June 2020
Volume 61, Issue 7
ARVO Annual Meeting Abstract  |   June 2020
Long-term Corneal Complications in Patients with Primary Sjögren’s Syndrome
Author Affiliations & Notes
  • Anisa Gire
    ophthalmology, Johns Hopkins University, North Bethesda, Maryland, United States
  • Priya Mathews
    Florida Eye Specialists & Cataract Institute, Brandon, Florida, United States
  • David Cui
    ophthalmology, Johns Hopkins University, North Bethesda, Maryland, United States
  • Esen K Akpek
    ophthalmology, Johns Hopkins University, North Bethesda, Maryland, United States
  • Footnotes
    Commercial Relationships   Anisa Gire, None; Priya Mathews, None; David Cui, None; Esen Akpek, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2020, Vol.61, 339. doi:
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      Anisa Gire, Priya Mathews, David Cui, Esen K Akpek; Long-term Corneal Complications in Patients with Primary Sjögren’s Syndrome. Invest. Ophthalmol. Vis. Sci. 2020;61(7):339.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose : To report long-term corneal complications in a longitudinal cohort of patients with primary Sjögren Syndrome (SS) diagnosed with American College of Rheumatology’s 2012 criteria.

Methods : Inclusion criteria consisted of patients at the Johns Hopkins Wilmer Eye Institute, a tertiary academic medical center, who had one clinic visit per calendar year for a minimum of 5 years. Sjogren’s patients were identified using an existing database from the Johns Hopkins Sjogren’s Syndrome Center and further narrowed using identified using ICD codes for dry eye syndrome/keratoconjunctivitis sicca. Sjogren’s patients met the American College of Rheumatology’s 2012 criteria, which requires 2 of the following: positive serum anti-SSA and/or anti-SSB, ocular staining score ≥3, and presence of focal lymphocytic sialadenitis in labial salivary gland biopsy. A retrospective chart review was performed of each patient from their initial visit to all subsequent follow-ups. These included 31 SS patients with corneal complications as a result of dry eye

Results : 31 SS patients with minimum 5 years of follow-up were included who had dry eye for a median of 9.5 years (range of 2 weeks to 20 years) at initial presentation. The cohort consisted of 22 (70%) female patients with average age of 69.1 (range of 53-92 years). Major corneal findings included 22 patients (70%) with corneal haze or opacifications, 9 patients (30%) with corneal ulcer, and 4 patients (13%) with corneal melt or perforation. 3 patients (75%) of patients with corneal ulcer required surgical intervention.
Treatment included preservative-free artificial tears in 25 patients (81%), 23 patients (74%) using cyclosporine 0.05% and 13 (42%) patients with punctal plugs. Major systemic complications included cardiovascular (19%), renal (19%), and associated autoimmune conditions including systemic lupus erythematosus (10%) and rheumatoid arthritis (10%).
Systemic complications more common in those with serious corneal sequelae.

Conclusions : Primary SS patients with a history of dry eye present with serious corneal manifestations which can be vision-threatening. Dry eye disease in primary SS patients should be managed and stabilized with aggressive ocular and systemic medications.

This is a 2020 ARVO Annual Meeting abstract.


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