Catastrophic antiphospholipid syndrome: treatment, prognosis, and the risk of relapse.
Antibodies, Monoclonal, Murine-Derived
Multiple Organ Failure
Adrenal Cortex Hormones
Lupus Erythematosus, Systemic
The "catastrophic" variant of the antiphospholipid syndrome (APS) is characterized by multiple vascular occlusive events, usually affecting small vessels and developing over a short period of time. Although patients with catastrophic APS represent less than 1% of all patients with APS, they are usually in a life-threatening situation with a 50% mortality rate. The purpose of this paper is to review the treatment strategies and prognostic factors in patients with catastrophic APS. A detailed description of the clinical and laboratory features of the syndrome can be found in the other articles of this issue.