Sequential Cilioretinal Artery Occlusion and Acute Anterior Uveitis as the Initial Ocular and Systemic Manifestation of Systemic Lupus Erythematosus: A Case Report

PURPOSE: To report the sequential occurrence of cilioretinal artery occlusion (CLRAO) and acute anterior uveitis as the initial ophthalmic and systemic manifestation of systemic lupus erythematosus (SLE). METHODS: Case report. RESULTS: A 28-year-old previously healthy man presented with sudden, painless visual loss in the right eye (BCVA 0.5). Multimodal imaging was consistent with right CLRAO accompanied by paracentral acute middle maculopathy. Work-up, prompted by a history of recurrent oral aphthous ulceration, revealed a positive antinuclear antibody and elevated anti-double-stranded DNA, with an isolated positive lupus anticoagulant (anticardiolipin and anti-β2-glycoprotein-I negative), fulfilling the 2019 EULAR/ACR criteria for SLE. Carotid imaging showed no atherosclerotic plaque but total occlusion of the right carotid system, consistent with a lupus-associated thrombotic vasculopathy. The next day, before systemic therapy, he developed unilateral acute anterior uveitis with vitreous involvement (BCVA 0.3; +3 anterior chamber cells; fine non-granulomatous keratic precipitates). After intravenous methylprednisolone, topical corticosteroid and cycloplegic therapy, the inflammation resolved and BCVA improved to 0.9 at one month. Long-term hydroxychloroquine and warfarin were maintained. CONCLUSION: A cilioretinal artery occlusion and acute anterior uveitis occurred in close succession as the initial manifestation of SLE. Rather than a causal sequence, they likely represent occlusive and inflammatory manifestations of the same active disease appearing within a narrow window. Awareness of this presentation may prompt earlier systemic evaluation.