Ocular Manifestations in Antiphospholipid Syndrome

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A 26-year-old white man had blurred vision for 4 weeks. A sluggish light reflex was noted in the left eye. Funduscopic examination showed left-sided optic disc swelling with dot hemorrhages and hard exudates.

The patient's platelet count was low (10,000/µL), and his partial thromboplastin time was elevated (60 s). Results of further testing for lupus anticoagulant and anticardiolipin antibodies were positive, and antiphospholipid syndrome was diagnosed. A workup for secondary causes of antiphospholipid syndrome was negative.

The patient responded well to treatment with prednisone. When the platelet count rose to 163,000/µL, warfarin was started. The corticosteroid therapy was tapered over a month, and warfarin was continued.

Antiphospholipid syndrome is characterized by thromboembolism or recurrent pregnancy loss in association with antibodies to certain plasma proteins-most commonly, lupus anticoagulant, anticardiolipin, and anti-b2 glycoprotein antibodies.¹ A variety of clinical manifestations have been reported. In a series of 1000 patients with antiphospholipid syndrome, deep venous thrombosis occurred in 32%, thrombocytopenia in 22%, livedo reticularis in 20%, stroke in 13%, superficial thrombophlebitis in 9%, pulmonary embolism in 9%, fetal loss in 8%, transient ischemic attack in 7%, and hemolytic anemia in 7%.²

In patients with ocular involvement, either the anterior or posterior eye segments may be affected. Posterior segment abnormalities are more common and include optic disc swelling, hard exudates, hemorrhages, and microaneurysms. Anterior segment abnormalities include rubeosis iridis, conjunctival microaneurysms, conjunctival telangiectasias, and lamellar cataracts.³

The optimal duration of anticoagulation therapy is uncertain; however, most studies favor life-long treatment. The elimination of secondary risk factors for thrombosis and atherosclerosis is also necessary.²

Consider antiphospholipid syndrome in patients with ocular vaso-occlusive disease who have abnormal coagulation study results or who have features of connective-tissue disease.

 

References:

REFERENCES:

1.

Palomo IG, Segovia FM, Alarcon ML, et al. An insight into the pathophysiology of thrombosis in antiphospholipid syndrome. Front Biosci. 2007;12:3093-3103.

2.

Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med. 2002;346:752-763.

3.

Suvajac G, Stojanovich L, Milenkovich S. Ocular manifestations in antiphospholipid syndrome. Autoimmun Rev. 2007;6:409-414.