Background/Purpose: Scleritis is a rare, potentially vision threatening ocular inflammatory disease.  30-40% of scleritis is associated with systemic autoimmune diseases with reports of worse visual outcomes in these patients in tertiary referral populations.  To determine if similar findings occur in a broader scleritis population, we assessed the characteristics and visual outcomes of scleritis patients with systemic inflammatory conditions in the IRIS® Registry (Intelligent Research in Sight), a national comprehensive eye disease clinical database maintained by the American Academy of Ophthalmology.

Methods: The IRIS Registry was queried from 2013-2019 to identify scleritis patients with systemic inflammatory diseases utilizing ICD-9 and ICD-10 codes; autoimmune diseases with at least 150 patients with concurrent scleritis are included in the present analysis. Demographic data, ocular features (scleritis subtypes, comorbid uveitis and macular edema) and visual acuity at index visit and 6 months later were compared.

Results: 111,314 scleritis patients were identified from the IRIS Registry; 10 systemic autoimmune conditions had at least 150 patients with concurrent scleritis (4,503 patients, 4.0%).  Rheumatoid arthritis was the most common associated systemic inflammatory disease. Patients with giant cell arteritis/polymyalgia rheumatic were on average older (70.2 ± 12.6 years) and juvenile idiopathic arthritis (JIA) patients on average younger (50.1 ± 22.6 years) compared with other autoimmune diseases at the time of scleritis presentation.  Current smoking was most common in multiple sclerosis (21%) and sarcoidosis (19%) patients.  Among specified scleritis subtypes, anterior scleritis was the most frequent for all systemic conditions.  Comorbid uveitis (19-75%) and cystoid macular edema (9-66%) were common with highest rates in JIA patients.  Ocular perforation occurred more often in patients with underlying autoimmune disease (3.8%) compared to patients without a systemic inflammatory condition (0.65%).  Best visual acuity at index was better than 20/80 for the majority of patients/diseases (90-97%) with the exception of JIA scleritis patients with 25% presenting with worse than 20/80 vision.  In general, best visual acuity at 6 months was stable to best presenting visual acuity with average acuities between 20/25-20/40 at six months compared to 20/30-20/40 at index for all diseases other than JIA.  JIA scleritis patients on average presented with worse vision (20/80) that was maintained but did not improve after 6 months.

Conclusion: Ocular perforation was more common in IRIS Registry scleritis patients with associated systemic inflammatory disease than scleritis patients with idiopathic disease.  Ocular comorbidities were also frequent in the systemic autoimmune disease scleritis population.  Despite these findings, vision in the majority of this patient population was relatively well maintained with the exception of JIA patients who presented with and maintained worse visual outcomes.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/demographic-and-ocular-characteristics-of-scleritis-patients-with-systemic-rheumatologic-disease-in-the-iris-registry-intelligent-research-in-sight/