恶性抗磷脂综合征7例临床分析并文献复习

目的：探讨恶性抗磷脂综合征（CAPS）的临床特点及预后，以期提高临床医生对该病的认识及诊治水平。方法：回顾性分析我院风湿科收治的7例恶性抗磷脂综合征患者临床表现、实验室检查及预后等资料并复习相关文献。结果：7例CAPS患者中，1例为原发APS，6例为SLE继发APS。静脉血栓发生5例次，主要是下肢深静脉血栓、眼中央静脉栓塞；动脉血栓13例次，以脑梗死为主（6例次）。6例抗心磷脂抗体阳性，3例抗β2-GPⅠ抗体阳性，3例狼疮抗凝物阳性。5例患者在1周内相继出现心、肺、肾等多器官功能衰竭而导致死亡。结论：CAPS患者短时间内发生大量微血栓形成，可导致多器官功能衰竭。本病的预后差，及早诊断、及时治疗是改善患者预后的关键。

Clinical Analysis of 7 Cases With Catastrophic Antiphospholipid Syndrome and Review of Literature

Objective:To investigate the clinical characteristics and prognosis of catastrophic antiphospholipid syndrome (CAPS), in order to improve physicain's recognization. Methods: The clinical manifestations and laboratory examinations of 7 patients with catastrophic antiphospholipid syndrome were analyzed retrospectively. Results: Of 7 CAPS patients, 1 was primary APS and 6 were secondary to SLE. Venous thrombosis occurred in 5 patients, mostly involved deep veins of lower extremities and central veins of retina. Of 13 arterial thrombosis, cerebral infarction (6 times) was the most commom one. Anticardiolipin antibody was positive in 6 patients, anti-beta-2 glycoprotein I antibody was positive in 3 patients, and lupus anticoagulant was positive in 3 patients. Death due to multiple organ failure within one week was seen in 5 patients. Conclusion: CAPS is characterized by development of extensive microvascular thromboses in multiple organs, occurring simultaneously or within a short period of time and causing multiple organ failure. As CAPS had high mortality, early diagnosis and treatment play key roles for improving the prognosis of CAPS.