抗磷脂综合征165例临床特征与分型

目的 分析抗磷脂综合征(APS)的临床特征与分型.方法 根据2006年更新的APS分类诊断标准及新的临床亚型的定义,回顾性分析北京协和医院住院的165例APS患者的临床分型,总结患者的临床表现及治疗,并分析抗磷脂抗体与血栓的相关性.结果 165例患者中,男:女为1:3.不同临床亚型的分类包括确诊APS 116例(70.3%),可能APS 34例(20.6%),血清阴性APS 10例(6.1%),微血管性APS 5例(3.0%).124例(75.2%)合并其他疾病,其中113例(91.1%)合并自身免疫病,以系统性红斑狼疮常见(79.6%).合并血栓者121例(73.3%),其中静脉血栓68例(56.2%),以下肢深静脉血栓最常见.仅有狼疮抗凝物(LA)阳性和仅有抗心磷脂抗体(ACL)阳性患者的血栓发生率分别为86.0%和65.5%(P<0.05).61例APTT延长的患者中,LA阳性50例(82.0%),ACL阳性34例(55.7%).结论 根据APS临床表现可分为多种临床亚型.APS合并血栓以静脉血栓多见.LA阳性较ACL阳性的患者血栓发生率高.APTT延长与LA的相关性较强.

Classification and clinical analysis of 165 patients with antiphospholipid syndrome

Objective Based on 2006 revised classification criteria of definite antiphospholipid syndrome(APS)and definition of new APS subsets,we investigated clinical features of APS patients,correlation between thrombotic events and related antibodies Was assessed as well.Methods 165 patients with APS were enrolled and analysed retrospectively.Results 124 female and 41 male patients were included.116 cases(70.3%),34 cases(20.6%),10 cases(6.1%),and 5 cases(3.0%)were classified as definite APS,probable APS,seronegative APS,and microangiopathic APS(MAPS) respectively.Among 124 patients accompanied with other diseases,113(91.1%)were autoimmune diseases,mostly systemic lupus erythematosus(79.6%).121(73.3%)patients had episodes of thrombosis,with majority of deep vein thrombosis.The patients with positive test of lupus anticoagulant (LA)only were more likely to have episodes of thrombosis than with positive anticardiolipid antibody(ACL) only(86.0%versus 55.7%;P<0.05).Among 61 patients with prolonged APTT,the presence of LA was detected in 50(82.0%)patients,and ACL in 34(55.7%)patients.Conclusion APS patients could be classified into several subgroups according to their clinical features.Venous thromboses are more common than arterial thromboses,and positive of LA correlates thrombosis more than ACL