Abstract
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.