Hawaii medical journal
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Hawaii medical journal · Dec 2004
Case ReportsCatastrophic antiphospholipid syndrome; a "CATASTROPHIC" case of systemic lupus erythematosus.
Less than 1% of patients with the antiphospholipid antibody syndrome (APS) can develop multiple, simultaneous organ-system thromboembolic disease, which is referred to as the catastrophic antiphospholipid antibody syndrome (CAPS). Roughly one-half of these patients have systemic lupus erythematosus (SLE). ⋯ Optimal treatment includes anticoagulation and high-dose corticosteroids, although IVIG and plasma exchange may also have a role. The overall mortality of CAPS is roughly 50%, but prompt clinical recognition of this rare but devastating syndrome may lead to improved outcomes.