Target antigen | Frequency, % | Staining pattern | Clinical association | Genetic association* |
---|---|---|---|---|
Centromere | 15–40 | Kinetochore | lcSSc, PAH | HLA-DQB1TNF-863AGRB10NOTCH4 |
Topoisomerase-1 (Scl70) | 10–40 | Speckled | dcSSc, lung fibrosis | HLA-DPA1/B1HLA-DPB2HLA-DRB1 |
RNA polymerase III | 4–25 | Fine speckled/nucleolar | Renal crisis, malignancy, PAH | HLA-DRB1HLA-DRB3HLA-DRB4HLA-DQB4EDNRA |
Fibrillarin (U3RNP) | 1–5 | Nucleolar/coilin | PAH, cardiac, myositis | HLA-DQB1 |
Pm-Scl | 3–6 | Nucleolar | Myositis overlap | None reported |
U1RNP | 5–35 | Speckled | Overlap features | HLA-DRB1HLA-DPB1 |
Th-To | 1–7 | Nucleolar | lcSSc, PAH, lung fibrosis | None reported |
U11/U12 | 1–5 | Nucleolar | Lung fibrosis | None reported |
*the table summarises reported associations that may differ between ethnic and geographically defined populations but are consistently seen for HLA region and specific ANA patterns suggesting that there is a genetic basis to the development of SSc-associated autoantibodies.
ANA = antinuclear antibodies; dcSSc = diffuse systemic sclerosis; HLA = human leukocyte antigen; lcSSc = limited systemic sclerosis; PAH = pulmonary arterial hypertension.