Catastrophic antiphospholipid syndrome: diagnosis and management in pregnancy.

Catastrophic antiphospholipid syndrome (CAPS) is a potentially lethal variant of antiphospholipid syndrome, characterized by multiorgan thrombosis in a short period of time, affecting mainly small vessels. In approximately 50% of CAPS cases, the catastrophic event is preceded by a trigger, mainly infections, or surgery, anticoagulation withdrawal, lupus flares, neoplasm, or pregnancy and puerperium. Treatment of CAPS is based on expert opinion and relies on a combination of several strategies, including anticoagulation, steroids, plasma exchange sessions, and/or intravenous immunoglobulins.