, n (%) | 5 (8.3) |
| Hematuria, n (%) | 38 (63.3) |
| Macroscopic hematuria, n (%) | 4 (6.7) |
| Proteinuria, n (%) | 56 (93.3) |
| 24 h Proteinuria, g/24 h | 2.7 (0.8, 6.3) |
| Nephrotic level proteinuria, n (%) | 26 (43.3) |
| Nephrotic syndrome, n (%) | 19 (31.7) |
| Serum albumin, g/L | 34.2 (23.8, 41.2) |
| Serum creatinine on diagnosis, μmol/L | 142.5 (87.8, 257.5) |
| Renal insufficiency, n (%) | 32 (53.3) |
| Hemoglobin, g/L | 118.1 ± 26.3 |
| Serum C3, g/L* | 0.94 ± 0.26 (n = 52) |
| Serum C4, g/L* | 0.25 ± 0.07 (n = 52) |
| cESR, mm/h | 36.5 (17.0, 71.3) (n = 52) |
| dANCA, n (%) | 7 (14.0) (n = 50) |
| eMPO-ANCA/fPR3-ANCA/both | 6/1/0 |
| Treatment | |
| gACEIs/hARBs, n (%) | 26 (43.3) |
| Immunosuppressive therapy, n (%) | 34 (56.7) |
| steroids, n (%) | 32 (53.3) |
| cytotoxic drugs, n (%) | 18 (30.0) |
| Plasmapheresis, n (%) | 4 (6.7) |
| Outcome | |
| Follow-up duration, month | 35.7 ± 21.4 |
| Progression to ESRD, n (%) | 14 (23.3) |
| Death, n (%) | 9 (15.0) |
| 1-year renal survival, n (%) | 50 (83.3) |
| 1-year patient survival, n (%) | 57 (95.0) |
a
AKD, acute kidney disease;
b
AKI, acute kidney injury;
c
ESR, erythrocyte sedimentation rate;
d
ANCA, anti-neutrophil cytoplasmic antibodies;
e
MPO, Myeloperoxidase;
f
PR3, proteinase 3;
g
ACEIs, angiotensin converting enzyme inhibitors;
h
ARBs, angiotensin receptor blocker.
*
Normal range of serum C3: 0.6–1.5 g/L, normal range of serum C4: 0.12–0.36 g/L.