Relation of systemic autoantibodies to the number of extraglandular manifestations in primary Sjögren's Syndrome: a retrospective analysis of 65 patients in the Netherlands.

Objectives: Extraglandular manifestations (EGM) are often seen in patients with primary Sjögren's syndrome and are probably due to a (more) disturbed immune system. Their relation to systemic autoantibodies remains controversial. We hypothesized that positive serology as reflected by the presence of 1 of more systemic autoantibodies is related to the number of EGM.

Methods: To this purpose, all patients, visiting a large nonacademic teaching hospital, with primary Sjögren's syndrome, according to the revised American-European classification criteria of 2002, were retrospectively analyzed.

Results: In this group of 65 patients, systemic autoantibodies were abundant: anti-Sjögren syndrome A antigen (SSA) and/or anti-Sjögren syndrome B antigen (SSB) (80%), immunoglobulinM-Rheumatoid factor (IgM-RF) (68%), and anti-nuclear antibodies (ANA) (77%). Their presence was often found together and correlated to the presence of hypergammaglobulinemia. There was a statistically significant correlation between the number of systemic autoantibodies and the total number of EGM (P = 0.025). Anti-SSA was the strongest predictor of the presence of EGM (OR = 4.67, P = 0.024).

Conclusions: These findings indicate that a more disturbed immune system, as reflected by B-cell hyperactivity, with autoantibody formation and hypergammaglobulinemia, is associated with more systemic manifestations in patients with primary Sjögren's syndrome.