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The presence of anti-centromere antibodies (ACA) is predictive of the development of Systemic Sclerosis (SSc) in either apparently health individuals or in patients with Connective Tissue Disease (CTD).1 ACA is found in approximately 30% of patients with SSc and has a distribution range of 11% to 40% worldwide with greater frequency in Caucasians than Asians or African-Americans.2 ACA is recognized as one of the serological markers of limited SSc with features of long lasting Raynaud's disease and subsequent development of progressive thickening of skin at the extremities, neck and face.1 About 10% to 20% of ACA-positive patients develop pulmonary arterial hypertension.3 ACA has been found to be associated with anti-Ro/SSA (both Ro-52 and Ro-60) in approximately 30% of SSc patients.3 Patients with SSc who are also positive for ACA and anti-mitochondrial antibodies have a higher rate of primary biliary cirrhosis.4