De Silvestro Giustina, Tison Tiziana, Marson Piero
Universita' di Padova, Padova, Italy.
G Ital Nefrol. 2012 Jan-Feb;29 Suppl 54:S78-83.
Antiphospholipid syndrome (APS) is a rare clinical disorder characterized by thromboembolic manifestations and/or obstetric complications. Along with the clinical symptoms and signs, serum antiphospholipid antibodies have to be detected. APS can be primary, i.e., without any concomitant disorders, or secondary to other autoimmune diseases, particularly systemic lupus erythematosus. Criteria for the diagnosis of APS have been clearly established. Hyperacute APS (or catastrophic antiphospholipid syndrome), often with a poor prognosis, must meet four criteria: involvement of three or more organs, rapid evolution of clinical manifestations, microangiopathic occlusion of small blood vessels at biopsy, and presence of antiphospholipid antibodies. The rationale for apheresis treatment is the removal of pathogenetic antibodies involved in the development of tissue damage. Our experience includes 23 patients, in particular 15 women treated for 19 pregnancies. According to the National Guidelines Program, the effectiveness of apheresis in catastrophic syndrome has a level of evidence of V/VI, with a strength of recommendation A; in highrisk pregnancy it has a level of evidence of V with a strength of recommendation B. It will be necessary to better define the prognosis of various categories of pregnant patients with APS, as well as useful laboratory parameters to monitor its clinical course and anticipate any complications of pregnancy.
抗磷脂综合征(APS)是一种罕见的临床疾病,其特征为血栓栓塞表现和/或产科并发症。除了临床症状和体征外,还必须检测血清抗磷脂抗体。APS可以是原发性的,即无任何并发疾病,也可以继发于其他自身免疫性疾病,尤其是系统性红斑狼疮。APS的诊断标准已经明确确立。超急性APS(或灾难性抗磷脂综合征)通常预后较差,必须满足四个标准:累及三个或更多器官、临床表现迅速进展、活检时小血管的微血管闭塞以及存在抗磷脂抗体。血浆置换治疗的原理是清除参与组织损伤发生的致病性抗体。我们的经验包括23例患者,特别是15名接受了19次妊娠治疗的女性。根据国家指南计划,血浆置换在灾难性综合征中的有效性证据等级为V/VI,推荐强度为A;在高危妊娠中,其证据等级为V,推荐强度为B。有必要更好地明确各类APS妊娠患者的预后,以及监测其临床病程并预测妊娠任何并发症的有用实验室参数。