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Catastrophic antiphospholipid syndrome: treatment, prognosis, and the risk of relapse
Silvia Bucciarelli1, Doruk Erkan, Gerard Espinosa
1Department of Autoimmune Diseases, Hospital Clínic, Catalonia, Spain.
Abstract:
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.
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