Previous studies had shown that anti-centromere antibodies were strongly associated with renal dysfunction in lcSSc patients and CENP-B was a major target antigen reported for AECA in lcSSc patients indicating the association between anti-centromere antibodies and AECA autoantibodies leading to AECA mediated endothelial dysfunction due to an underlying autoimmune mechanism [18–21]. The gene discussed is CENPB; the disease is endothelial dysfunction.