Clinical characteristics and management of non-infectious necrotizing scleritis

Abstract

Purpose : In this retrospective observational study, we aimed to describe the clinical characteristics and management of two types of non-infectious scleritis: 1) Necrotizing scleritis with signs of inflammation and 2) Necrotizing scleritis without signs of inflammation (scleromalacia perforans).

Methods : Medical records of patients in a tertiary eye care hospital were reviewed from January 2015 to October 2022. Demographics and clinical data of patients with the two types of non-infectious necrotizing scleritis were collected and assessed.

Results : A total of 12 eyes of seven patients were included. Median age was 65 (range, 41-89) years. Median follow-up was 18 months (range, 1-37). Five patients (71%) had bilateral involvement. Systemic disease existed in four patients (71%), including two cases with rheumatoid arthritis, one with granulomatosis polyangiitis, and one with connective tissue disease (Marfanoid features). Eight eyes had necrotizing scleritis with signs of inflammation and four eyes (33%) had scleromalacia perforans. Three eyes (25%) had peripheral ulcerative keratitis and six eyes (50%) had concomitant anterior uveitis. All patients (100%) received systemic corticosteroids; five (71%) were treated with immunomodulatory therapy, and three patients (43%) were given biologics. Among the seven patients, five (71%) patients were treated with cyclophosphamide therapy to control ocular inflammation. Four eyes (33%) of three patients required surgical repair. Patients who underwent scleral patch surgery were managed with monthly intravenous cyclophosphamide and methylprednisolone infusion(s); disease stabilization was achieved following surgery. Three out of five patients (60%) who were treated with cyclophosphamide had infusion held due to leukopenia (WBC: <4000mL) with subsequent resumption when WBC count increased to >4000mL.

Conclusions : Necrotizing scleritis is a devastating disease that requires aggressive treatment. Monthly cyclophosphamide therapy along with methylprednisolone infusions may be beneficial to control non-infectious necrotizing scleritis and to provide promising outcomes following surgical repairment. Close hematological monitoring is required for all patients during cyclophosphamide treatment.

This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.

 

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