Department of Clinical Chemistry and Hematology, University Medical Center Utrecht, 3584CX Utrecht, The Netherlands.
Curr Rheumatol Rep. 2011 Feb;13(1):70-6. doi: 10.1007/s11926-010-0144-8.
Patients diagnosed with the antiphospholipid syndrome typically suffer from vascular thrombosis, pregnancy morbidity, or a combination of the two. Due to the high prevalence of these clinical symptoms, the diagnosis of antiphospholipid syndrome is almost completely dependent on the detection of antiphospholipid antibodies in patient plasma. However, not every individual with antiphospholipid antibodies in his or her plasma suffers from thrombosis and/or pregnancy morbidity, which suggests the existence of different populations of antiphospholipid antibodies. Although many antigens have been identified in relation to the antiphospholipid syndrome, β2-glycoprotein I is regarded as clinically most significant. During the past decade, evidence has accumulated to suggest the presence of a dominant epitope on the first domain of β2-glycoprotein I. Several studies have detected a specific population of antibodies recognizing a cryptic epitope on domain I, at least comprising arginine 39 to arginine 43. In contrast to antibodies recognizing other domains of β2-glycoprotein I, anti-domain I antibodies are found to be highly associated with clinical symptoms. This review discusses several studies that have investigated a role for domain I within the antiphospholipid syndrome on a predominantly diagnostic level.
患有抗磷脂综合征的患者通常会遭受血管血栓形成、妊娠并发症，或两者兼有。由于这些临床症状的高患病率，抗磷脂综合征的诊断几乎完全依赖于患者血浆中抗磷脂抗体的检测。然而，并非每个血浆中有抗磷脂抗体的个体都会遭受血栓形成和/或妊娠并发症，这表明存在不同的抗磷脂抗体群体。尽管已经确定了许多与抗磷脂综合征相关的抗原，但β2-糖蛋白 I 被认为在临床上最为重要。在过去的十年中，有证据表明β2-糖蛋白 I 的第一个结构域上存在一个主要表位。几项研究检测到一种识别结构域 I 上隐蔽表位的特定抗体群体，至少包括精氨酸 39 到精氨酸 43。与识别β2-糖蛋白 I 其他结构域的抗体不同，抗结构域 I 抗体被发现与临床症状高度相关。这篇综述讨论了几项研究，这些研究主要在诊断水平上探讨了结构域 I 在抗磷脂综合征中的作用。
