Two cases of lupus retinopathy with rapid progressive visual impairment treated with multiple immunosuppressants

Case 1, a 16-year-old male was admitted to our hospital because of a sudden decrease of visual acuity (VA) in the left eye. He was diagnosed with systemic lupus erythematosus (SLE) because of malar rashes, alopecia, leukopenia, hypocomplementemia, and a positive test for anti-nuclear antibody and antiphospholipid antibody (APA). Fundus examination showed bilateral cotton wool spots and retinal artery occlusions. After treatment with a methylprednisolone (mPSL) pulse, intravenous cyclophosphamide (IVCY) and rituximab (RTX) infusions, his VA improved from right (0.8) and left (0.4) at admission to right (1.2) and left (1.0). Case 2, a 21-year-old female was diagnosed as SLE from the findings of malar rashes, oral ulcer, leukopenia, and a positive test for anti-double-stranded-DNA antibody and APA. Two months later, she presented with abnormal sensation in her fingers, consciousness disorder, and visual impairment. Magnetic resonance imaging showed multiple high-intensity areas in both of her cerebral lesions. Neuropsychiatric SLE was diagnosed. Additionally, the ophthalmic examination showed severe ischemic retinopathy in both eyes. Despite intensive therapies with mPSL pulses, plasmapheresis, IVCY, and RTX infusions, there was no significant VA improvement [right (0.06) and left (counting fingers)]. The incidence of retinal involvement was 7%-26% of SLE patients and it was characterized by a wide variety of visual outcomes. These cases suggested that lupus retinopathy was significantly associated with disease activity or central nervous system involvement. Therefore it is important, visually and prognostically, to immediately and intensively treat patients who have severe lupus retinopathy. RTX can be an option in such treatment.