Orbital Microscopic Polyangiitis With Secondary Angle Closure Due to Uveal Effusion.

A 63 year-old man presented with 3 weeks of worsening, painless left-sided proptosis, ptosis, and diplopia. Examination of the affected eye revealed normal visual acuity, normal intraocular pressure in the presence of a shallow anterior chamber, grade 1 relative afferent pupillary defect, restricted motility in all directions of gaze, and 6 mm of proptosis. Ultrasound biomicroscopy revealed uveal effusions with anterior rotation of the ciliary body causing appositional angle closure. B-scan ultrasonography and MRI demonstrated enhancement of the posterior globe and an infiltrative mass involving the lacrimal gland and multiple extraocular muscles. Laboratory studies revealed positive perinuclear antineutrophil cytoplasmic antibody with elevated myeloperoxidase antibodies, consistent with a diagnosis of microscopic polyangiitis. Biopsy showed nonspecific dacryoadenitis. Intravenous and oral corticosteroids were associated with rapid improvement of all examination findings. The patient was later switched to rituximab steroid-sparing therapy and has shown no recurrence of disease at 3-months follow-up.