Severe Vaso-occlusive Retinopathy as the Primary Manifestation in a Patient with Systemic Lupus Erythematosus

Severe vaso-occlusive retinopathy as the initial manifestation of systemic lupus erythematosus (SLE) is rare. We report a 16-year-old female who developed bilateral visual impairment. Fundus examinations showed bilateral “cherry-red spot” appearance, multiple confluent cotton wool spots, and widespread arterial occlusion. Laboratory studies showed leukopenia, antinuclear antibody (+), and anti-double-stranded DNA antibody (+). Malar rashes, oral ulcers, and bilateral knee joint tenderness were noted during physical examination. SLE was diagnosed and pulse therapy started immediately. Best corrected visual acuity of the left eye improved to 6/10 after treatment. However, there was no visual improvement in the right eye. Four months later, bilateral panretinal laser photocoagulation was performed due to retinal neovascularization. However, tractional retinal detachment of the right eye and vitreous hemorrhage of the left eye still occurred. After undergoing cryoretinopexy of the right eye and intravitreous tissue plasminogen activator injection of the left eye, the visual acuity of the patient's right eye remained hand movement only at 10 cm, but that of the left eye returned to 6/10. The ocular and systemic conditions were stable in the follow-up period of more than 2 years. This case demonstrates that in patients with severe vaso-occlusive retinopathy, a generalized immunological disorder, like SLE, should be suspected.