Of the most common, anti-centromere antibodies (ACA), seen in 20–30% of all SSc cases, demonstrate reactivity against centromeric nucleoproteins such as the DNA-binding protein Centromere Protein B (CENP-B), and are predictive of limited disease with lower frequency of pulmonary fibrosis and associated mortality (Steen et al., 1988; Ho and Reveille, 2003). Here, CENPB is linked to systemic sclerosis.