摘要
目的探讨抗磷脂综合征(APS)的临床特征和血清学特点。方法回顾性分析总结100例APS患者的临床表现和免疫学改变。结果原发性APS(PAPS)37例,继发性APS(SAPS)63例,其中继发于系统性红斑狼疮(SLE)46例、狼疮样综合征14例。80%患者出现血管性血栓形成,其中静脉血栓43例、动脉闭塞18例、静脉血栓和动脉闭塞15例。52%为单一部位,32%有2个部位,15%多个部位血栓。56%单次,25%2次,19%多次血栓形成。血栓事件以下肢深静脉血栓(36%)、肺栓塞(30%)和脑卒中(26%)常见。51%(34/67)女性出现病态妊娠,胎死宫内占37%,习惯性自发性流产13%。血小板减少71例。抗心磷脂抗体(AEL)和狼疮抗凝物(IA)的阳性率分别为84%和58%。SLE继发APS组关节炎、白细胞减少、抗核抗体(ANA)阳性和低补体血症较PAPS多见。男性患者下肢深静脉血栓和LA阳性较多见;而女性白细胞减少、ANA阳性和ACL阳性较多见。结论APS以血栓形成及病态妊娠为特点,高滴度ACL和(或)LA阳性是其免疫学特征。其临床表现受基础疾病(SLE)和性别的影响而有不同。
Objective To analyze the clinical and immunological manifestations of antiphospholipid syndrome （APS） in a cohort of 100 patients. Methods The clinical and serologic features of APS （Sapporo preliminary criteria） in 100 patients were analyzed retrospectively. Results The cohort consisted of 79 female patients and 21 male patients with a mean age of 36±13 years at diagnosis. Primary APS was presented in 37% of patients; APS was secondary to systemic lupus erythematosus （SLE） in 46%, lupus-like syndrome in 14%. Eighty percent of the patients had thrombosis,43 （54%） patients had venous thrombosis, 18 （22%） had arterial thrombosis, 15 （19%） had both arterial and venous thrombosis, 4 （5%） had thrombosis of microeireulation. Forty-two （52%） patients presented thrombosis at a single site, 26 （32%） at two sites, 12 （15%） at three or more sites. Forty-five （56%） patients experienced one thrombotic episode, 20 （25%） patients had only one recurrence, and 15 （19%） had more thrombosis. The most common manifestations of thrombosis were deep vein thrombosis （36%）, pulmonary embolism （30%） and stroke （26%）, with heart, kidney, gastrointestinal tract and other organs involvements. Thirty-four （51%） female patients had spontaneous fetal losses including intrauterine fetus death and recurrent spontaneous abortion. Seventy-one （71%） patients developed thrombocytopenia. The presence of anticardiolipin antibody （ACL） was detected in 84 patients （84%）. Among 90 patients with APS, alone ACL was detected in 38 patients （42%）, both ACL and lupus anticoagulant （LA） were detected in 36 （40%）, LA alone in 16 （18%）. Patients with APS associated with SLE or lupus-like syndrome had higher frequency of arthritis, leukopenia, antinuclear antibodies （ANA） and low complement levels. Female patients had a higher frequency of leukopenia, ANA and ACL. Male patients had a higher prevalence of deep venous thrombosis in the lower limbs and LA. Conclusion APS is an autoimmune disorder characterized by recurrent arterial and venous thrombosis, fetal loss, or thrombocytopenia with the presence of ACL and/or LA. In APS secondary to with SLE, the patient＇s sex can modify the disease expression and define specific subsets of APS.
出处
《中华风湿病学杂志》
CAS
CSCD
2007年第11期675-678,共4页
Chinese Journal of Rheumatology
关键词
抗磷脂综合征
血栓形成
抗心磷脂抗体
狼疮抗凝物
Antiphospholipid syndrome
Thrombosis
Anticardiolipin antibody
Lupus anticoagulant
