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Patients with systemic sclerosis (SSc) were studied with regard to the presence of antinuclear antibodies (ANA) and their clinical correlates (n = 230), pulmonary function (n = 176), and mortality and causes of death (n = 344). ANA were found in 85%. Anti-centromere antibodies were found in 34%, anti-Scl-70 antibodies in 13% and anti-U1-RNP antibodies in 6.5%. These serological groups were associated with limited SSc, diffuse SSc, and myositis/arthritis, respectively. The most prevalent finding at first lung function test was isolated reduction of diffusion capacity (47%). Further deterioration of diffusion capacity was related to the presence of anti-centromere antibodies and increased sedimentation rate. The standardized mortality rate (SMR) was 2.9, higher in young patients (SMR = 13) and patients with diffuse SSc (SMR = 4.5).


Journal article


Ugeskr Laeger

Publication Date





3084 - 3090


Adolescent, Adult, Aged, Antibodies, Antinuclear, Blood Sedimentation, Cause of Death, Centromere, Female, Humans, Lung Volume Measurements, Male, Middle Aged, Prognosis, Scleroderma, Systemic, Survival Rate