Retinal vasculitis is an uncommon but potentially sight-threatening manifestation of systemic lupus erythematosus (SLE). The most common findings include retinal hemorrhage, “cotton-wool” spots, and vasoocclusion1,2. While the exact pathogenesis is unclear, it is thought that antiphospholipid antibodies, immune complex deposition, and complement activation are involved1. Systemic corticosteroids and cyclophosphamide are established treatment options for vasculitis, but case studies show promise with rituximab and plasma exchange3,4,5.
A 38-year-old woman with established SLE presented with acute onset of painless blurred vision and “floaters” in her right eye. She reported a 2-day history of headache, photosensitivity, arthralgia, and muscle weakness. Laboratory investigations revealed hemoglobin 9.8, erythrocyte sedimentation rate 58 mm/h, creatine phosphokinase 259, and proteinuria. Best corrected visual acuities were counting fingers at 6 feet in the right eye and 20/30 in the left eye. Visual fields were full to confrontation; external examination showed conjunctival hyperemia in both eyes. Slit lamp examination revealed anterior chamber cells, flare, and keratic precipitates in both eyes. Intraocular pressures were normal. There were 1+ white cells in the vitreous in both eyes. Fundus examination (Figure 1), fluorescein angiogram (Figure 2 and 3), and optical coherence tomography (Figure 4) were consistent with retinal vasculitis. Prednisone dose was increased from 10 mg to 60 mg daily and she completed 7 monthly infusions of cyclophosphamide. After 2 weeks, visual acuity in the right eye had improved to 20/200 and macular thickening had resolved. Five months later, visual acuity in the right eye measured 20/30, with fundus photography revealing resolution of retinal hemorrhages and intraocular inflammation (Figure 5). Mycophenolate mofetil is currently being used as maintenance therapy.