Table 3:
Clinical data at diagnosis and outcomes of 101 adults based on biomarkers of convertases activity
| Low C3 normal sC5b-9 |
low C3 high sC5B-9 |
Normal C3 normal sC5b-9 |
Normal C3 High sC5b-9 |
p value | |
|---|---|---|---|---|---|
| Patients (Nb) | 20 (19.8%) | 37 (36.6%) | 30 (29.7%) | 14 (13.8%) | 0.0008 |
| Clinical data at diagnosis | |||||
| Sex (% of male) | 9/20 (45%) | 22/37 (59%) | 18/30 (60%) | 8/14 (57%) | 0.71 |
| Age (years) | 33[21–48] | 25[20–42] | 39 [25–64] | 41[24–62] | 0.04 |
| Proteinuira (g/day) | 3[2.5–6.2] | 4.4 [2.3–7.6] | 3.3[2.2–6.7] | 3.5 [1.2–9.1] | 0.85 |
| Nephrotic range proteinuria | 8/18 (44%) | 18/32 (56%) | 12/27 (44%) | 5/10 (50%) | 0.79 |
| Estimated GFR (ml/min per 1.73 m2) | 42 [17–100] | 86 [36–100] | 64 [32–98] | 31 [17–58] | 0.08 |
| Stage 3–5 CKD n (%) | 11/26 (42%) | 12/30 (40%) | 14/29 (48%) | 9/12 (75%) | 0.20 |
| Immunological data at diagnosis | |||||
| C3 level (660 to 1250 mg/L) | 463 [213–576] | 314 [182–475] | 913 [722–1015] | 1034 [777–1250] | <0.0001 |
| Low C3 level (<660 mg/L) (%) | 100% | 100% | 0 (0%) | 0 (0%) | <0.0001 |
| Soluble C5b-9 (ng/mL) | 273 [234–390] | 964[783–1803] | 317 [220–399] | 630 [502–682] | <0.0001 |
| High sC5b-9 (>460ng/mL) (%) | 0 (0%) | 100% | 0% | 100% | <0.0001 |
| C4 level (93 to 380 mg/L) | 221 [149–269] | 215[179–280] | 264[196–317] | 254[191–334] | 0.15 |
| Factor H level (% of normal) | 105 [67–115] | 103 [78–124) | 106 [89–124] | 114 [101–138] | 0.43 |
| Factor I level (% of the normal) | 98 [92–111] | 104 [86–125] | 109 [93–120] | 119 [97–140] | 0.096 |
| C3NeF n (%) | 12/20 (60%) | 22/36(61%) | 3/30 (10%) | 3/13 (23%) | <0.0001 |
| % of stabilisation | 60 [33–92] | 49 [33–81] | 26 [26–26] | 36 [21–456] | 0.52 |
| C5NeF n (%) | 2/10 (20%) | 11/23 (48%) | 0/13 (0%) | 2/5 (40%) | 0.02 |
| Anti-FH auto-antibodies n (%) | 4/19 (21%) | 3/36 (8%) | 0/29 (0%) | 1/14 (7%) | 0.08 |
| Rare/pathogenic variant n (%) | 2/18 (11%) | 5/32 (16%) | 6/23 (26%) | 4/14 (29%) | 0.15 |
| First line therapy | |||||
| Immunosuppressive treatment n (%) | 9/15 (60%) | 23/31 (74%) | 9/21 (43%) | 7/12 (58%) | 0.11 |
| Monoclonal anti-C5 n (%) | 4/9(44%) | 5/23 (22%) | 1/9 (11%) | 1/7 (14%) | 0.34 |
| Follow-up | |||||
| Median Follow-up (months) | 48 [8–72] | 51 [22–89] | 30 [12–70] | 21 [3–40] | 0.13 |
| ESKD n (%) | 11/19 (58%) | 13/37 (35%) | 4/30 (13%) | 7/14 (50%) | 0.004 |
| Duration of evolution until ESKD (months) | 26 [1–76] | 44[18–89] | 37 [14–99] | 3 [1–20] | 0.059 |
Abbreviations: CKD, chronic kidney disease stage, FH, factor H, sC5b9: soluble C5b9, IQR, interquartile, ESKD, end stage kidney disease; eGFR, estimated Glomerular Filtration Rate, MPGN, membranoproliferative glomerulonephritis.