Purpose: To report vision-threatening ocular manifestations of primary Sjögren's syndrome (SS). Design: Retrospective review. Participants: Consecutive patients evaluated at an SS center between January 2007 and May 2011. Methods: Data collection was completed in March 2013. The 2002 American-European consensus criteria were used for diagnosis of SS. Main Outcome Measures: Frequency of extraglandular ocular findings and timing of their diagnosis relative to that of SS and dry eye were assessed. Results: One hundred sixty-three patients were included. Almost all patients (98%) had a history of dry eye for an average of 10.4 years (median, 7.9 years) before presentation. One or more extraglandular ocular manifestations were present in 40 patients (25%), and vision-threatening findings were present in 22 patients (13%). Twelve patients (55%) with a vision-threatening ocular finding did not have a diagnosis of SS at presentation. Sixty-eight patients (42%) had extraglandular systemic manifestations of SS. Patients with vision-threatening ocular findings were 3.9 times more likely to have systemic involvement (95% confidence interval, 1.4-11.0; P = 0.010). Peripheral neuropathy, interstitial nephritis, and vasculitis were more common in those with visionthreatening ocular findings compared with patients without (P < 0.05 for all). Conclusions: These results from a tertiary referral-based cohort demonstrate that primary SS frequently is associated with ocular and systemic complications. Dry eye precedes these findings on average by 1 decade. Therefore, ophthalmologists should consider assessing for SS in patients with clinically significant dry eye.
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