Scleritis With Orbital Mass: A Vision-Threatening Masquerade

This case series describes two atypical presentations of scleritis associated with intraorbital mass lesions, highlighting the diagnostic challenges posed by this uncommon association. It emphasizes the need to consider IgG4-related disease and other inflammatory orbital disorders in the differential diagnosis when routine investigations are inconclusive.

The patients presented to a tertiary care ophthalmology centre in North India.

A 40-year-old male presented with a seven-month of visual decline in the left eye with recurrent redness and pain over the last five years, managed with prolonged systemic steroid therapy for scleritis. A similar episode in the right eye a decade earlier progressed to complete vision loss with proptosis and ophthalmoplegia, requiring exenteration for a suspected orbital tumour. Visual acuity was finger counting at 1/2 metre. Examination showed temporal scleral thinning with a 1 × 1 mm scleral melt, anterior chamber inflammation, vitritis, and disc edema. Inflammatory markers and serum IgG4 were elevated. Contrast-enhanced MRI revealed a multicompartmental orbital mass. Orbital and scleral biopsy demonstrated fibrotic changes with >10 IgG4-positive plasma cells per high-power field. Systemic corticosteroids followed by rituximab improved vision to 6/18 with resolution of inflammation and healing of the scleral melt. A 76-year-old female presented with one month of decreased vision with pain and lid swelling. Examination showed 180° peripheral corneal thinning with perforation, iris prolapse, and a 6 × 5.5 mm scleral melt. Fundus revealed peripheral exudative retinal detachment. PET-CT showed metabolically active orbital soft-tissue thickening initially suspected to be lymphoma. Biopsy revealed dense inflammatory infiltration consistent with idiopathic orbital inflammatory disease. She underwent scleral and crescentic corneal patch grafting with systemic steroid therapy.

Scleritis associated with an orbital mass should prompt early tissue diagnosis rather than prolonged steroid therapy alone. Reliance on long-term corticosteroids may mask the underlying pathology and lead to irreversible morbidity, including globe loss. Early confirmation of IgG4-related or other inflammatory orbital disease allows timely initiation of appropriate immunomodulatory therapy, which is critical to prevent steroid-related complications and preserve vision.