Clinical characteristics of scleritis patients with emphasized comparison of associated systemic diseases (anti-neutrophil cytoplasmic antibody-associated vasculitis and rheumatoid arthritis)

Purpose We evaluated patient profiles, clinical features, associated systemic diseases, treatment modalities, and ocular complications in cases of scleritis and episcleritis. Study design Retrospective. Methods Clinical data of 128 patients referred to the ophthalmology clinic at Jichi Medical University Hospital during the 4-year period from April 2011 to March 2015, and diagnosed with scleritis or episcleritis were examined. Gender, average onset age, unilateral or bilateral manifestation, classification type, associated systemic diseases, and treatments were retrospectively investigated. Results The cohort consisted of 57 men and 71 women. Average onset age was 54.3 +/- 17.4 years. Diffuse anterior scleritis was the most common type. It was noted in 43 (32.8%) patients, followed by episcleritis in 35 (27.3%), nodular anterior scleritis in 23 (18.0%), necrotizing anterior scleritis in 22 (17.2%), and posterior scleritis in 6 (4.7%). Eighteen (81.8%) of 22 patients with necrotizing anterior scleritis required some type of systemic medication, including corticosteroid, cyclophosphamide, cyclosporine, azathioprine, methotrexate, or rituximab administration. Forty (31.3%) had associated systemic diseases, which included 10 with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and 8 with rheumatoid arthritis (RA). Patients with ANCA-associated vasculitis had a tendency to develop scleritis first and had significantly worse visual prognoses compared to those with RA. Conclusions Approximately 30% of the patients with scleritis and episcleritis had complications involving systemic diseases, including ten patients with ANCA-associated vasculitis and 8 with RA. ANCA-associated vasculitis was more often diagnosed after scleritis and patients suffered poorer visual prognoses than those with RA.