Antiphospholipid Antibody Syndrome

This young woman presented with severe vision loss of the left eye and an unusual constellation of ischemic fundus findings, including both acute and chronic arterial and venous occlusions. She was subsequently diagnosed with the antiphospholipid antibody syndrome.

Most obvious is an acute superior macular branch retinal arterial occlusion. Scattered retinal hemorrhages in all quadrants are consistent with a prior central retinal vein occlusion. Both the major arteries and veins are sclerosed and there is disc neovascularization indicating chronic ischemia.

Despite anti-VEGF injections and panretinal photocoagulation she eventually went to no light perception vision. Remarkably the other eye remains normal.

Learning Points:
Antiphospholipid antibody syndrome, also known as anticardiolipin antibody syndrome or lupus anticoagulant syndrome, is caused by autoantibodies directed against phospholipid-bound proteins.

These predispose patients to venous and arterial thrombi and increase the risk for pregnancy complications and miscarriage.

The syndrome is more common in women and in those with other autoimmune disorders including systemic lupus.