Survivorship in a population based cohort of patients with Sjogren's syndrome, 1976-1992

Objective, Sjogren's syndrome (SS) has been associated with development of lymphoid malignancies and other significant medical complications, but the effect of SS on survival in a population based sample has not been reported . We analyzed survival in an incidence cohort of patients diagnosed with SS in residents of Olmsted County, Minnesota, USA, between 1976 and 1992. Methods, All records of physician diagnosed SS were reviewed, as well as al l records from patients diagnosed with xerostomia and keratoconjunctivitis sicca, and records of patients with rheumatoid arthritis (RA) and systemic lupus erythematosus. The case definition for SS required 2 of 3 criteria: k eratoconjunctivitis sicca, xerostomia, or serologic abnormality. Confoundin g illnesses were excluded. All patients were white. Results. Of the 74 cases of SS identified, 50 (67%) had primary SS and 24 ( 33%) secondary SS, An average of 7.2 years of followup was available for pa tients with primary SS and 9.9 years for patients with secondary SS. Compar ed with the general population, patients with SS had increased mortality (p = 0.04). When patients with primary and secondary SS were studied separate ly, increased mortality was found in patients with secondary SS (p < 0.005) but not primary SS (p = 0.86). Conclusion. In this population based cohort, patients with primary SS did n ot have increased mortality. However, mortality may have been increased in patients with secondary SS, the majority of whom had RA.