Thrombotic cerebral arteriopathy in patients with the antiphospholipid syndrome.
Thrombotic cerebral arteriopathy in patients with the antiphospholipid syndrome.
复制标题
抗磷脂综合征患者的血栓性脑动脉病。
作者：
Hughson;McCarty Ga;Sholer Cm;Brumback Ra
目标
在血清抗磷脂抗体水平高的患者的小脑膜动脉中发现了一种独特类型的慢性脑血管病。本研究描述了血管病变的特征并研究了其发病机制。
设计
系统性红斑狼疮 (SLE) 和抗磷脂综合征死亡患者脑血管疾病的比较研究。
患者
将一名原发性抗磷脂综合征患者和一名患有系统性红斑狼疮和抗磷脂综合征的患者尸检中观察到的脑血管疾病与两名没有血清抗磷脂抗体的系统性红斑狼疮患者的结果以及15名患有可能预期或已知发生脑膜动脉病理变化的疾病的患者的结果进行比较（六名患有高血压脑血管疾病的患者，一名患有血栓性血小板减少性紫癜的患者，七名患有马兰氏或细菌性紫癜的患者）心内膜炎，以及一名患有左心室附壁血栓的患者）。通过连续组织学切片以及组织化学和免疫组织化学方法研究了多个脑组织块。在每个抗磷脂综合征病例中，对肾脏进行了免疫荧光和电子显微镜 (EM) 研究，并对大脑和脉络丛进行了 EM 研究。
结果
抗磷脂综合征患者的软脑膜动脉存在纤维蛋白血栓，并因内膜纤维组织或肌内膜细胞增殖而广泛阻塞。阻塞动脉的纤维和细胞段经常含有纤维蛋白血栓，并表现出不同的再通阶段。在组织的后期阶段，纤维网在动脉腔内形成。阻塞的动脉被追踪到位于皮层灰质下方的小梗塞。抗磷脂综合征患者的组织均未显示出活动性或已治愈的炎性血管炎或血管免疫复合物沉积的证据。在没有抗磷脂综合征的患者的软脑膜动脉中没有发现再通的血栓、纤维和细胞闭塞以及纤维网。
结论
抗磷脂综合征的脑血管变化源自慢性血栓性微血管病。这些发现支持抗磷脂抗体可导致血管内血栓反复发作的假设。
OBJECTIVE
A distinctive type of chronic cerebral vasculopathy was identified in the small leptomeningeal arteries of patients with high levels of serum antiphospholipid antibodies. This study characterizes the vascular lesions and investigates their pathogenesis.
DESIGN
A comparative study of cerebrovascular disease in patients dying of systemic lupus erythematosus (SLE) and the antiphospholipid syndrome.
PATIENTS
Cerebrovascular disease observed in autopsies on a patient with primary antiphospholipid syndrome and a patient with SLE and antiphospholipid syndrome was compared with findings on two SLE patients who did not have serum antiphospholipid antibodies and with findings on 15 patients having diseases in which pathological changes of meningeal arteries might be anticipated or are known to occur (six patients with hypertensive cerebrovascular disease, one patient with thrombotic thrombocytopenic purpura, seven patients with marantic or bacterial endocarditis, and one patient with a left ventricular mural thrombus). Multiple blocks of brain tissue were studied by serial histologic sections and histochemical and immunohistochemical methods. Immunofluorescent and electron microscopic (EM) studies were performed on kidneys and EM studies on brain and choroid plexus in each case of antiphospholipid syndrome.
RESULTS
Leptomeningeal arteries of antiphospholipid syndrome patients disclosed fibrin thrombi and widespread obstruction by a proliferation of intimal fibrous tissue or myointimal cells. The fibrous and cellular segments of obstructed arteries frequently contained fibrin thrombi and displayed varying stages of recanalization. In late stages of organization, fibrous webs were formed across arterial lumens. Obstructed arteries were traced to small infarcts localized to an underlying column of cortical gray matter. None of the tissues from antiphospholipid syndrome patients showed evidence of an active or healed inflammatory vasculitis or of vascular immune complex deposits. Recanalized thrombi, fibrous and cellular occlusions, and fibrous webs were not found in the leptomeningeal arteries of patients who did not have the antiphospholipid syndrome.
CONCLUSIONS
The cerebrovascular changes of the antiphospholipid syndrome are derived from a chronic thrombotic microangiopathy. The findings support the hypothesis that antiphospholipid antibodies can cause recurring episodes of intravascular thrombosis.