Table 1: Summary of the reported genes associated with SSc.

Function Gene Replication Largest Size (SSc/Control) Reference
Immune Regulation
  HLA yes 1300/1000 for HLA alleles [8-23, 11, 12]]
  STAT4 yes 2753/4569 [11, 12, 25-27]
  CD247 yes 2753/4569 [11, 33]
  TBX21 replicated in a single study 880/507 [27]
T cell PTPN22 inconsistent in subtype association 1100/740 [37-39]
differentiation TNFSF4 yes 3014/3125 [43, 44]
proliferation IL23R inconsistent reports 1402/1038 [48, 49]
activation IL2RA no 3023/2735 [151]
  IL-21 no 4493/5856 [52]
  SCHIP1IL12A replicated in a single study 4017/5935 [137]
  CD226 replicated in a single study 991/1008 [55]
B cell signaling BANK1 yes 2380/3270 [57, 58]
C8orf13-BLK yes 1031/1014 [59-61]
innate immunity PLD4 no 315/21054 [63]
TLR-2 replicated in a single study 1170/925 [65]
NLRP1 replicated in a single study 1059/625 [70]
ATG5 replicated in a single study 4017/5935 [137]
Inflammation
interferon regulation IRF5 yes 2753/4569 [11, 12, 73-75]
IRF8 replicated in a single study  3175/4971 [123]
NF-κB signaling TNFAIP3 yes 1018/1012 [77-79]
IRAK1 inconsistent reports 3065/2630 [81, 82]
NFKB1 no 5270 /8326 [158]
TNIP1 replicated in a single study 564/1776 [12]
cytokine/chemokine FAS inconsistent genotypes 2900/3186 [87, 88]
MIF yes 3800/4282 [89, 90]
HGF no 314/103 [93]
OPN no 357/864 [98]
IL-6 no 2749/3189 [153]
CXCL8 no 151/147 [102]
CCR6 no 2411/7084 [105]
CTGF inconsistent reports 500/500 [107-111]
regulation of monocytes ITGAM inconsistent reports 4337/5326 [114-116]
regulation of cytokines CAV1 replicated in a single study 791/843 [119]
Transcription MECP2 replicated in a single study 3065/2630 [82]
SOX5 replicated in a single study  3175/4971 [123]
JAZF1 no 6835/14274 [165]
DNA cleavage DNASEIL3 replicated in a single study 4017/5935 [137]
XRCC1 no 177 case only [172]
XRCC4 no 177 case only [172]
Kinase activity PXK no 6835/14274 [165]
CSK no 5270/8326 [158]
GRB10 replicated in a single study  3175/4971 [123]
Cell differentiation NOTCH4 replicated in a single study  3175/4971 [123]
Cell morphogenesis RHOB replicated in a single study 564/1776 [12]
Unknown KIAA0319L no 6835/14274 [165]
PSD3 no 5270/8326 [158]
PSOR1C1 replicated in a single study 564/1776 [12]