Necrotizing Scleritis in Granulomatosis With Polyangiitis: A Clinical Challenge for an Ophthalmologists

Granulomatosis with polyangiitis (GPA) is a granulomatous disease with multisystem involvement, frequently with ocular manifestations. It can lead to ocular morbidity due to tissue melting and necrosis.

A 35-year-old male presented with a painless nodular lesion in the left eye, along with gradual diminution of vision and redness for 2 months. Eight months ago, he was diagnosed with GPA and left eye anterior uveitis. He was treated with topical steroids, topical cycloplegic, pulse cyclophosphamide, oral prednisolone, and azathioprine. At presentation, best corrected visual acuity in the right eye was 6/6 and 1/60 in the left. Left eye showed scleral thinning and necrosis in the superonasal quadrant with +1 cells in the anterior chamber. Intraocular pressure was 10 mmHg. Funduscopy examination showed exudative retinal detachment with hyperemic disc. Since refractory to topical and systemic immunomodulators, he underwent corneoscleral patch graft in the left eye for the progressive scleral thinning along with injection rituximab. Significant improvement was seen at 1 month with corneoscleral patch graft in situ, decreased inflammation with formed anterior chamber depth. However, at 2 months follow-up, the left eye showed worsening of ocular symptoms with progressive scleral thinning, necrosis, and graft lysis with total retinal detachment.

GPA-associated necrotizing scleritis is challenging. A collaborative timely management with aggressive compliant immunosuppressive therapy is a necessity to avoid ocular morbidity.