Table 1.
Baseline patient characteristics
| Patients (n) | 14 |
| female sex (n, [%]) | 8 (57.1) |
| kidney transplant at diagnosis (n, [%]) | 6 (42.9) |
| age at diagnosis (years, [IQR]) | 27.0 (20.75–57.25) |
| time diagnosis to eculizumab (months, [IQR])a | 11.0 (4.5–105.0) |
| eGFR at treatment start (mL/min/1.73 m2, [IQR]) | 40.59 (31.39–72.48) |
| UPCR at treatment start (g/g [IQR]) | 2.51 (0.75–5.21) |
| serum albumin at treatment start (g/dl, [IQR]) | 3.73 (3.08–4.21) |
| urine blood at treatment start (dipstickb, 0–5, [IQR]) | 3.0 (3.0–4.0) |
| eculizumab treatment duration (months, [IQR]) | 10.0 (5.0–52.5) |
| Histopathology findings | |
| glomerula (n, [IQR]) | 15.5 (12.0–20.0) |
| global sclerosis (%, [IQR]) | 7 (5–25) |
| partial sclerosis (%, [IQR]) | 0 (0–0) |
| IF/TA (%, [IQR]) | 10 (5–30) |
| crescents (%, [IQR]) | 0 (0–0) |
| mesangial proliferation (n, [%]) | 13 (92.9) |
| leukocyte infiltration (0–3, [IQR]) | 1 (0–1) |
| Immunohistochemistry findingsc | |
| C3 (0–3, [IQR]) | 3 (2–3) |
| C4d (0–3, [IQR]) | 0 (0–0) |
| C5b-9 (0–3, [IQR]) | 1 (1–2) |
| IgA (0–3, [IQR]) | 0 (0–1) |
| IgG (0–3, [IQR]) | 0 (0–1) |
| IgM (0–3, [IQR]) | 1 (1–2) |
| Electron microscopy findings | |
| DDD typical deposits (n, [%]) | 4 (30.8) |
EGFR, UPCR, hematuria, serum albumin levels are median values and interquartile ranges in the first month of eculizumab treatment. a Time from diagnosis to eculizumab initiation indicates the time from first diagnosis to first eculizumab treatment. b hematuria measured by urine dipstick (scale from 0 to 5). c Data are graded on a range from 0 to 3 based on staining intensity
Abbreviation: IQR Interquartile range