恶性抗磷脂综合征的临床研究进展

Clinical study of catastrophic antiphospholipid syndrome/Asherson's syndrome

恶性抗磷脂综合征是1992年由Asherson提出的一种系统性自身免疫性疾病,临床以短期内进行性多脏器损伤、组织病理学证实有多发小血管栓塞及血清抗磷脂抗体滴度增高为特点。本病虽不足抗磷脂综合征发病的1%,但死亡率可达50%。未来的研究除考虑在血小板持续下降的情况下如何贯彻抗凝治疗外,应更关注于如何维持长期临床缓解、提高生存率的研究。

The descriptive adjective ＂catastrophic＂ was added to the antiphospholipid syndrome （APS） in 1992 by Asherson to highlight an accelerated form of this syndrome resulting in multiple organ failure. Patients with catastrophic APS have in common： progressive multiple organ injury in a short time, histopathologic evidence of multiple small vessel occlusions and laboratory confirmation of the presence of antiphospholipid antibodies, usually in higher titer. Although less than 1% of patients with the APS develop this complication, unfortunately, the mortality is extremely high （ around 50% ）. The combination of large - dose heparin, steroids plus repeated - dose gamma globulins or plasma exchange is used for the treatment of the patients with this severe condition. Additionally, besides how to perform anticoagulation therapy when platelets downfall continually, how to maintain clinical long -term relapse and increase survival rate should be paid more attention in the future.