Fifty female patients with primary Sjögren's syndrome diagnosed according to the Copenhagen criteria were evaluated for both glandular and extraglandular involvement. They were divided into two groups based on the presence or absence of antinuclear and anti-ENA antibodies (ANA/anti-ENA). ANA/anti-ENA negative patients presented with milder and later glandular and extraglandular disease and required less frequent corticosteroid treatment. No significant differences were noted in extraglandular manifestations with the exception of leukopenia which was noted only in ANA/anti-ENA positive cases.
