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Sera from 56 patients with circumscribed scleroderma (CS) were tested for the presence of antinuclear antibodies (ANA) by indirect immunofluorescence on HEp-2-cells. 16 patients (28.6%) had ANA, mostly in low titers of 1: 40 and with uncharacteristic fluorescence patterns. Nucleolar antibodies as in progressive systemic sclerosis were seen in a few cases only. Two female patients demonstrated antibodies with a centromere-like pattern. ANA were most frequently found in patients with atrophodermia idiopathica progressiva (60%) and in linear CS (44%). Rheumatoid factor was present in 62% of patients with linear CS; it was less frequent in other forms of CS. Antibodies against double-stranded DNA were found only in one female patient, but three patients with systemic involvement had antibodies to single-stranded DNA. Antibodies to soluble nuclear Scl-70, Ul-RNP and LA(SSB) antigens or to the cytoplasmatic Ro(SSA)-antigen, respectively, were not detected in the patients with CS. These results demonstrate that humoral autoimmune phenomena may occur in CS, mainly in patients with linear CS. These phenomena, however, are less frequent and specific than in progressive systemic sclerosis or in other collagen vascular diseases.

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