Bucciarelli Silvia, Erkan Doruk, Espinosa Gerard, Cervera Ricard
Department of Autoimmune Diseases, Hospital Clínic, Catalonia, Spain.
Clin Rev Allergy Immunol. 2009 Jun;36(2-3):80-4. doi: 10.1007/s12016-008-8107-9.
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.
抗磷脂综合征(APS)的“灾难性” variant 表现为多发性血管闭塞事件,通常累及小血管且在短时间内发生。尽管灾难性 APS 患者占所有 APS 患者的比例不到 1%,但他们通常处于危及生命的状况,死亡率达 50%。本文旨在综述灾难性 APS 患者的治疗策略及预后因素。该综合征的临床和实验室特征的详细描述可在本期的其他文章中找到。