Catastrophic Antiphospholipid Syndrome (CAP, Asherson Syndrome)

 

Features:

(1) presence of multiple vascular occlusions, especially involving small blood vessels (DIC)

(2) occurs after a triggering event (infection, trauma, obstetrical delivery, other)

(3) develops rapidly (fulminant)

(4) associated with antiphospholipid antibodies

(5) patients often develop the systemic inflammatory response syndrome (SIRS) with multiple organ failures

(6) thrombocytopenic and microangiopathic anemia common

 

Patients at risk:

(1) SLE

(2) lupus-like disease

(3) primary antiphospholipid syndrome

 

Common causes of death:

(1) multi-organ failure

(2) cerebral involvement

(3) cardiac involvement

(4) infection