To report vision-threatening ocular manifestations of primary Sjögren's syndrome (SS). Retrospective review. Consecutive patients evaluated at an SS center between January 2007 and May 2011. Data collection was completed in March 2013. The 2002 American-European consensus criteria were used for diagnosis of SS. Frequency of extraglandular ocular findings and timing of their diagnosis relative to that of SS and dry eye were assessed. 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 vision-threatening ocular findings compared with patients without (P < 0.05 for all). 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. Copyright © 2015 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.
