The catastrophic antiphospholipid (Asherson's) syndrome in 2004--a review

An unusual variant of the antiphospholipid syndrome (APS) termed the Catastrophic Antiphospholipid Syndrome (CAPS) in 1992 by Asherson is described. The condition may arise "de-novo" in a patient previously not suspected of having an APS or during the course of a "Primary" APS or Secondary APS (most commonly SLE). The patient may already be on therapy. "Trigger" factors (infections most commonly) have been identified in 45% of patients but in the majority, they remain unidentified. Clinically, the patients present with small vessel occlusions involving organs (e.g. bowel, brain, heart, kidney) but large vessels occlusions do occur. Unusual organs are involved and the clinical features depend on which organs are affected. Because of tissue necrosis, the Systemic Inflammatory Response ensues ("SIRS") and many patients develop ARDS. Despite seemingly adequate therapy (parenteral heparin, steroids, antibiotics), the mortality remains high (approximately 50%).