Table 5 Autoantibodies in systemic sclerosis
From: The clinical relevance of autoantibodies in scleroderma
Autoantibody | Methods of testing | Prevalence in SSc | Clinical and serologic associations | Prognosis |
|---|---|---|---|---|
Anti-centromere | IIF | 20–30% | CREST | Better prognosis than anti-Scl-70 |
|  | IB |  | lcSSc | ↑ Survival compared with anti-Scl-70 or anti-nucleolar antibodies |
|  | ELISA |  | ↓ Pulmonary fibrosis | No benefit in following levels over time |
| Â | Â | Â | Pulmonary hypertension | Â |
Anti-Scl-70 | ID | ~15–20% | Mutually exclusive with ACA | Worse prognosis |
| Â | CIE | Â | dcSSc | ? Levels by ELISA fluctuate with extent of disease involvment |
| Â | IB | Â | Pulmonary fibrosis and secondary cor pulmonale | Â |
| Â | ELISA | Â | Â | Â |
Anti-PM-Scl | ID | ~3% | lcSSc | Benign/chronic course with better response to steroids |
| Â | IP | (Rare in Japanese) | PM/SSc overlap | Â |
Anti-Th/To | IP | ~2–5% | Mutually exclusive with ACA | Worse prognosis with reduced 10-year survival |
| Â | Â | (More common in Japanese) | lcSSc | Â |
|  |  |  | ↓ Joint involvement |  |
|  |  |  | ↑ puffy fingers, small bowel involvement, hypothyroidism |  |
AFA | IP | ~4% | Mutually exclusive with ACA, anti-Scl-70, anti-RNAP | Seen in younger patients with greater internal organ involvement |
|  |  | 16–22% in patients of African descent | dcSSc |  |
| Â | Â | 4% in Caucasians | Myositis, pulmonary hypertension, renal disease | Â |
Anti-RNAP | IP | ~20% | dcSSc | Increased mortality |
|  |  |  | Anti-RNAP II with ↓ lung function |  |
| Â | Â | Â | Cor pulmonale unrelated to pulmonary fibrosis | Â |
Anti-Ku | IB | Infrequent | Overlap syndrome with scleroderma features | Â |
| Â | IP | Â | Â | Â |
| Â | ELISA | Â | Â | Â |
Anti-Ro | ID | Infrequent | Seen in one-third to one-half of SSc patients with sicca complex | Â |
| Â | ELISA | Â | Â | Â |
Anti-Sm | IIF | Rare | SLE overlap | Poor prognosis |
| Â | IP/CIE/ID | Lupus nephritis, renal crisis | Â | Â |
| Â | ELISA | Pulmonary hypertension | Â | Â |
| Â | HA | Â | Â | Â |
Anti-ribonucleoprotein | IIF | ~8% | MCTD | Â |
| Â | IP/CIE/ID | Â | Less CNS and renal diseases | Â |
| Â | ELISA | Â | Raynaud's, puffy hands, sicca, myositis, esophageal disease | Â |
| Â | HA | Â | lcSSc | Â |
| Â | Â | Â | Septal hypertrophy | Â |
| Â | Â | Â | Cor pulmonale secondary to pulmonary hypertension | Â |
| Â | Â | Â | Negatively correlates with dsDNA and low complement glomerulonephritis in those with SLE overlap | More benign prognosis with favorable response to steroids |
Anti-phospholipid antibodies | ELISA | ~20–25% with <1% SSc with APS | Mutually exclusive with anti-centromere antibodies | Associations inconsistent – needs further study |
|  |  |  | aCL with ↑ disease severity and ↓ proximal skin involvement, scarring, esophageal hypomotility in one study |  |
|  |  |  | β2gp/aCL with pulmonary hypertension |  |
|  |  |  | Associations inconsistent – needs further study |  |