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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 % men

13 (65)

6 (60)

3 (42.9)

45 (54.9)

0.057

age at diagnosis

15 (9–21)1

14 (11–39)

16 (11–17)

28 (13–41)

0.036

microhematuria, present

12 (60)

7 (70)

4 (80)

47 (58.8)

0.735

gross hematuria, present

6 (30)

2 (20)

0 (0)

15 (18.8)

0.461

nephrotic syndrome, present

12 (60)

5 (50)

2 (28.6)

42 (53.2)

0.552

renal impairment, present

9 (45)

1 (10)

0 (0)

35 (44.3)2,3

0.026

renal failure, present

2 (10)

1 (10)

0 (0)

9 (11.4)

0.824

trigger, present

8 (40)

1 (11.1)

1 (14.3)

11 (13.8)

0.045

familiarity, present

2 (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)1

0.52 (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)1

287 (115–1063)

1716 (1450–2127)1,2

368 (244–553)

0.0004

LPV carriers

5 (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)3,4

0.033

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, present

0 (0)

0 (0)

0 (0)

6 (7.3)

0.359

anti-C1q, present

2 (10)

1 (10)

3 (25.2)2

8 (9.7)

0.044

anti-C3, present

1 (5)

0 (0)

2 (28.57)1

2 (2.5)

0.011

anti-Factor B, present

2 (10)

0 (0)

0 (0)

4 (5)

0.608

Any additional antibody to C3NeF and/or C4NeF

3 (16.6)

1 (11.1)

4 (57.14)3

15 (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
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172