Summary of patterns of ANA reactivity and clinical and genetic associations in systemic sclerosis.
| Target antigen | Frequency, % | Staining pattern | Clinical association | Genetic association* |
|---|---|---|---|---|
| Centromere | 15–40 | Kinetochore | lcSSc, PAH | HLA-DQB1 |
| TNF-863A | ||||
| GRB10 | ||||
| NOTCH4 | ||||
| Topoisomerase-1 (Scl70) | 10–40 | Speckled | dcSSc, lung fibrosis | HLA-DPA1/B1 |
| HLA-DPB2 | ||||
| HLA-DRB1 | ||||
| RNA polymerase III | 4–25 | Fine speckled/nucleolar | Renal crisis, malignancy, PAH | HLA-DRB1 |
| HLA-DRB3 | ||||
| HLA-DRB4 | ||||
| HLA-DQB4 | ||||
| EDNRA | ||||
| 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-DRB1 |
| HLA-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.