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Antinuclear antibodies are seen in a number of autoimmune disorders such as systemic lupus erythematosus, mixed connective tissue disease, rheumatoid arthritis, Sjogren syndrome, and progressive systemic sclerosis.
One autoantibody that produces a distinct staining pattern is the anticentromere antibody (ACA). ACA occurs in patients with the calcinosis, Raynaud phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia (CREST) syndrome variant of progressive systemic sclerosis.
Evaluation of patients with clinical signs and symptoms compatible with systemic sclerosis
A positive test for anticentromere antibody (ACA) is strongly associated with calcinosis, Raynaud phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia (CREST) syndrome. In various reported clinical studies, ACA occur in 50% to 96% of patients with CREST syndrome.
The presence of detectable levels of ACA may antedate the appearance of diagnostic clinical features of CREST syndrome, in some cases by several years.
Anticentromere antibody have also been described in some patients with primary biliary cirrhosis, and rarely, in patients with rheumatoid arthritis or lupus erythematosus.
Kallenberg CG: Anti-centromere antibodies (ACA). Clin Rheum 1990;9:136-139