Service de Neurologie, AP-HP, Hôpital Lariboisière, Paris, France.
Mov Disord. 2011 Nov;26(13):2422-7. doi: 10.1002/mds.23863. Epub 2011 Jul 13.
The aim of this work was to describe chorea during systemic lupus erythematosus or antiphospholipid antibodies and its long-term outcome.
We retrospectively analyzed clinical features, laboratory findings, imaging characteristics, and outcome in a series of 32 patients.
Most patients were women (28 of 32), and mean age at onset of chorea was 20.6 (9-62) years. Chorea was inaugural for 28 patients. Improvement was observed with various treatments. During follow-up (12.2 ± 11.3 years), severe manifestations of systemic lupus erythematosus were rare. Antiphospholipid antibodies were repeatedly positive for 90% of the patients. Twelve patients developed arterial thrombosis. Prophylactic treatment with antithrombotic therapy might reduce the risk of further thrombosis (8% versus 57%; P = 0.01). Cardiac valvulopathy occurred in 22 patients during follow-up. Chorea relapsed in 8 cases.
Chorea had a good outcome in itself. This long-term follow-up shows, for the first time, that these patients have substantial risk for further arterial thrombosis.
本研究旨在描述系统性红斑狼疮或抗磷脂抗体患者的舞蹈症及其长期预后。
我们回顾性分析了 32 例患者的临床特征、实验室检查、影像学特征和结局。
大多数患者为女性(32 例中有 28 例),舞蹈症发病的平均年龄为 20.6(9-62)岁。28 例患者为首发症状。多种治疗方法均观察到改善。在随访期间(12.2±11.3 年),系统性红斑狼疮的严重表现罕见。90%的患者抗磷脂抗体持续阳性。12 例患者发生动脉血栓形成。预防性抗血栓治疗可能降低进一步血栓形成的风险(8%比 57%;P=0.01)。随访期间 22 例患者发生心脏瓣膜病。8 例患者出现舞蹈症复发。
舞蹈症本身预后良好。本次长期随访首次表明,这些患者存在动脉血栓形成的重大风险。