McNeil H P, Chesterman C N, Krilis S A
University of New South Wales, School of Medicine, St. George Hospital, Kogarah, Australia.
Adv Immunol. 1991;49:193-280. doi: 10.1016/s0065-2776(08)60777-4.
Having reviewed the literature on the association of aPL antibodies with clinical manifestations, it is clear that this group of autoantibodies are of considerable importance. The presence of aPL antibodies in some but not all individuals confers a risk of a clinical syndrome characterized by recurrent arterial or venous thrombosis, thrombocytopenia, hemolytic anemia, or positive Coombs' test, and in females, recurrent idiopathic fetal loss. In SLE, the risk is approximately 40%, compared with a risk of 15% in the absence of aPL antibodies. However, only one half of persons possessing these antibodies have SLE, and overall the risk is around 30%. In some circumstances, such as in chlorpromazine or infection-associated aPL antibodies, there appears to be no increased risk. At the other end of the spectrum are seen patients whose only clinical manifestations comprise features of this clinical syndrome, and this entity has been designated the primary antiphospholipid syndrome (PAPS). aPL antibodies are also important because they are not uncommon. They have been found frequently in women with idiopathic recurrent fetal loss (30%), in non-autoimmune patients with ischemic heart disease (20%), or venous thrombosis (up to 30%), or stroke (4-47%), and in chronic immune thrombocytopenia (30%). These autoantibodies can be detected using sensitive solid-phase immunoassays employing the CL antigen, or in appropriate coagulation tests to detect LA activity. These assays are simple to perform but require care in selection of the best test and in interpretation of results. Current tests do not distinguish between those persons at risk of the clinical events and those not at risk. Detection of specific isotypes (especially IgG) and antibody level may aid in such a designation. Treatment of aPL antibody-associated syndromes remains a controversial subject. Since thromboses are associated with significant morbidity and potential mortality, there is a good argument for long-term preventive antithrombotic therapy, at least for as long as the antibodies are detectable, in those patients in whom clinical complications have previously occurred. It is not generally recommended that this treatment be offered to individuals in whom aPL antibodies are detected but who have not suffered previous thromboses, since the risk of such events does not appear to be equal within a group of aPL antibody-positive persons. This particularly applies to pregnant women, since live births and uncomplicated pregnancies are observed regularly in the presence of aPL antibodies without specific treatment. A previous history of at least one unexplained, late fetal loss is considered a prerequisite before intervention in subsequent pregnancies.(ABSTRACT TRUNCATED AT 400 WORDS)
在回顾了关于抗磷脂（aPL）抗体与临床表现相关性的文献后，很明显这组自身抗体具有相当重要的意义。部分而非全部个体中存在aPL抗体，会使个体面临一种临床综合征的风险，该综合征的特征包括反复发生的动脉或静脉血栓形成、血小板减少、溶血性贫血或库姆斯试验阳性，在女性中还包括反复不明原因的胎儿丢失。在系统性红斑狼疮（SLE）中，这种风险约为40%，而在不存在aPL抗体的情况下风险为15%。然而，拥有这些抗体的人中只有一半患有SLE，总体风险约为30%。在某些情况下，如氯丙嗪或感染相关的aPL抗体，似乎不存在风险增加的情况。在谱系的另一端，有一些患者仅有的临床表现就是这种临床综合征的特征，这种情况被称为原发性抗磷脂综合征（PAPS）。aPL抗体也很重要，因为它们并不罕见。在不明原因反复胎儿丢失的女性中（30%）、患有缺血性心脏病的非自身免疫患者中（20%）、静脉血栓形成患者中（高达30%）、中风患者中（4 - 47%）以及慢性免疫性血小板减少患者中（30%）都经常发现aPL抗体。这些自身抗体可以使用采用CL抗原的敏感固相免疫测定法检测，或者在适当的凝血试验中检测狼疮抗凝物（LA）活性。这些检测方法操作简单，但在选择最佳检测方法和解读结果时需要谨慎。目前的检测方法无法区分有临床事件风险的人和无风险的人。检测特定的同种型（尤其是IgG）和抗体水平可能有助于进行这种区分。aPL抗体相关综合征的治疗仍然是一个有争议的话题。由于血栓形成与显著的发病率和潜在死亡率相关，对于那些先前已发生临床并发症的患者，有充分的理由进行长期预防性抗血栓治疗，至少在可检测到抗体期间进行治疗。一般不建议对检测到aPL抗体但未发生过血栓形成的个体进行这种治疗，因为在aPL抗体阳性人群中，此类事件的风险似乎并不相同。这尤其适用于孕妇，因为在存在aPL抗体且未进行特殊治疗的情况下，经常能观察到活产和正常妊娠。在干预后续妊娠之前，至少有一次不明原因的晚期胎儿丢失病史被视为一个前提条件。（摘要截取自第400个单词）
