Ocular manifestations of the antiphospholipid syndrome

Abstract

Antiphospholipid syndrome (APS) is an autoimmune disease characterised by a heterogenous group of antibodies directed against negatively charged phospholipids including antiphospholipid antibodies (aPL), anticardiolipin antibodies (aCL) and β-2 glycoprotein I (aβ-2-GP1). The major features of this disorder include arterial and venous thrombosis and recurrent fetal loss. The vasculature of the eye is frequently involved and may be the presenting manifestation. A diagnosis of APS should be considered in a young patients without traditional thromboembolic risk factors presenting with ocular vaso-occlusive disease. Management of these patients involves a team-approach with a haematologist/oncologist or rheumatologist to manage the coagulation status of these patients to prevent further systemic vascular occlusions.

Branch Retinal Artery Occlusion as a Presenting Feature of Anti-Phospholipid Antibody Syndrome and Homocysteinemia

“…8 However, presence of APS with hyperhomocysteinemia presenting with BRAO as its initial manifestation has not been described previously and is highlighted by means of this case report. Lowering of homocysteine levels with a combined folic acid, Vitamin B6, and Vitamin B12 supplementation should be considered in such patients to further reduce the risk of future thrombotic events.…”

“…7 Virtually any organ can be involved, and the presentation varies from acute ischaemia to chronic, indolent ischaemia. 8 The most common sites of venous thromboses are the deep veins of the legs, while the cerebral circulation is the most commonly affected arterial site. Ocular involvement can occur in 8-88% of patients of APS.…”

“…Ocular involvement can occur in 8-88% of patients of APS. 8,10 Ocular manifestations can be the first presenting clinical sign of APS. The severity of complications ranges from mild to potentially visually devastating.…”

“…Presenting symptoms may include monocular or binocular transient vision loss, transient diplopia, partial visual field loss, blurred vision, redness, pain, or headache. 8 Anterior, posterior and neuro-ophthalmic findings can all occur; however, posterior segment findings are the most common. 8,11 Retinal findings include venous tortuosity, dilation and flame haemorrhages.…”

“…8 Anterior, posterior and neuro-ophthalmic findings can all occur; however, posterior segment findings are the most common. 8,11 Retinal findings include venous tortuosity, dilation and flame haemorrhages. Classically, APS has been associated with unilateral and bilateral central retinal vein occlusion (CRVO), central retinal artery occlusion (CRAO), branched retinal vein occlusion (BRVO) and branched retinal artery occlusion (BRAO).…”