Scleroderma subsets are best detected by the simultaneous analysis of the autoantibody profile using commercial ELISA

Objective: Antinuclear antibodies are often present in patients with systemic sclerosis but do not provide useful prognostic information if studied individually. Although an autoantibody profile should be studied, this has never been done in a large series of patients. Methods: Anticentromere, anti-topoisomerase-I, anti-extractable-nuclear-antigen, antihistone and anticardiolipin antibodies were studied by means of enzyme immunosorbent assay in 90 systemic sclerosis patients. Results: We confirmed that anticentromere antibodies characterize limited forms of the disease with less frequent visceral involvement while anti-topoisomerase-I antibodies characterize diffuse forms with severe gastrointestinal, heart and lung involvement. Antihistone antibodies alone or associated with anticentromere antibodies characterize a disease subset with more frequent visceral involvement and a probably poor outcome. Patients with anticardiolipin antibodies, instead, did not display severer heart or vascular involvement. Conclusions: Systemic sclerosis patients are often found to have antinuclear antibodies. In addition to the well-known disease subsets identified by anti-topoisomerase-I and anticentromere antibodies, another one can be established, characterized by antihistone antibodies often associated with anticentromere antibodies. Patients displaying this profile have high prevalence of lung, kidney and heart involvement.