Case Presentation: Twenty three year old female presented with complaints of severe headache and dizziness since past 2 weeks. Her past medical history was significant for SLE, heart failure with reduced ejection fraction and lupus pericarditis. CT head showed possible left cerebellar stroke of indeterminate age and hence was admitted for the stroke workup. Few hours later, she complained of sudden onset of painless loss of vision in her right eye. She was emergently transferred to ophthalmology clinic and was found to have diffuse macular edema with central cherry red spot and boxcarring of blood in arterioles consistent with CRAO. Ocular massage and paracentesis were attempted without success to resume the flow. The initial differentials were lupus vasculitis and arterial/cardiac embolism. Her workup for stroke was unremarkable – TTE/TEE with no thrombus, MRI/MRA brain showed no evidence of acute stroke however it showed chronic microangiopathic changes related to lupus. She was started on aspirin and oral high dose steroids for lupus flare (low C3, C4 level and high anti dsDNA); initiated therapeutic anticoagulation with enoxaparin bridged to warfarin for Antiphospholipid (APS) (positive for anticardiolipin and beta 2-microglobulin antibodies, lupus anticoagulant negative). On day four, she started having painful bluish discoloration of her left 4 and right 5 toe with intact pulses bilaterally. CT angiogram of the chest ruled out shaggy aorta. On day five, she had acute onset of left blurry vision with findings of CRAO on slit-lamp. Ocular massage was performed immediately and was started on topical levobunolol and acetazolamide. CT angiogram brain was unremarkable and fluorescein angiography showed delayed arterial filling. She fulfilled the criteria of Catastrophic Antiphospholid Syndrome (CAPS) and was started on intravenous pulse steroids and plasmapharesis. Her INR goal was increased to 3-3.5 with continuation of azathioprine and hydroxychloroquine. Her vision in left eye improved from 20/200 to 20/20 on near card test by the end of treatment.
Discussion: The incidence of CAPS in APS pts is 0.8% and reported mortality is 50% despite immunosuppression and anticoagulation. Large vessel occlusions in the brain and acute respiratory distress syndrome are the main reasons for mortality. CAPS is a triad of thrombosis of at least three different organ systems/tissues over a period of 1 week with histopathologic evidence of multiple small vessel occlusions and high titers of antiphospholipid antibodies. Major organs involved in >50% of cases are kidney, lungs, brain, heart and skin; with retinal involvement being <5%. The primary focus of treatment is to suppress thrombotic activity with immunosuppression, anticoagulation with INR goal of 2-3(if recurrent clot, then goal 3-3.5) and IVIG/plasma exchange. Our patient with acute development of bilateral retinal artery occlusion and bilateral bluish discoloration of toes in the presence of high APL titles was having probable CAPS after ruling out sepsis, DIC and systemic embolism. Plasmapheresis, IV steroids, higher goal of anticoagulation and immunosuppressants helped with the improvement in her left eye vision from 20/200 to 20/20 and halted further crisis.
Conclusions: It is important for the physicians to be cognizant for this fatal presentation of Catastrophic Antiphospholipid Syndrome causing bilateral retinal artery occlusion; and its aggressive management for potential recovery and to prevent further damage, as in our patient.