恶性抗磷脂综合征的临床研究新进展

The catastrophic antiphosphoHpid syndrome/Asherson＇s syndrome： an overview and update

恶性抗磷脂综合征（CAPS）是一种短期内进行性的广泛血栓形成导致多脏器功能衰竭直至死亡的自身免疫病。本文主要根据临床注册研究，探讨其临床表现、发病机制、诊断标准、治疗推荐及预后等。本病发病率低但死亡率极高，未来研究应在CAPS注册的基础上，更关注于治疗方案的不断改良及如何维持长期临床缓解、降低死亡率的研究。

Catastrophic antiphospholipid syndrome （ CAPS）, i.e. Asherson＇s syndrome, is characterized by diffuse small vessel thrombosis and acute multiple organ involvement which resulting in multiorgan failure over a short period of time. Although less than 1% of patients with the antiphospholipid syndrome （APS） develop this complication, unfortunately, the mortality is extreme high. The purpose of this paper is to summarize characteristics of CAPS which including clinical manifestation, pathogenesis, classification criteria, therapy strategy and prognosis. In parallel with CAPS registry, CAPS task force should pay more attention in advanced treatment recommendations revised which maintaining clinical long- term remission and increasing survival rate in the future.