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Table 3 Clinical and complement characteristics of IC-MPGN/C3G patients classified based on their nephritic factor status

From: C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy

 C3NeF positive patients
n = 20
C4NeF positive patients
n = 10
Double positive patients for C3NeF and C4NeF
n = 7
Double negative patients
n = 82
p
sex % men13 (65)6 (60)3 (42.9)45 (54.9)0.057
age at diagnosis15 (9–21)114 (11–39)16 (11–17)28 (13–41)0.036
microhematuria, present12 (60)7 (70)4 (80)47 (58.8)0.735
gross hematuria, present6 (30)2 (20)0 (0)15 (18.8)0.461
nephrotic syndrome, present12 (60)5 (50)2 (28.6)42 (53.2)0.552
renal impairment, present9 (45)1 (10)0 (0)35 (44.3)2,30.026
renal failure, present2 (10)1 (10)0 (0)9 (11.4)0.824
trigger, present8 (40)1 (11.1)1 (14.3)11 (13.8)0.045
familiarity, present2 (10)1 (11.1)0 (0)7 (8.6)0.863
serum C3(0.9–1.8 g/L)0.34 (0.2–0.77)10.52 (0.25–0.96)0.2 (0.17–1.07)0.84 (0.48–1.04)0.01
serum C4(0.15–0.55 g/L)0.25 (0.13–0.33)0.23 (0.19–0.28)0.15 (0.11–0.26)0.22 (0.17–0.31)0.57
sC5b-9(110–252 ng/mL)575 (384–1206)1287 (115–1063)1716 (1450–2127)1,2368 (244–553)0.0004
LPV carriers5 (27.7)3 (50)1 (14.3)13 (16.5)0.19
Classical pathway activity(48–103 CH50/mL)36 (12.5–59)39 (18–59)14 (0–46)47.5 (31–61)0.077
Alternative pathway activity (70–125%)1.5 (1–89)57.5 (0.75–68.5)1 (0–58)65 (13–86)3,40.033
C1q (60–180 mg/L)108 (93–146)111 (80.25–145)104 (73–124.5)101 (76–121.5)0.46
Factor H (250–880 mg/L)573.5 (360–697)546.5 (360–639)495 (384–763)538 (370–742)0.977
Factor I (70–130%)92.5 (78–103)82 (67–102.5)90 (78–107)92.5 (78–111)0.598
Factor B(70–130%)85 (65–98.5)84 (65–107)86 (66–107)86 (65.5–103)0.971
Factor D (0.51–1.59 μg/mL)2.15 (1.06–3.72)1.83 (0.78–5.28)0.48 (0.33–2.5)2.44 (0.98–4.13)0.16
C3a (70–270 ng/mL)113 (77–274)124 (72–190)138 (53–188)137 (91–221)0.805
Bb (0.49–1.42 μg/mL)2.24 (1.52–3.4)0.93 (0.89–2.27)1.14 (0.71–2.37)1.45 (1.01–2.08)0.079
C4d (0.7–6.3 μg/mL)3.97 (3.25–8.9)3.23 (2.66–4.41)3.71 (0.59–6.2)5.77 (3.04–9.27)0.21
anti-Factor H, present0 (0)0 (0)0 (0)6 (7.3)0.359
anti-C1q, present2 (10)1 (10)3 (25.2)28 (9.7)0.044
anti-C3, present1 (5)0 (0)2 (28.57)12 (2.5)0.011
anti-Factor B, present2 (10)0 (0)0 (0)4 (5)0.608
Any additional antibody to C3NeF and/or C4NeF3 (16.6)1 (11.1)4 (57.14)315 (19.73)0.09
  1. Reference ranges are shown in the first coloumn in parentheses. The data are given as median and interquartile range or number and percentages. P-values are given as the results of χ2 or Kruskal-Wallis tests
  2. 1 Significantly different from nephritic factor negative patients.2 Significantly different from C4NeF positive patients.3 Significantly different from double positive patients. 4Significantly different from C3NeF positive patients. LPV: likely pathogenic variant; C3NeF: C3 nephritic factor; C4NeF: C4 nephritic factor.
  3. There are some missing values in the following data: proteinuria (n = 3), renal impairment/failure (n = 3), trigger (n = 2), familiarity (n = 1), sC5b-9 (n = 15), LPV (n = 9), C1q (n = 13), Factor D (n = 23), C3a (n = 19), Bb (n = 23), C4d (n = 23), anti-Factor H (n = 2), anti-C1q (n = 8), anti-C3 (n = 3), anti-Factor B (n = 3)
  4. Significance level was determined at a value of p < 0.05