风湿

原发性抗磷脂综合征患者远期预后

作者:作者:Taraborelli M,et al 翻译:北医三院段亚娟(libra_duanyajuan@163.com) 发布:柴静 审核:赵金霞 来源:中国风湿病公众论坛 日期:2017-08-12
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         前沿进展 | 原发性抗磷脂综合征患者远期预后:一项多中心回顾性研究

        目的:研究原发性抗磷脂综合征(PAPS)患者远期发生复发性血栓形成、产科并发症、器官损伤、严重并发症及发展成为结缔组织病(CTD)的风险。

        方法:回顾收集6个研究中心病史≥15年诊断原发性抗磷脂综合征患者的临床资料。

        结果:共计纳入115名患者:88%为女性,1983年至2014年间,平均诊断年龄为33(± 10)岁。在中位为18年(15-30年)的随访期间,50名(44%)患者至少发生1次血栓事件,共有75次,年发生率为3.5%。对于既往有血栓病史的患者更易再次发生血栓(p=0.002)。6名(5%)患者发生灾难性抗磷脂综合征。对于既往有血栓病史的患者,应用口服抗凝药物并没有有效地预防血栓复发(p=0.26)。52名女患者共计有87次怀孕,成功率达78%。29%患者发生器官功能损伤,器官功能损伤的发生与既往血栓史(p=0.004)、血管事件(p < 0.001)显著相关,尤其是中风,但与人口统计学、血清学、治疗无关。对于所有接受抗凝药物的患者,18名患者出现了严重的出血,共计24次。6名(5%)患者发生了严重感染,1名患者死亡。8名患者诊断有实体肿瘤(7%)。16名(14%)患者发展为自身免疫性疾病,13名(11%)为完全的结缔组织病。

        结论:原发性抗磷脂综合征患者尽管经过治疗,但仍有很高比例患者发生新的血栓事件和器官损伤,而发展成为结缔组织病并不多见。

        附原文:OBJECTIVE:To assess the longterm frequencyof thrombotic recurrences, obstetrical complications, organ damage, severecomorbidities, and evolution toward connective tissue disease (CTD) in primaryantiphospholipid syndrome (PAPS).METHODS:Medical records of patients with PAPSfollowed in 6 centers for≥15 years were retrospectively reviewed.RESULTS:One hundred fifteenpatients were studied: 88% women, followed between 1983 and 2014 with a mean (±SD) age at diagnosis of 33 (± 10) years. During a median followup of 18 years(range 15-30), 50 patients (44%) had at least a thrombotic event for a total of75 events and an annual incidence of 3.5%. Thromboses were more frequent inpatients with previous thrombotic history (p = 0.002). A catastrophicantiphospholipid syndrome occurred in 6 patients (5%). The use of oralanticoagulants in patients with thrombotic onset did not appear to beprotective against recurrences (p = 0.26). Fifty-two women had 87 pregnancies,successful in 78%. Twenty-nine percent of patients accrued functional damage. Damagewas significantly associated with a thrombotic history (p = 0.004) and witharterial events (p < 0.001), especially stroke, but not with demographics,serology, or treatment. Twenty-four major bleeding episodes were recorded in 18patients, all receiving anticoagulants. Severe infections affected 6 patients(5%), with 1 fatality. A solid cancer was diagnosed in 8 patients (7%).Altogether, 16 patients (14%) developed an autoimmune disease and 13 (11%) afull-blown picture of CTD.CONCLUSION:Despite therapy, a high proportion ofpatients experienced new thrombotic events and organ damage, while evolutiontoward CTD was infrequent.

        引自:Taraborelli M, Reggia R, Dall'Ara F, et al.Longterm Outcome of Patients with Primary Antiphospholipid Syndrome: ARetrospective Multicenter Study[J]. Journal of Rheumatology, 2017, 75(Suppl2):305.1-305.

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