Abstract
A previously healthy 22-year-old African American woman presented with bilateral vision loss associated with headache. Her ocular examination was significant for bilateral retinal arterial “boxcarring,” retinal whitening, retinal hemorrhages, and cherry red spots. She was diagnosed with bilateral central retinal artery occlusions and was hospitalized due to concomitant diagnosis of stroke and hypercoagulable state. She was also found to be in heart failure and kidney failure. Rheumatology was consulted and she was diagnosed with catastrophic antiphospholipid syndrome in association with systemic lupus erythematosus. Approximately 7 months after presentation, the patient's vision improved and remained stable at 20/200 and 20/80.
Highlights
Catastrophic antiphospholipid syndrome (CAPS) is a rare autoimmune entity consisting of life threatening intravascular thrombosis leading to multiorgan failure
We present a rare case of CAPS in a previously healthy 22-year-old African American woman presenting with bilateral CRAO with profound vision loss
Though there can be overlap, catastrophic antiphospholipid syndrome is a distinct entity from primary antiphospholipid syndrome (APS) and systemic lupus erythematosus
Summary
Catastrophic antiphospholipid syndrome (CAPS) is a rare autoimmune entity consisting of life threatening intravascular thrombosis leading to multiorgan failure. The disease may present as a primary condition or in association with an underlying process, most commonly systemic lupus erythematosus (SLE). Specific diagnostic criteria include the involvement of at least three organ systems, evidence of intravascular thrombosis, and presence of antiphospholipid antibodies [1]. With only 5.8% of cases reporting retinal findings [2]. There has been one case report in the literature describing CAPS associated with choroidal thrombosis with good visual prognosis [3]. We present a rare case of CAPS in a previously healthy 22-year-old African American woman presenting with bilateral CRAO with profound vision loss
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