抗磷脂综合征7例患者临床、影像及病理特点分析

目的探讨抗磷脂综合征(antiphospholipid syndrome,APS)的临床表现、生化检查、神经影像和皮肤病理特点。方法回顾性分析7例诊断为APS患者的临床资料、神经影像和皮肤病理资料。结果慢性起病3例,临床表现为反复头痛、记忆力下降为主;神经影像学检查结果示病灶分布于大脑皮质下白质及侧脑室旁白质,呈脑梗死或脱髓鞘改变,颅内中等血管狭窄、小血管闭塞或继发扩张;皮肤活检病理检查:光镜示真皮内小血管周围少量淋巴细胞浸润,电镜示上皮组织下小血管管壁增厚、管腔狭窄或闭塞。结论 APS以反复头痛或脑梗死为主要临床表现,影像学以多灶性、不对称性、脑白质缺血或脱髓鞘信号改变为特点,皮肤病理检查以小血管狭窄或闭塞为特点。熟悉APS的临床、影像学与病理学特点有利于对其做出早期诊断。

Study on clinical manifestation,imaging and pathology features of 7 patients with antiphospholipid syndrome

Objective To explore the clinical manifestation ,biochemical examinations ,neuroimaging and pathological characteristics of antiphospholipid syndrome (APS) .Methods The clinical ,neuroimaging and pathological data of 7 APS patients ,diagnosed by serum antibody with thrombus or miscarriages events ,were analyzed retrospectively .Results The onset of the disease was chronic in 3 patients with recurring headache and declined memory .Lesions were distributed in subcortical and periventricular white matter ,and mostly presented with multiple infarcts and demyelination on neuroimaging .Stenosis was observed in the intracranial medium vessels and occlusion or secondary expansions were observed in the small vessels .Small number of lymphocytes around the small vessels in derma were observed under a light microscope . Electronmicroscopy showed that capillary walls were thickened ,and stenosis or totally occlusion were observed in some small vessels .Conclusions Recurrent headaches and cerebral infarction are the main clinical manifestations of APS .Signal changes of multiple cerebral ischemia and demyelinating ,asymmetrically involved in both lobes of the brain white matter ,are the imaging characteristics of APS patients . Microvascular stenosis or occlusions are the main pathological changes .These clinical ,imaging and pathological features are helpful in early diagnosis of neurological APS .