Peripheral ulcerative keratitis (PUK) in autoimmune diseases: a severe ocular manifestation not always associated with systemic disease activity.

Abstract

Peripheral ulcerative keratitis (PUK) is a rare and severe ocular manifestation frequently associated with systemic autoimmune diseases. This retrospective two-center observational study including patients with PUK and a systemic autoimmune disease at two tertiary referral centres aimed to characterize clinical and histopathologic features, treatment strategies, relapses, and ocular outcomes of autoimmune-associated PUK, and to analyse its relationship with systemic disease activity. A total of 24 patients (35 eyes) were identified, 62.5 % female, with a median age of 58.5 years and a mean follow-up of 14.4 years. Rheumatoid arthritis (RA) (45.8 %) and granulomatosis with polyangiitis (GPA) (29.2 %) were the most frequent underlying diseases. PUK preceded or coincided with the diagnosis of systemic disease in 41.7 % of cases. At presentation, 45.8 % had bilateral involvement and 58.3 % had associated scleritis. More than half of patients experienced relapses, with a mean annual relapse rate of 0.43. Corneal complications were common and visual acuity worsened or was severely impaired in 37.5 % and 16.7 % of patients, respectively. Most patients received systemic glucocorticoids and additional immunosuppressive therapy, frequently biologic agents, mainly TNF blockers and rituximab, and 20.8 % underwent ocular surgery. Remarkably, 58.3 % patients had no evidence of systemic activity at PUK onset and 83.3 % of PUK relapses were developed with the autoimmune disease in remission. We conclude that autoimmune-associated PUK may precede systemic diagnosis and often occurs with an inactive systemic disease. These findings emphasize the need for careful autoimmune evaluation in all PUK cases and sustained ophthalmologic surveillance even during remission of the systemic disease.