The physiopathology of the catastrophic antiphospholipid (Asherson's) syndrome: compelling evidence.

Catastrophic antiphospholipid (Asherson's) syndrome (cAPS) was described in the past as a severe variant of the antiphospholipid syndrome (APS). Currently growing evidence suggests it is a unique condition. This statement is based on several clinical and physiopathological features that although not well understood define cAPS by itself.

Catastrophic antiphospholipid (Asherson's) syndrome.

Catastrophic antiphospholipid syndrome is characterized by multiple organ involvement developing over a very short period of time, histopathological evidence of multiple small vessel occlusions, and laboratory confirmation of the presence of antiphospholipid antibodies. Knowledge of its treatment is vital as the outcome can be lethal.

Morbidity and mortality in the catastrophic antiphospholipid syndrome: pathophysiology, causes of death, and prognostic factors.

The catastrophic variant of the antiphospholipid syndrome (APS) is a condition characterized by multiple vascular occlusive events, usually affecting small vessels and evolving over a short period of time, together with laboratory confirmation of the presence of antiphospholipid antibodies. The pathogenesis of catastrophic APS is not completely understood. The mortality rate was ~50% in the earliest published series, but recently it has clearly fallen by some 20% due to the use, as first-line therapies, of full anticoagulation, corticosteroids, plasma exchanges, and intravenous immunoglobulins.