Table 2 SSc-associated autoantibodies observed in this study
From: Stress granules and RNA processing bodies are novel autoantibody targets in systemic sclerosis
Alias | Associated proteins | Disease subset | Clinical associations | Prevalence in this dataset (avg/freq) | Reference | |||
|---|---|---|---|---|---|---|---|---|
Control (n = 4) | RNAP3 (n = 4) | TOP1 (n = 4) | CENP (n = 5) | |||||
Major autoantibodies | ||||||||
RNA Pol III | POLR3A | dSSc | Renal crisis, cancer | - | +++ (28/4) | - | - | |
Scl70 | TOP1 | dSSc | Poor prognosis, internal organ involvement, and proteinuria | + (3/2) | + (4/4) | +++ (19/4) | - | Mehra, et al. 2013 [3] |
Centromere | CENPB, CENPH | lSSc/CREST | PAH, ILD | - | - | - | + (1/2) | Mehra, et al. 2013 [3] |
Other SSc autoantibodies present in our dataset | ||||||||
Endothelial Cell | TUBB, VCL, LMNA, RPLP0 | SSc | PAH | + (1/1) | ++ (6/4) | + (4/2) | + (0/1) | |
Fibroblast | ENO1, G6PD, HSPA1A, HSPA1B, VIM | SSc | PAH | + (3/4) | +++ (12/4) | ++ (5/3) | ++ (8/5) | |
Histone | H1FX, HIST1H1B, HIST1H4A | SSc | PF, internal organ involvement, decreased survival | + (1/1) | + (3/3) | + (1/1) | - | Mehra, et al. 2013 [3] |
B23 | NPM1 | dSSc, CENP- lSSc | PAH | + (4/4) | ++ (7/4) | ++ (5/4) | ++ (6/5) | Mehra, et al. 2013 [3] |
Ku | XRCC5, XRCC6 | lSSc | Myositis | + (3/3) | +++ (12/4) | ++ (8/4) | + (2/3) | |
Su | AGO2 | SSc, PM/Scl | Unknown | - | + (1/2) | + (3/1) | - | Satoh, et al. 2013 [38] |
Mitochondrial (M2) | DLD, PDHB | lSSc | Strong association with primary biliary cirrhosis | + (1/1) | + (2/3) | + (1/1) | - | Mehra, et al. 2013 [13] |
Pm/Scl | EXOSC1-10 | SSc | PF, digital ulcers; decreased risk of PAH and GI symptoms | + (2/2) | ++ (5/3) | + (2/2) | - | Mehra, et al. 2013 [13] |
hnRNPs | HNRNPA1-3, HNRNPL | SSc | Common in SARDs | + (0/1) | ++ (7/4) | + (3/4) | + (2/4) | Siapka, et al. 2007 [39] |
U1 | SNRNPA, SPRNP70 | SSc | Co-occurrence with SS-A/SS-B, PAH, overlap syndrome | - | + (2/4) | + (1/2) | + (0/1) | |
U5 | SNRNP200 | SSc, PM/Scl | Unknown | ++ (6/3) | ++ (9/4) | ++ (8/3) | + (1/2) | Kubo, et al. 2002 [40] |
RO52/TRIM21 | TRIM21 | SSc | ILD, other autoimmune diseases | ++ (6/3) | +++ (12/4) | ++ (6/4) | ++ (8/4) | Mehra, et al. 2013 [3] |
RuvB | RUVBL1, RUVBL2 | dSSc | Common in SARDs, older age at onset, male sex | + (1/1) | ++ (7/4) | + (3/4) | + (2/4) | Kaji, et al. 2014 [18] |
Annexin V | ANXA5 | dSSc, CENP- lSSc | Digital ischemia | + (2/2) | ++ (7/4) | + (4/3) | + (3/4) | Mehra, et al. 2013 [3] |
SS-B/LA | SS-A, SS-B | SSc | ILD, other autoimmune diseases | - | + (3/4) | + (2/2) | + (0/1) | Mehra, et al. 2013 [3] |
Peroxiredoxin | PRDX1 | SSc | Disease duration, PF, cardiac involvement, TOP1+ patients | + (2/4) | ++ (8/4) | + (3/3) | + (4/4) | Mehra, et al. 2013 [3] |
hUBF/NOP90 | UBTF | lSSc | Mild organ involvement, favorable prognosis | - | + (1/2) | - | - | Mehra, et al. 2013 [3] |
Th/To | POP1 | lSSc | PF, renal crisis, poor prognosis, myositis, PAH | + (1/2) | + (1/1) | + (3/3) | - | |
PL-12 | AARS | SSc, PM/DM | ILD without myositis | - | - | + (1/1) | + (1/1) | Hamaguchi, et al. 2013 [19] |
OJ | IARS | SSc, PM/DM | ILD without myositis | + (1/1) | - | + (3/3) | - | Hamaguchi, et al. 2013 [19] |
EJ | GARS | SSc, PM/DM | ILD, myositis | - | + (3/4) | + (2/2) | + (0/1) | Hamaguchi, et al. 2013 [19] |
Jo-1 | HARS | SSc, PM/DM | ILD, myositis | - | + (1/4) | - | - | Hamaguchi, et al. 2013 [19] |
PL-7 | TARS | SSc, PM/DM | ILD, myositis | + (2/2) | ++ (8/4) | ++ (6/4) | + (2/4) | Hamaguchi, et al. 2013 [19] |
Ha | YARS | SSc, PM/DM | Interstitial pneumonia | - | + (0/1) | - | - | Hashish, et al 2005 [41] |
Zo | FARSA, FARSB | SSc, PM/Scl | Anti-synthetase syndrome | - | + (2/4) | - | - | Betteridge, et al. 2007 [42] |
SSc autoantibodies not detected in our dataset | ||||||||
Fibrillarin | U3RNP | dSSc | More frequent in blacks; severe disease, poor prognosis | - | - | - | - | Mehra, et al. 2013 [3] |
U11/U12 RNP | SNRNP35 | SSc | Lung fibrosis, gastrointestinal involvement | - | - | - | - | Mimori, 1999 [43] |
PDGFR | PDGFR | SSc | Unknown | - | - | - | - | Svegliati Baroni, et al. 2006 [44] |
MMP | MMP family | dSSc | Skin, lung, and vascular fibrosis | - | - | - | - | Mehra, et al. 2013 [3] |
tPA | PLAT | lSSc | PAH | - | - | - | - | Mehra, et al. 2013 [3] |
IFI16 | IFI16 | lSSc | Common in SARDs | - | - | - | - | Mehra, et al. 2013 [3] |
Fibrillin 1 | FBN1 | dSSc | Choctaw and Japanese patients; absent in Caucasians | - | - | - | - | Mehra, et al. 2013 [3] |
Vascular Receptors | AGTR2, EDN1 | SSc | TOP1+ patients, renal crisis | - | - | - | - | Mehra, et al. 2013 [3] |
ATF2 | ATF2 | SSc | Longer disease duration, decreased lung function | - | - | - | - | Mehra, et al. 2013 [3] |