Objectives To correlate the main features at diagnosis with survival in a multicenter international cohort of patients with primary Sjögren syndrome (SS).
Methods The Big Data Sjögren Project was formed in 2014 to take a “high-definition” picture of the main features of primary SS by merging international SS databases (9302 consecutive patients recruited from 21 countries of the 5 continents)
Results Data about survival at the last visit was available in 6240 patients (5831 women, mean age at diagnosis of 53yrs, 82% White). After a mean follow-up of 7.1yrs, 407 (6%) patients died. Patients who died were more frequently men (10% vs 6%, p=0.004) and White (96% vs 81%, p<0.001), had a higher mean age at diagnosis (64 vs 52yrs. p<0.001) and a higher frequency of abnormal ocular (95% vs 87%, p<0.001) and oral (90% vs 79%, p<0.001) tests in comparison with survivors; sicca symptoms and salivary gland biopsy were unrelated to survival. Immunologically, patients who died showed a higher frequency of RF (52% vs 46%, p=0.039) and cryoglobulins (18% vs 7%, p<0.001). Logistic regression identified as independent variables the age at diagnosis (OR 1.07), male gender (OR 1.77), abnormal ocular (OR 5.79) and oral (OR 2.10) tests, and cryoglobulins (OR 3.10). Time-adjusted multivariate Cox proportional-hazards regression analysis confirmed age at diagnosis (OR 1.12, CI95% 1.10–1.14), male gender (OR 2.12, CI95% 1.36–3.29) and cryoglobulins (OR 2.43, CI95% 1.72–3.42) as independent variables related to death.
Conclusions An older age at diagnosis, male gender and serum cryoglobulins were the principal risk factors associated with death in this large multiethnic cohort of patients with primary SS.
Disclosure of Interest None declared