Cardiovascular Unit, University of Debrecen Medical and Health Sciences Center, Debrecen, Debrecen, Hungary.
Lupus. 2010 Nov;19(13):1520-6. doi: 10.1177/0961203310374336. Epub 2010 Aug 4.
Antiphospholipid syndrome (APS) is a distinct clinical entity characterized by arterial and venous thromboembolic events, recurrent fetal loss and the presence of antiphospholipid antibodies in the patients' sera. In primary APS, there is no detectable underlying disease, while overlap APS is associated with clinical syndromes including systemic autoimmune diseases, infections, or malignancies. We carried out a retrospective analysis of serological and clinical manifestations as well as assessed outcome-measures in 165 patients with primary APS. Thrombotic manifestations and possible signs of autoimmune diseases were determined at the time of the diagnosis, followed by the analysis of recurrent thrombotic events and effects of therapy during the follow-up period. Among the 165 patients with primary APS at onset, 105 patients (63%) remained primary APS after a mean 5.2 years of follow-up. In 14% of the patients, subsequently APS became associated with various characteristics of undifferentiated connective tissue disease. Finally 23% of patients evolved into a definitive systemic autoimmune disease during a mean 9.75 years of follow-up. Recurrent thrombotic events were registered in 24% of patients. Our results suggest that primary APS may be considered as a potential early phase of a dynamic transition towards a well-defined systemic autoimmune disease.
抗磷脂综合征（APS）是一种以动脉和静脉血栓栓塞事件、复发性胎儿丢失以及患者血清中存在抗磷脂抗体为特征的独特临床实体。在原发性 APS 中，没有可检测到的潜在疾病，而重叠性 APS 与包括系统性自身免疫性疾病、感染或恶性肿瘤在内的临床综合征相关。我们对 165 例原发性 APS 患者的血清学和临床表现进行了回顾性分析，并评估了预后指标。在诊断时确定了血栓形成表现和可能的自身免疫疾病迹象，然后在随访期间分析了复发性血栓形成事件和治疗效果。在 165 例原发性 APS 患者中，105 例（63%）在平均 5.2 年的随访后仍为原发性 APS。在 14%的患者中，随后 APS 与各种未分化结缔组织病的特征相关。最后，23%的患者在平均 9.75 年的随访中发展为明确的系统性自身免疫性疾病。24%的患者出现了复发性血栓形成事件。我们的结果表明，原发性 APS 可能被视为向明确的系统性自身免疫性疾病动态转变的潜在早期阶段。
