Objective: To determine whether the clinical and laboratory characteristics of anticentromere antibody (ACA) positive, anti-SSA/Ro antibody (SSA) negative primary Sjogren's syndrome (SS) differ from SSA positive, ACA negative primary SS.

Methods: Twelve patients with ACA positive primary SS (ACA SS) and 19 patients with SSA positive primary SS (SSA SS) were examined. We compared the age, laboratory data, proportion with Raynaud's phenomenon (RP), activity of natural killer cells (NK), titer of antibodies against Epstein-Barr virus, and histological findings of minor labial salivary glands. The presence of anti-chromo antibodies (AChA) was evaluated by immunoblotting of patients' sera.

Results: The mean age of the ACA SS group was higher than that of the SSA SS group (p < 0.05). Serum IgG level was lower in ACA SS than in SSA SS (p < 0.0001). Serum IgG level of the ACA SS group with one exception was close to the normal range. Leukocytopenia was less frequently observed in ACA SS than in SSA SS (p < 0.05). RP was seen more frequently in the ACA SS group than the SSA SS group (p < 0.05). NK activity of the ACA SS group was higher than that of the SSA SS group (p < 0.05). Most of the ACA SS patients' NK activity was normal, in contrast to the tendency for NK activity in SS to be low. Virus capsid antigen IgA titer of the ACA SS group was lower than that of the SSA SS group (p < 0.05). Histological findings of minor labial salivary glands of both groups showed a similar severity of lymphocytic infiltrates, destruction of normal structures, and pattern of infiltrating lymphocyte subsets. AChA was positive in 11 of the 12 sera of ACA SS patients.

Conclusion: The results confirm that ACA positive primary SS differs from SSA positive classic SS in several significant respects.