Review of severe vaso-occlusive retinopathy in systemic lupus erythematosus and the antiphospholipid syndrome: associations, visual outcomes, complications and treatment

Abstract

Purpose: To discuss the pathogenesis of severe vaso-occlusive retinopathy in systemic lupus erythematosus (SLE), the association with antiphospholipid antibodies, and its implications for management and prognosis.

Methods: An illustrative case history of a woman with SLE and severe vaso-occlusive retinopathy in the presence of antiphospholipid antibodies is presented. A literature review of previously reported cases and previously published data on the topic was performed and forms the basis for discussion.

Results: This is a rare form of retinopathy in SLE as distinct from the more common, benign form, being classically a microangiopathy with diffuse capillary non-perfusion and small arterial or arteriolar occlusions in the retina. Poor visual outcomes with visual loss are reported in 80% of cases with neovascularization occurring in 40% of cases. It is associated with antiphospholipid antibodies, typically characterized by microthrombosis and immune complex mediated vasculopathy rather than a true vasculitis. There is a strong association between this severe form of retinopathy and central nervous system manifestations of SLE. Anticoagulation has a role in the secondary prevention of thrombosis in the presence of antiphospholipid antibodies, but the role of aspirin and immunosuppression is unclear in the treatment of this condition. Vigilant ophthalmic follow up and aggressive treatment of neovascularization and vitreous haemorrhage can prevent further visual loss. These points are highlighted in the brief case report presented.

Conclusion: Severe vaso-occlusive retinopathy is a rare form of retinopathy in SLE often associated with poor visual prognosis and neovascularization. It may be a manifestation of the antiphospholipid syndrome. Treatment is aimed at preventing further thrombosis and complications arising from neovascularization.