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目的分析抗磷脂综合征(APS)的临床特点、诊断和治疗方法,提高对本病的认识。方法对诊断明确、资料完整的61例APS患者的临床特点进行回顾性分析。结果61例患者中,男女比为1:3.1。共有原发性APS 10例和继发性APS51例,在51例继发性APS中,继发于自身免疫性疾病者48例(94.1%),其中系统性红斑狼疮33例(64.7%)。61例患者中有49例发生血栓事件,共93例次,以消化系统(21例次,22.6%),肺部(19例次,20.4%),神经系统(17例次,18.3%)和下肢深静脉血栓(16例次,17.2%)多见,另外还包括肾上腺和冠脉血栓等。37例有生育史的女性患者中17例(45.9%)有病态妊娠史。61例患者中有47例(77.0%)抗心磷脂抗体(ACL)阳性,38例(62.3%)狼疮抗凝物(LA)阳性,二者之间存在相关性(p<0.01)。结论APS以血栓形成、病态妊娠和ACL和/或LA阳性为特点;血栓好发部位为消化系统、肺、神经系统和深静脉。ACL、LA和临床表现之间存在显著相关性(P<0.01),但LA与临床表现的相关性大于ACL。抗血小板和长期抗凝治疗是主要的治疗方法。
Objective To analyze the clinical features, diagnosis and treatment of antiphospholipid syndrome (APS) and to improve the understanding of this disease. Methods The clinical features of 61 APS patients with definite diagnosis and complete data were retrospectively analyzed. Results 61 patients, male to female ratio was 1: 3.1. A total of 10 cases of primary APS and 51 cases of secondary APS in 51 cases of secondary APS, secondary to autoimmune diseases in 48 patients (94.1%), including 33 cases of systemic lupus erythematosus (64.7%). A total of 93 episodes of thromboembolic events occurred in 49 of 61 patients, with digestive system (21 cases, 22.6%), lung (19 cases, 20.4%), nervous system (17 cases, 18.3%) and Lower extremity deep venous thrombosis (16 cases, 17.2%) more common, in addition to adrenal and coronary thrombosis. Seventeen (45.9%) of 37 women with childbearing history had a history of morbid pregnancy. Of the 61 patients, 47 (77.0%) had positive anticardiolipin antibodies (ACL) and 38 (62.3%) had positive lupus anticoagulants (LA) (p <0.01). Conclusions APS is characterized by thrombosis, morbid pregnancy, and positive ACL and / or LA; the site of the thrombus is predominantly digestive, pulmonary, nervous and deep veins. There was a significant correlation between ACL, LA and clinical manifestations (P <0.01), but the correlation between LA and clinical manifestations was greater than that of ACL. Anti-platelet and long-term anticoagulant therapy is the main treatment.