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. 2017 Oct 13;29(1):283–294. doi: 10.1681/ASN.2017030258

Table 3.

Clinical features, complement assessment, genetic screening, and histologic features in patients classified according to the clusters obtained through cluster analysis

Variable Cluster 1 Cluster 2 Cluster 3 Cluster 4 Overall P Value
N 62 32 33 46
Sex, % men 61 41 55 61 0.24
Data at onset
 Age, yr 15 (±11.6) 17.2 (±13) 14.5 (±10.4) 28.8 (±19.8)a,b,c <0.001
 Microhematuria, % 90 93 94 64a,b,c <0.001
 Gross hematuria, % 37 34 50 20a,c 0.04
 Proteinuria, % 93 94 85 89 0.51
 Nephrotic syndrome, % 29 72a,c,d 24 24 <0.001
 Renal impairment, % 10 19 6 39a,c <0.001
 Trigger event, % 38 31 23 22 0.29
 Familiarity for nephropathy, % 23c 16 3 11 0.05
Serum C3, mg/dl 34.7 (±30.2) 34.5 (±37.9) 31 (±25.6) 86.7 (±35.6)a,b,c <0.001
Serum C4, mg/dl 21.1 (±10.3) 19.1 (±13) 23.5 (±7.9) 23.6 (±7.5) 0.17
Plasma SC5b-9, ng/ml 1494 (±1325)c,d 1558 (±1285)c,d 591 (±1062) 416 (±395) <0.001
Low serum C3 and normal C4, % 81 72c 94 50a,b,c <0.001
LPV carriers, % 32 22 15 4a,b 0.004
C3NeF positive, % 53 53 75 9a,b,c <0.001
LPV carriers and/or C3NeF, % 75 63 79 14a,b,c <0.001
Data during follow-up, %
 Nephrotic syndrome 52 88a,c,d 52 50 0.003
 High BP 26 44 27 63a,c <0.001
 CKD 18b,c,d 47 36 54 <0.001
 ESRD 2d 6 6 22 0.003
 Thrombotic microangiopathy 2 0 0 13a,c <0.01
Histologic features
 Time onset to biopsy, yr, median (IQR) 0.6 (0.2–3.6) 0.2 (0.1–1.7) 0.6 (0.2–1.8) 0.4 (0.0–3.3) 0.31
Light microscopy
 Sclerotic glomeruli, % 4 (±8) 8 (±15)c 1 (±3) 17 (±21)a,b,c <0.001
 Crescents, % 1 (±4) 3 (±9) 18 (±31)a,b,d 4 (±10)a <0.001
 Degree of mesangial proliferatione 2 (±0.9) 1.4 (±1.1)a,c 2 (±0.8) 1.7 (±0.9) 0.01
 Degree of endocapillary proliferatione 1.2 (±1.1) 1.6 (±1) 1.3 (±1.2) 0.5 (±0.8)a,b,c <0.001
 Degree of interstitial inflammatione 0.5 (±0.6)b,d 0.8 (±0.6) 0.6 (±0.8) 1 (±1)c <0.001
 Degree of interstitial fibrosise 0.3 (±0.6) 0.5 (±0.6) 0.3 (±0.6) 0.8 (±0.9)a,c <0.001
 Degree of arteriolar sclerosise 0.2 (±0.5) 0.2 (±0.5) 0 (±0.2) 0.6 (±1)a,b,c <0.001
IFe
 C3 2.7 (±0.5) 2.7 (±0.5) 2.8 (±0.3) 2.5 (±0.7)a,c 0.03
 IgA 0.1 (±0.4) 0.7 (±0.8)a,c,d 0.1 (±0.3) 0.2 (±0.7) <0.001
 IgG 0.4 (±0.7)d 2 (±0.9)a,c,d 0.5 (±0.8) 1 (±1.2) <0.001
 IgM 0.7 (±0.8) 1.2 (±0.9) 0.8 (±0.7) 0.8 (±1) 0.12
 C1q 0.3 (±0.5)d 1.6 (±0.9)a,c,d 0.3 (±0.6) 0.6 (±1) <0.001
 Fibrinogen 0.4 (±0.8) 0.6 (±0.9) 0.3 (±0.8) 0 (±0.1)a,b,c 0.01
Electron microscopy, %
 Mesangial deposits 83b,c,d 52 44 53 <0.001
 Subepithelial deposits 57c,d 38 19 36 0.004
 Subepithelial hump-like deposits 20 14 19 16 0.88
 Subendothelial deposits 84 93 9a,b,d 66a,b <0.001
 Intramembranous granular deposits 59 41 16a,b,d 41 <0.001
 Intramembranous highly electron-dense ribbon-like deposits 7 0 73a,b,d 0 <0.001

Quantitative variables are expressed as mean (±SD) unless otherwise specified. Serum C3: reference 90–180 mg/dl; serum C4: reference 10–40 mg/dl; plasma SC5b-9: reference ≤400 ng/ml. IQR, interquartile range.

a

Significantly different versus cluster 1.

b

Significantly different versus cluster 2.

c

Significantly different versus cluster 3.

d

Significantly different versus cluster 4.

e

Degrees of mesangial proliferation, endocapillary proliferation, interstitial inflammation, interstitial fibrosis, and arteriolar sclerosis as well as IF findings were graded using a scale of 0–3, including 0, trace (0.5+), 1+, 2+, and 3+.