Esen Karamursel Akpek1, Priya Mathews2, Sarah Hahn2, Michelle Hessen2, Jean Kim3, Thomas Grader-Beck3, Julius Birnbaum3, Alan N Baer3. 1. Ocular Surface Diseases and Dry Eye Clinic, The Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, Maryland; Jerome L. Greene Sjögren's Syndrome Center, The Johns Hopkins University School of Medicine, Baltimore, Maryland. Electronic address: esakpek@jhmi.edu. 2. Ocular Surface Diseases and Dry Eye Clinic, The Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, Maryland. 3. Jerome L. Greene Sjögren's Syndrome Center, The Johns Hopkins University School of Medicine, Baltimore, Maryland.
Abstract
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 vision-threatening 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.
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 vision-threatening 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.
Authors: Vatinee Y Bunya; Mina Massaro-Giordano; Frederick B Vivino; Maureen G Maguire; Alan N Baer; John A Gonzales; Gui-Shuang Ying Journal: Cornea Date: 2019-12 Impact factor: 2.651
Authors: Fabíola Reis de Oliveira; Ana Carolina F Motta; Carolina Maria Módulo; Denny Marcos Garcia; John A Chiorini; Paulo Louzada-Junior; Eduardo Melani Rocha Journal: Adv Rheumatol Date: 2022-07-01