Management of the ocular manifestations of antiphospholipid syndrome

This literature review details the diagnosis of antiphospholipid syndrome (APS) and management of its ocular manifestations.

Summary

Ocular involvement is an important clinical manifestation of APS and can vary in presentations. The typical symptoms include monocular or binocular blurring of vision, amaurosis fugax, transient diplopia and transient visual field loss. Headaches and migraine-like visual symptoms have also been reported. Ocular manifestations of APS can present with anterior and posterior segment involvement and have a neuro-ophthalmological pathology.

Clinical findings in the anterior segment include conjunctivitis sicca, conjunctival vascular telangiectasias and microaneurysms, punctate epithelial keratopathy and limbal keratitis. Posterior segment involvement is typically associated with vaso-occlusive conditions such as arterial and vein occlusion, vasculitis and ischemic lesions. Neuro-ophthalmologic manifestations usually include nonarteritic or arteritic ischemic optic neuropathy (NAION or AION), extraocular motility disorders and central nervous system infarctions. Researchers also describe anterior uveitis associated with retinal vasculitis, episcleritis and scleritis as clinical findings of APS.

Anticoagulation therapy remains the standard treatment of APS, but novel target-specific therapy is under development.

Limitations

Since this is a review of previously published data, there may have been omissions of significant research and misinterpretation of the original source data.

Clinical significance

Antiphospholipid syndrome should be kept in mind in cases of vaso-occlusive ocular conditions and uveitis. Early diagnosis and treatment of APS can prevent thrombotic events and life-threatening morbidities. A multidisciplinary approach is crucial for treatment and complication prophylaxis.