Preventing death in the catastrophic antiphospholipid (Asherson) syndrome.

• Vol 9 • August 2007 628 In 1992, Ronald Asherson [1] proposed a new and potentially fatal subset of the antiphospholipid syndrome that was named catastrophic APS, which is characterized by acute multi-organ failure (three or more damaged organ systems and histopathological evidence of multiple microthrombosis). Later, in 2003, his name was formally linked to the condition. The trigger is most commonly an infection [2], but other causes have been recognized, such as trauma, surgical procedures, malignancies, low international normalized ratio or warfarin withdrawal, obstetric causes and lupus flares. The “double” or “treble” hit hypothesis, applicable to patients with multi-organ failure in general, is also evident in some patients with catastrophic APS. Thus, a combination of two or three triggering factors has been evident in several cases. The mortality rate is about 50%, even in seemingly adequately treated patients. Among the causes of death, renal, pulmonary and multi-organ failure has been reported, but the most common cause of death is stroke. We describe here two cases in which death from catastrophic APS was prevented due to early detection of trigger factors, promptness in suspecting the diagnosis, and aggressive treatment in the intensive care unit.