Antinuclear antibodies in systemic sclerosis

Antinuclear autoantibodies are present in more than 90% of patients with systemic sclerosis (SSc). Some of these antibodies can be regarded as disease-specific: anticentromere antibodies, anti-topoisomerase I and anti-RNA polymerase I–III identify the three major serological subsets. Other important antibodies recognize nucleolar antigens, such as fibrillarin, Th/To and PM/Scl. All of these specific serological reactivities are mutually exclusive, have definite clinical associations and prognostic implications. This makes antinuclear antibodies a valuable tool in classifying and managing patients with SSc. SSc pathogenesis remains unclear and the role of autoantibodies is debated. Studies exploring their relationships with new emerging disease mechanisms are still in progress.