This paper looks at the problem confronting a doctor evaluating a patient with anticentromere antibody who does not have evidence of disease along the spectrum from CREST (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia) to progressive systemic sclerosis. Of 33 people with anticentromere antibody, 21 had CREST and two had scleroderma. Of the other 10 with a positive anticentromere antibody, three had systemic lupus erythematosus (two with digital vasculitis), three very active seronegative polyarthritis, three Raynaud's phenomenon, and one a claudication syndrome involving the legs. A positive antinuclear antibody test does not always indicate the presence of a connective tissue disease, but the presence of anticentromere antibody without systemic sclerosis or CREST often indicates the presence of another sometimes serious underlying rheumatic or connective tissue disease.
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