Cervera R, Asherson R A, Lie J T
Department of Internal Medicine, Hospital Clínic, Barcelona, Catalonia, Spain.
Semin Arthritis Rheum. 1995 Feb;24(4):262-72. doi: 10.1016/s0049-0172(95)80036-0.
Since the original description and definition of the antiphospholipid syndrome (APS), a number of distinct clinical manifestations related to it have appeared in the literature. These include vascular obstruction of both veins and arteries, thrombus formation on the endocardium and its consequences, as well as a group of other conditions where vascular obstructive mechanisms are either incompletely understood or unproven, eg, chorea, avascular necrosis, and pulmonary hypertension. Single vessel (large/medium) involvement or multiple vascular occlusions may cause a wide variety of presentations. Any combination of vascular occlusive events may occur in the same individual, and the time interval between them also varies considerably from weeks to months or even years. Rapid chronological occlusive events occurring over days to weeks have been termed the "catastrophic" APS. Most of these complications may be ascribed to the hypercoagulable state of which antiphospholipid antibodies appear either to be "markers" or intimately connected with the highly complex coagulation mechanisms resulting in thrombotic occlusions.
自从抗磷脂综合征(APS)最初被描述和定义以来,文献中出现了许多与之相关的独特临床表现。这些表现包括静脉和动脉的血管阻塞、心内膜血栓形成及其后果,以及一组其他情况,在这些情况中,血管阻塞机制要么未被完全理解,要么未得到证实,例如舞蹈症、无血管性坏死和肺动脉高压。单支血管(大/中)受累或多处血管闭塞可能导致各种各样的症状。血管闭塞事件的任何组合都可能发生在同一个体中,而且它们之间的时间间隔也有很大差异,从数周数月到数年不等。在数天至数周内迅速发生的按时间顺序排列的闭塞事件被称为“灾难性”APS。这些并发症大多可归因于高凝状态,抗磷脂抗体在其中似乎要么是“标志物”,要么与导致血栓闭塞的高度复杂的凝血机制密切相关。