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

BACKGROUND

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.