LONG-TERM FOLLOW-UP OF PATIENTS WITH SJOGRENS-SYNDROME

Objective. To assess long-term outcome in patients with isolated kerat oconjunctivitis sicca (KCS), primary Sjogren's syndrome (SS), and seco ndary SS. Methods. In 112 patients referred because of dry eyes, an op hthalmologic diagnosis of KCS was made based on results of the Schirme r I test, the tear fluid lysozyme concentration, and rose bengal stain ing. Subsequent assessments, including sublabial salivary gland biopsy , were performed. Followup assessments were performed 10-12 years afte r initial diagnosis. Results. Six patients were excluded because no bi opsy specimen was available. Seventy-three percent of the remaining 10 6 patients were female, with a mean age of 53.5 years and a mean sympt om duration of 3.9 years. Application of the 1987 classification crite ria of Daniels and Talal revealed a diagnosis of isolated KCS in 56 pa tients, primary SS in 31, and secondary SS in 19. At baseline, 2 of 56 patients with isolated KCS and 8 of 31 with primary SS exhibited mild features of organ-specific autoimmune disease. At followup, 2 of 38 p atients with isolated KCS and 4 of 21 with primary SS had developed ne w features related to autoimmune disease, not necessitating treatment with corticosteroids; none of the patients developed major glandular c omplications. Three of 30 patients with primary SS died of malignant l ymphoma. In 1 of these patients, the possibility could not be excluded that sicca symptoms and infiltrates seen on sublabial salivary gland biopsy had occurred concomitantly with early stages of lymphoma. Malig nant lymphoma did not develop in any of the patients with isolated KCS or secondary SS. Conclusion. Primary Sjogren's syndrome is characteri zed by a stable and rather mild course of glandular and extraglandular manifestations, in marked contrast to the increased risk for developm ent of malignant lymphoma in these patients. Since patients with isola ted KCS do not have an increased risk for development of malignant lym phoma, a presumptive diagnosis of primary SS should be confirmed in pa tients with sicca syndrome.