Table 1.
Autoantibodies associated with Systemic Sclerosis.
| Autoantibody target | Prevalence (%) | Clinical association | Vasculopathy | Fibrosis | Other | References |
|---|---|---|---|---|---|---|
| Centromere | 20–40 | lcSSc & PAH | − | − | – | (54) |
| Topoisomerase I | 9–42 | dcSSc & ILD | − | − | – | (54) |
| Fibrillarin (U3 RNP) |
3–20 | dcSSc | + | + | – | (54, 55) |
| RNA polymerase III | 4–11 | dcSSc, SRC & malignancy | − | − | – | (54) |
| Th/To | 2–5 | SRC | − | − | – | (54) |
| Ku | 2–5 | Overlap syndrome | − | − | – | (54) |
| Endothelial cell [anti-VCAM, ICAM-1, NAG-2] |
22–85 | PAH | + | − | Leukocyte adhesion | (56–58) |
| Angiotensin II type 1 Receptor (AT1R) |
85 | PAH & Digital ulcer | + | + | – | (56, 59) |
| Endothelin type A receptor (ETAR) | 85 | PAH ulcer | + | + | – | (56, 59) |
| M3R | 65–80 | Gastrointestinal involvement | − | − | Inhibition of type 3 muscarinic receptor | (60, 61) |
| Vascular smooth muscle cells | 80 | – | + | − | – | (56–58) |
| Fibroblast | 25–60 | dcSSc with PAH, ILD | − | + | Adhesive mechanism | (62–64) |
| MMP-1 & MMP-3 | 50 | dcSSc | − | + | – | (65) |
| PDGF-Receptor | 33 | – | − | + | – | (66) |
| MSRA | 33 | ILD & cardiac involvement | + | + | Reinforcement of oxidative stress | (67) |
| PRX I | 33 | ILD & cardiac involvement | + | + | – | (67) |
| CD22 | 22 | – | − | − | Antagonize inhibitory CD22 signal | (68) |
Anti-AT1-Rc Abs, anti-angiotensin II type 1 receptor Abs; anti-ETa-Rc Abs, anti-endothelin 1 type A receptor Abs; anti-M3R Abs, anti-muscarinic acetylcholine receptor M3; anti-MSRA Abs, anti-Methionine Sulfoxide Reductase A Abs; anti-PDGF-Receptor Abs, anti-platelet-derived growth factor receptor Abs; anti-Prx I Abs, peroxiredoxin I; anti-RNA polymerase III Abs, anti-ribonucleid acid polymerase enzyme III Abs; anti-U3-RNP Abs, anti-U3 ribonucleoprotein Abs, anti-topoisomerase 1 Ab; Anti-VCAM Abs, anti-vascular cell adhesion molecules Abs; anti-VSMC Abs, anti-vascular smooth muscle cell Abs; CCL, chemokine ligand; CD, cluster of differentiation; CENP, centromeric protein with a molecular weight of 17 (CENP-A), 80 (CENP-B) and 140 kDa (CENP-C); dcSSc, diffuse cutaneous form of systemic sclerosis; ICAM, intercellular adhesion molecules; ILD, interstitial lung disease; Ku, p70/p80 is DNA-binding protein involved in DNA repair and regulation; lcSSc, limited cutaneous form of systemic sclerosis; MMP, matrix metalloproteinases; PAH, pulmonary arterial hypertension; SRC, scleroderma renal crisis; TM4SF7 or Nag-2, cell surface tetraspanin transmembrane 4 superfamily molecules member 7; Th/To, 7-2 RNP and 8-2 RNP that are subunits of human mitochondrial RNase and RNase P ribonucleoprotein complexes.