Primary Sjogren's syndrome in the North East of England: a long-term follow-up study

Objective. Although primary Sjogren's syndrome is often a benign condition, characterized by lymphocytic infiltration of salivary and lacrimal glands, some patients develop systemic features. We have previously found that ant i-Ro antibodies identified patients with more systemic disease, with increa sed incidence of parotid swelling, lymphadenopathy and lymphoma. Methods. We have followed up a cohort of 100 patients over 10 yr, to establ ish whether the phenotypic expression of disease changed, and whether the d ifferent autoantibody patterns expressed at presentation could be used to p redict outcome. Results. While seronegative patients (ANA, RF, Ro and La negative) remained polysymptomatic, they did not develop systemic complications or serologica l changes. Thirty-nine per cent of ANA- or RF-positive patients who were ne gative for Ro and La were given revised diagnoses over the follow-up period , including rheumatoid arthritis, systemic lupus erythematosus, mixed conne ctive tissue disease and scleroderma. Parotid swelling and lymphadenopathy were more common in Ro/La-positive patients, where the relative risk of dev eloping non-Hodgkin's lymphoma was 49.7. Conclusion. Both HLA B8 and DR3 were present in 79% of Ro/La-positive patie nts, but were found together in only 4% of seronegative patients, supportin g the view that these clinical subgroups of primary Sjogren's syndrome are both serologically and immunogenetically distinct. Patients who are initial ly autoantibody (including Ro and La) negative do not evolve into 'systemic ' Sjogren's syndrome or other connective tissue diseases.