- Huijng Huang, physician internist,
- Lindi Jiang, physician internist
- Correspondence to: L Jiang, zsh-rheum{at}hotmail.com
This man in his early 30s developed bilateral scleritis, oculomotor deficits, and proptosis with incomplete eyelid closure resulting in corneal ulceration and severe conjunctivitis (fig 1). Orbital magnetic resonance imaging showed optic atrophy and enlargement of the extraocular muscles and lacrimal gland. The patient had received a diagnosis of granulomatosis with polyangiitis (GPA) vasculitis 11 years previously, and he had experienced disease progression and subsequent blindness despite treatment with steroids, immunosuppressants, monoclonal antibody immunotherapy, and radiotherapy.
Retro-orbital masses can develop secondary to GPA because of extension of the granulomatous inflammatory lesion from the paranasal sinuses into the orbit, or direct invasion of orbital granuloma.1 Differential diagnoses of retro-orbital masses include Graves’ ophthalmopathy, sarcoidosis, orbital lymphoma, IgG4 related disease, and orbital cellulitis. Patients might experience persistent visual impairment and blindness despite aggressive treatment.2