抗磷脂综合征61例临床分析

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。

Clinical analysis of 61 patients with antiphospholipid syndrome

Objective To investigate the clinical manifestations, diagnosis and treatment of anti-phospholipid syndrome (APS). Methods 61 patients with defined APS admitted from Jan 1986 to Dec 2002 were analyzed retrospectively. Results 10 patients with primary APS and 51 patients with secondary APS were analyzed. Women were affected 3. 1 times as that of men. 48 of the 51 (94. 1% ) patients with secondary APS were complicated with other autoimmune diseases, including 33 cases (64. 7% ) of systemic lupus erythematosus. Vascular thrombosis was presented in around 80. 3% of the patients in this study. Thrombosis frequently involved the gastrointestinal system (21 cases, 22.6%), pulmonary system (19 cases ,20. 4% ) , the cerebral vascular system ( 17 cases, 18. 3% ) , lower limb deep venous system ( 16 cases, 17. 2% ) , and infrequently coronary arteries or adrenal glands. The abnormal pregnancy rate in the 37 married women was 45. 9%. The prevalence of anticardiolipin antibody (ACL) and lupus anticoagulant (LA) was 77. 0% and 62. 3% respectively. LA was associated with ACL Conclusion APS occurs most commonly among young women, is a disorder characterized by recurrent venous or arterial thrombosis and/or fetal losses associated with positive ACL or LA. Thrombosis frequently occurs in gastrointestinal system, pulmonary system, cerebral vascular system and deep venous system. The association between clinical features of APS and antiphospholipid antibody is significant. LA is a stronger risk factor for thrombosis and abnormal pregnancy than ACL Antiplatelet with low-dosage aspirin and long-term anticoagulation are main therapeutics.