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Catastrophic antiphospholipid syndrome (CAPS)
CAPS stands for catastrophic antiphospholipid syndrome (Asherson’s syndrome), which occurs in <1% of patients with Antiphospholipid Antibody Syndrome (both PAPS and SAPS).
Symptoms of Catastrophic antiphospholipid syndrome
Although rare, it is the initial presentation of Antiphospholipid Antibody Syndrome in 50% of patients who develop CAPS.
It is a very severe presentation and defined as having three or more organs involved simultaneously or within 1 week accompanied by histology showing predominately small vessel thrombosis in a patient with aPL abs.
The most common presenting manifestations are cardiopulmonary (25%) with acute respiratory failure from hemorrhage (11%), CNS manifestations (22%), abdominal pain (22%), renal insufficiency (14%), and cutaneous disease (10%). As the disease progresses, these organs are involved over 60% of the time. Thrombocytopenia occurs in 60% and hemolytic anemia in 33%.
In those who develop thrombotic microangiopathy, thrombocytopenia and hemolysis can be severe and needs to be differentiated from thrombotic thrombocytopenia purpura (TTP), hemolytic uremic syndrome (HUS), malignant hypertension, and disseminated intravascular coagulation. In pregnant patients, it must be separated from HELLP syndrome. The inciting event for CAPS is unknown in 45% of patients. Infections (20%) and surgery (14%) even when minor are known as inciting events.
Even with aggressive therapy, mortality is 30% to 50%. If they survive the episode of CAPS, 33% of patients get recurrent thrombosis over the next 5 years even on adequate anticoagulation.
