Abstract
ACA are directed towards centromeric proteins (CENP) with M.W. of 17 KD (CENP-A), 80 KD (CENP-B) and 140 KD (CENP-C). At least 6 epitopes on these proteins are recognized by ACA, but CENP-B (that has been partially cloned) seems to be the primary autoantigen. ACA are clinically associated with the CREST syndrome or limited cutaneous systemic sclerosis (prevalence ±55%), and are also found in 30% of patients with primary biliary cirrhosis (who demonstrate additionally symptoms of CREST), in a small proportion of patients with proximal scleroderma, but hardly in any other condition. The presence of ACA in patients presenting with Raynaud's phenomenon has strong predictive value for the evolution to limited cutaneous systemic sclerosis including CREST.
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