抗磷脂综合征57例临床分析

Antiphospholipid syndrome: A clinical analysis of 57 patients

目的分析抗磷脂综合征(antiphospholipid syndrome,APS)的临床表现、诊断及治疗,以提高本病的诊治水平。方法回顾性分析2004-2013年在本院诊断明确的57例APS患者的临床特征和免疫学特点。结果 57例中男性14例(24.6%),女性43例(75.4%),确诊时平均年龄(37±14)岁;原发性APS 21例(36.8%),继发性APS 36例(63.2%)。血栓发生率71.9%,血栓复发率41.5%,原发性APS的血栓复发率高于继发性APS(P<0.05);病态妊娠发生率63.3%。本组患者65.4%抗心磷脂抗体(anticardiolipin antibodies,ACL)阳性,71.9%抗β2-糖蛋白Ⅰ抗体阳性,42%血小板减少。在确诊APS前,43.9%被诊断为血栓事件,33.3%被诊断为自身免疫性疾病,8.8%被诊断为血小板减少。结论 APS临床表现以血栓形成、病态妊娠为主,免疫学特点为高滴度的ACL和(或)抗β2-糖蛋白Ⅰ抗体阳性。APS临床症状多样化导致其误诊率较高。

Objective To improve the diagnosis and treatment of antiphospholipid syndrome （APS） by analyzing its clinical manifestations,diagnosis and treatment.Methods Clinical and immunological features of 57 APS patients admitted to our hospital from 2004 to 2013 were retrospectively analyzed.Results Of the 57 APS patients,14 （24.6%） were males and 43 （75.4%） were females with an average age of （37 ± 14） years,21 （36.8%） were diagnosed with primary APS and 36 （63.2%） were diagnosed with secondary APS.The incidence of thrombosis was 71.9% and its recurrence rate was 41.5%.The recurrence rate of thrombosis was higher in primary APS patients than in secondary APS patients （P 〈 0.05）.The incidence of abnormal pregnancy was 63.3%.The anticardiolipin antibodies （ACL） and anti-β 2 glycoprotein Ⅰ antibodies were positive in 65.4% and 71.9% APS patients,respectively.The incidence of thrombocytopenia was 42%.Before APS was diagnosed,43.9%,33.3% and 8.8% patients were misdiagnosed as thrombosis,autoimmune diseases and thrombocytopenia,respectively.Conclusion APS is mainly manifested as thrombosis and abnormal pregnancy with positive ACL and/or anti-β 2 glycoprotein Ⅰ antibodies,and is thus frequently misdiagnosed.