Sequential ocular surgeries and multimodal immunosuppression for bilateral, medically refractory seronegative scleritis with severe peripheral corneal stromal thinning: a case report

Abstract

Severe peripheral corneal stromal thinning and perforation can occur in autoimmune or idiopathic scleritis despite intensive systemic immunosuppression. CASE PRESENTATION: A 36-year-old man with bilateral, seronegative, treatment-refractory scleritis developed circumferential peripheral corneal stromal thinning, progressing to multiple perforations in one eye and traumatic dehiscence of a previous patch graft in the fellow eye. Multiple steroid-sparing agents—including mycophenolate, tumor necrosis factor-alpha (TNF-α) and interleukin-6 (IL-6) inhibitors, alkylating agents, calcineurin inhibitors, and Janus kinase (JAK) inhibition—were ineffective or poorly tolerated. Annular penetrating keratoplasty (14 mm outer / 6.5 mm inner diameter) restored anterior-segment anatomy and provided 360° structural support. Visual acuity improved from counting fingers to 20/200 before a later decline due to posterior subcapsular cataract, while the graft and central cornea remained clear. Maintenance therapy with methylprednisolone 28–48 mg/day, intravenous immunoglobulin every three weeks, and weekly rituximab achieved sustained inflammatory quiescence without recurrence of scleritis or uveitis. CONCLUSIONS: In extensive peripheral stromal thinning refractory to standard therapy, early annular keratoplasty can preserve ocular integrity when localized grafts are inadequate. Long-term disease control may require biologic escalation—particularly rituximab, with or without intravenous immunoglobulin, under multidisciplinary supervision.