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.