Ocular complications and mortality in peripheral ulcerative keratitis and necrotising scleritis: The role of systemic immunosuppression

Importance Inflammatory ocular diseases are associated with systemic disease, ocular morbidity and mortality. Background To examine clinical characteristics of subjects with peripheral ulcerative keratitis (PUK) and necrotising scleritis. Design Retrospective case series in a single tertiary centre over 10 years. Participants All patients presenting with necrotising scleritis, PUK or scleritis with corneal infiltrates. Fifty-two eyes (41 subjects) were identified. Methods Demographic and clinical data analysis. Main Outcome Measures Visual loss, ocular complications and mortality. Results Mean follow up was 7.0 +/- 5.3 years, presentation age 51.3 +/- 18.6 years, and 23 subjects (56.1%) were female. Overall, 27 eyes presented with PUK, 26 with necrotising scleritis, and 22 with corneal infiltrates associated with active scleritis. Associated systemic diagnosis was observed in 12 subjects at presentation, and in 21 subjects (51.2%) by final follow up. Recurrence occurred in 23 eyes (44.2%): 10 eyes experiencing PUK or necrotising scleritis; 13 eyes with non-necrotising scleritis. Perforation occurred in six eyes (11.5%). Moderate vision loss occurred in 10 eyes (19.2%) and severe loss in six eyes (11.5%). Five subjects (12.2%) were deceased by study conclusion, of whom four were not on immunomodulatory therapy (IMT). A marked difference in estimated survival was observed with IMT, with mean estimated survival of 10.7 years without treatment, compared to 24.7 years with IMT (P = .045). Conclusions and Relevance PUK and necrotising scleritis represent a severe inflammatory disease with high rates of perforation, visual loss and mortality. IMT was associated with a lower rate of mortality and longer estimated survival in this group.