PDF(Pubmed)
Abstract：
Takayasu arteritis (TAK) is a rare primary systemic inflammatory vasculopathy. It is classified as a large-vessel vasculitis and is known to cause inflammatory aneurysms and vascular stenosis. Vascular Ehlers-Danlos syndrome (vEDS) is an autosomal dominant condition known to cause multiple aneurysms and arterial dissection at a young age owing to a mutation in the gene for type III collagen, COL3A1. Here, we present a case of TAK associated with vEDS with the development of multi-organ infarction of the brain, kidney, and spleen owing to multiple arterial aneurysms and stenosis of the internal carotid artery. The patient was successfully treated using anti-inflammatory agents, glucocorticoids, and tocilizumab with the addition of interventional radiology. In our case, a high inflammatory response led to vasculitis being the main cause of the disease with concurrent vEDS. When patients develop multiple aneurysms, stenosis, and dissections leading to multiple organ infarctions, a systemic differential diagnosis to consider concurrent vasculitis syndrome and non-inflammatory vasculopathy, including hereditary disorders, is important even with time constraints.
摘要：
大动脉炎（TAK）是一种罕见的原发性全身性炎症性血管病变。它被归类为大血管血管炎，已知会引起炎性动脉瘤和血管狭窄。血管Ehlers-Danlos综合征（vEDS）是一种常染色体显性遗传病，已知在年轻时由于III型胶原蛋白的基因突变而导致多发性动脉瘤和动脉夹层，COL3A1.这里,我们介绍了与vEDS相关的TAK与多器官脑梗死的发展，肾,和脾脏由于多发性动脉瘤和颈内动脉狭窄。患者使用抗炎药成功治疗,糖皮质激素,和托珠单抗加上介入放射学。在我们的案例中,高炎症反应导致血管炎是并发vEDS的主要原因。当患者出现多发性动脉瘤时，狭窄,解剖导致多器官梗塞，系统性鉴别诊断考虑并发血管炎综合征和非炎性血管病变，包括遗传性疾病,即使有时间限制也很重要。
