title: Ocular and systemic morbidity in a longitudinal cohort of Sjögren's syndrome.
abstract: OBJECTIVE
  To report vision-threatening ocular manifestations of primary Sjögren's syndrome (SS).
  
  
  METHODS
  Retrospective review.
  
  
  METHODS
  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.
  
  
  METHODS
  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.affiliation: 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.
authors.forename: Esen Karamursel
authors.initials: EK
authors.lastname: Akpek
authors.affiliation: Ocular Surface Diseases and Dry Eye Clinic, The Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Priya
authors.initials: P
authors.lastname: Mathews
authors.affiliation: Ocular Surface Diseases and Dry Eye Clinic, The Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Sarah
authors.initials: S
authors.lastname: Hahn
authors.affiliation: Ocular Surface Diseases and Dry Eye Clinic, The Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Michelle
authors.initials: M
authors.lastname: Hessen
authors.affiliation: Jerome L. Greene Sjögren's Syndrome Center, The Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Jean
authors.initials: J
authors.lastname: Kim
authors.affiliation: Jerome L. Greene Sjögren's Syndrome Center, The Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Thomas
authors.initials: T
authors.lastname: Grader-Beck
authors.affiliation: Jerome L. Greene Sjögren's Syndrome Center, The Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Julius
authors.initials: J
authors.lastname: Birnbaum
authors.affiliation: Jerome L. Greene Sjögren's Syndrome Center, The Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Alan N
authors.initials: AN
authors.lastname: Baer
mesh_terms: D000328:Adult; D000368:Aged; D015331:Cohort Studies; D005128:Eye Diseases / Q000150:complications* / Q000175:diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009017:Morbidity; D009395:Nephritis, Interstitial / Q000150:complications* / Q000175:diagnosis; D010523:Peripheral Nervous System Diseases / Q000150:complications* / Q000175:diagnosis; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis; D013997:Time Factors; D014657:Vasculitis / Q000150:complications* / Q000175:diagnosis