Severe Vaso-Occlusive Retinopathy in Systemic Lupus Erythematosus: A Case Series

This study aims to describe the clinical profile of severe vaso-occlusive retinal disorders in patients with systemic lupus erythematosus (SLE) and it is a retrospective case series. The clinical characteristics of three patients with SLE with vascular occlusions in four eyes were described. Branch retinal artery occlusion (BRAO) was present in all three patients with combined non-ischemic central retinal vein occlusion (NICRVO) in one patient and evolving ischemic CRVO in another patient. Additional branch retinal artery insufficiency was observed in the other eye of a patient with BRAO. Antinuclear antibody (ANA) titer was elevated in all patients. One patient had a positive lupus anticoagulant with elevated activated partial thromboplastin time (aPTT), and concurrent homocysteinemia was present in another patient. Intravitreal anti-vascular endothelial growth factor (ranibizumab) injection was administered to two eyes. Intravenous methyl prednisolone (IVMP) injection along with oral azathioprine was used in all patients with the need for anticoagulation in two patients along with SLE treatment. Vision in two eyes did not improve to the functional level despite aggressive therapy. Visually blinding severe vaso-occlusive retinopathy in the form of BRAO with or without CRVO can manifest in patients with SLE. Undetected antiphospholipid syndrome and homocysteinemia may be associated risk factors for such ophthalmic complications.