Table 1:
Autoantibodies associated with systemic sclerosis and their clinical correlates
Cutaneous subtype | Clinical correlates | |
---|---|---|
Anti-centromere | Limited cutaneous systemic sclerosis | Pulmonary arterial hypertension |
Anti-topoisomerase 1 (anti-ScI 70)* | Diffuse cutaneous systemic sclerosis | Progressive interstitial lung disease |
Anti-RNA polymerase 3 | Diffuse cutaneous systemic sclerosis | Renal crisis and malignancy |
Anti-U1 ribonucleoprotein (anti-ribonucleoprotein) | Limited cutaneous systemic sclerosis | Mixed connective tissue disease |
Anti-U3 ribonucleoprotein (anti-fibrillarin) | Diffuse cutaneous systemic sclerosis | Pulmonary arterial hypertension and myositis |
Anti-Pm-Scl | Limited cutaneous systemic sclerosis | Myositis |
Anti-Th/To | Limited cutaneous systemic sclerosis | Interstitial lung disease and pulmonary arterial hypertension |
Testing for systemic sclerosis-associated auto-antibodies is recommended early in the evaluation for systemic sclerosis because it can help establish a systemic sclerosis diagnosis and inform risk-stratification strategies. Patients will seldomly produce more than one systemic sclerosis-related auto-antibody concurrently.
Immunodiffusion is the preferred method for detecting anti-topoisomerase 1 antibodies, given that multibead assays are associated with high false-positive rates.