Purpose: The objective of the study is to report a rare case of severe vaso-occlusive retinopathy with bilateral simultaneous Central Retinal Artery Occlusion (CRAO) in a patient with Systemic Lupus Erythematosus (SLE). Observations: A female patient aged 22 years, presented with a sudden onset of painless diminution of vision in both eyes for three weeks. She had systemic features of SLE for six months at the time of the study. A diagnosis of bilateral CRAO was made after an examination of the fundus and by ruling out other causes of severe vaso-occlusion based on clinical and angiogram findings. Her antiphospholipid antibody (APLA) levels were normal. The visual prognosis was poor even after treatment with intravenous steroids and panretinal photocoagulation. Conclusion and importance: This case highlights the importance of bilateral CRAO as an initial presentation of severe systemic disease. This case demonstrates that despite apparent normal APLA levels, a state of hypercoagulability can exist in SLE patients. In addition, it demonstrates that severe vaso-occlusive complications such as CRAO, which results in blindness, can also develop in a patient with apparently well-controlled SLE. Therefore, it is important to take cognizance of this sight-threatening complication in SLE patients at initial presentation. A holistic approach to management, both systemic and ocular, is required to prevent sight-threatening complications from vaso-occlusion. Early and aggressive intervention can be beneficial in the prevention of severe visual loss.
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