Supplemental Table 1. The expression profiles of extracellular matrix-related genes in
the presence or absence of EBI3 in the PCR array
Symbol
Name
Fold change
Upregulated genes by EBI3
TGFBR2
Transforming growth factor, beta receptor 2
EGF
18.58
Epidermal growth factor
18.20
ITGB3
Integrin, beta 3
17.22
THBS1
Thrombospondin 1
14.48
DCN
Decorin
8.09
IL1A
Interleukin 1, alpha
7.04
SMAD family member 3
6.35
Matrix metallopeptidase 3
4.98
SMAD3
MMP3
GREM1
gremlin 1
4.16
LTBP1
Latent transforming growth factor beta binding protein 1
3.42
ITGA3
Integrin, alpha 3
2.80
PLAU
Plasminogen activator, urokinase
2.26
Downregulated genes by EBI3
ITGB1
COL3A1
JUN
Integrin, beta 1
Collagen, type 3, alpha 1
0.04
0.08
Jun proto-oncogene
0.12
ACTA2
Actin, alpha 2
0.13
SMAD4
SMAD family member 4
0.18
CEBPB
HGF
SERPINE1
CCAAT/enhancer binding protein, beta
Hepatocyte growth factor
0.20
0.25
Serpin peptidase inhibitor, clade E, member 1
0.27
PDGFA
Platelet-derived growth factor alpha polypeptide
0.27
ITGA1
Integrin, alpha 1
0.33
Lysyl oxidase
0.35
Collagen, type 1, alpha 2
0.38
LOX
COL1A2
TIMP3
Tissue inhibitor of metalloproteinases 3
0.41
MMP1
Matrix metallopeptidase 1
0.41
TIMP4
Tissue inhibitor of metalloproteinases 4
0.42
A mixture of equal amounts of mRNAs from 3 normal fibroblasts was prepared in the
presence or absence of EBI3, and mRNA expression profile was evaluated using PCR
array. Relative expression level was calculated as 1/2(the raw Ct of each mRNA – Ct of housekeeping
genes)
. Genes up- or downregulated more than 2-fold by EBI3 stimulation, which was
validated by real-time PCR, are shown.
Supplemental Table 2. Correlation of serum IL-35 levels with the clinical and
serological features in patients with systemic sclerosis
Patients with elevated
Patients with normal
IL-35 Levels (n=16)
IL-35 Levels (n=17)
Mean age at onset (y)
57.3
60.1
Mean duration of disease (y)
70.9
62.6
Type (diffuse:limited)
6:10
7:10
MRSS (point)
10.5
12.9
Pitting scars
43.8
47.1
Ulcers
25.0
29.4
Nailfold bleeding
43.8
58.8
Raynaud’s phenomenon
87.5
100.0
Telangiectasia
6.3＊
35.3
Contracture of phalanges
68.8
76.5
Calcinosis
0
5.9
Diffuse pigmentation
25.0
23.5
Short SF
43.8
52.9
Sicca symptoms
31.3
35.3
Mean percent VC
101.4
92.7
Mean percent DLco
79.8
79.8
Pulmonary hypertension
43.8
58.8
Esophagus
37.5
29.4
Heart
18.8
41.2
Kidney
0
11.8
Joint
31.3
23.5
Thrombosis
0
0
Clinical features
Organ involvement
Pulmonary fibrosis
ANA specificity
Anti-topoⅠ
31.3
23.5
Anti-centromere
43.8
52.9
Anti-U1 RNP
12.5
17.6
Unless indicated, values are percentages.
*P<0.05 versus patients with normal IL-35 levels using Fisher’s exact probability test.
ANA, anti-nuclear Abs; Anti-centromere, anti-centromere Ab; Anti-topo I, antitopoisomerase I Ab; Anti-U1 RNP, anti-U1 RNP Ab; DLco, diffusing capacity of lung
carbon monoxide; MRSS, modified Rodman skin thickness score; SF, sublingual
frenulum; VC, vital capacity.