Cervera Ricard, Espinosa Gerard, Bucciarelli Silvia, Gómez-Puerta José A, Font Josep
Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain.
Autoimmun Rev. 2006 Dec;6(2):81-4. doi: 10.1016/j.autrev.2006.06.009. Epub 2006 Jul 21.
Although less than 1% of patients with the antiphospholipid syndrome (APS) develop the catastrophic variant, its potentially lethal outcome emphasizes its importance in clinical medicine today. However, the rarity of this variant makes it extraordinarily difficult to study in any systematic way. In order to put together all the published case reports as well as the new diagnosed cases from all over the world, an international registry of patients with catastrophic APS ("CAPS Registry") was created in 2000 by the European Forum on Antiphospholipid Antibodies. Currently, it documents the entire clinical, laboratory and therapeutic data of more than 300 patients whose data has been fully registered. This registry can be freely consulted at the Internet (www.med.ub.es/MIMMUN/FORUM/CAPS.HTM) and it is expected that the periodical analysis of these data will allow us to increase our knowledge of this condition.
尽管抗磷脂综合征(APS)患者中不到1%会发展为灾难性变体,但其潜在的致命后果凸显了其在当今临床医学中的重要性。然而,这种变体极为罕见,以至于以任何系统的方式对其进行研究都异常困难。为了汇总所有已发表的病例报告以及来自世界各地的新诊断病例,2000年欧洲抗磷脂抗体论坛创建了一个灾难性抗磷脂综合征患者国际登记处(“CAPS登记处”)。目前,它记录了300多名数据已完全登记患者的全部临床、实验室和治疗数据。该登记处可在互联网上免费查询(www.med.ub.es/MIMMUN/FORUM/CAPS.HTM),预计对这些数据的定期分析将使我们增加对这种病症的了解。