系统性红斑狼疮并发抗磷脂综合征临床研究进展

抗磷脂综合征(the antiphospholipid syndrome,APS)是以持续存在抗磷脂抗体(antiphospholipid antibodies,aPL)为特征,以动静脉血栓形成和妊娠并发症为主要特征的一种自身免疫性疾病。APS常伴发其他疾病,最常继发于系统性红斑狼疮(systemic lupus erythematosus,SLE)。SLE的存在或缺失可能会改变临床或血清学APS表达。除了经典的表现外,SLE相关的APS的临床表现还多见于关节痛、关节炎、自身免疫性溶血性贫血、网状青斑、癫痫、肾小球血栓和心肌梗死。SLE和APS/APL病人的管理应包括血管风险因素精确分层。SLE合并APS/aPL预后较差,因此亟需新的治疗方法。未来的目标是通过早期诊断和最佳预防性治疗改善病人的预后。

Clinical research advance in the antiphospholipid syndrome in patients with systemic lupus erythematosus

The antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis and pregnancy morbidity in the presence of pathogenic autoantibodies known as antiphospholipid antibodies (aPL).APS may be associated with other diseases,mainly systemic lupus erythematosus (SLE).The presence or absence of SLE might modify the clinical or serological expression of APS.Apart from the classical manifestations,the clinical manifestation of SLE associated APS are inculding arthralgias,arthritis,autoimmune hemolytic anemia,livedo reticularis,epilepsy,glomerular thrombosis and myocardial infarction.The management of patients with SLE and APS/aPL should include an accurate stratification of vascular risk factors.Novel approaches are needed since the prognosis of SLE patients with APS/aPL is still worse than that of SLE patients with negative aPL.The goal for the future is to improve the outcome of these patients by means of early recognition and optimal preventative treatment.