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大动脉炎诊断延迟50年:临床表现与治疗见解

A 50-Year Delay in the Diagnosis of Takayasu Arteritis: Clinical Presentation and Therapeutic Insights.

作者信息

Yasuda Takuya, Hagino Noboru

机构信息

Department of Nephrology and Rheumatology, Nakadori General Hospital, Akita, JPN.

Division of Rheumatology, Teikyo University Medical Center in Chiba, Ichihara, JPN.

出版信息

Cureus. 2025 May 18;17(5):e84361. doi: 10.7759/cureus.84361. eCollection 2025 May.

DOI:10.7759/cureus.84361
PMID:40535368
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12174477/
Abstract

Takayasu arteritis (TAK) is a rare inflammatory disease that affects large blood vessels, primarily targeting the aorta and its major branches. However, its etiology remains unknown. Although TAK is reported worldwide, it has a higher prevalence among women of Asian descent. Here, we report a rare case of TAK diagnosed 50 years after the onset of initial symptoms in a 76-year-old Japanese woman. The patient initially experienced numbness in her left upper extremities, followed by loss of consciousness. She had occasionally experienced difficulties with blood pressure measurements during routine medical examinations since the age of 20 years. Several months before admission, she had experienced visual darkening during physical activity and orthostatic dizziness that resolved with recumbency. Laboratory test results, including complete blood count, liver and renal function, C-reactive protein levels, and electrolyte levels, were within the normal limits. Computed tomography angiography (CTA) revealed stenosis of the bilateral subclavian arteries and the left common and internal carotid arteries, along with prominent collateral circulation, suggesting TAK. Given the substantial risk associated with corticosteroid-based therapy, we initiated treatment with aspirin and atorvastatin. After discharge, infliximab was started at 200 mg for three doses and then increased to a dose of 300 mg (5 mg/kg) every six weeks. Orthostatic symptoms markedly improved after the sixth infusion. Dizziness did not worsen during follow-up. Annual follow-up CTA and carotid ultrasonography showed no arterial stenosis progression.  This case report reveals that despite normal inflammatory marker levels, vascular stenosis of the carotid and subclavian arteries may cause dizziness that can be improved with infliximab therapy. Cases of TAK with marked progression despite normal inflammatory marker levels have been reported. Although the reversibility of vascular stenosis remains uncertain, infliximab therapy considerably improved dizziness and orthostatic symptoms. In patients with unexplained dizziness and orthostatic symptoms, large-vessel vasculitis, such as TAK, should be considered in the differential diagnosis, even in the absence of elevated inflammatory markers.

摘要

高安动脉炎(TAK)是一种罕见的炎症性疾病,主要累及大血管,以主动脉及其主要分支为主要病变部位。然而,其病因尚不清楚。尽管TAK在世界各地均有报道,但在亚洲裔女性中患病率较高。在此,我们报告一例罕见的TAK病例,该病例为一名76岁的日本女性,在初始症状出现50年后才得以确诊。患者最初出现左上肢麻木,随后意识丧失。自20岁起,她在常规体检时偶尔会出现血压测量困难。入院前几个月,她在体力活动时出现视力模糊,直立性头晕,平卧后症状缓解。实验室检查结果,包括全血细胞计数、肝肾功能、C反应蛋白水平和电解质水平,均在正常范围内。计算机断层扫描血管造影(CTA)显示双侧锁骨下动脉及左侧颈总动脉和颈内动脉狭窄,伴有明显的侧支循环,提示TAK。鉴于基于皮质类固醇的治疗存在重大风险,我们开始使用阿司匹林和阿托伐他汀进行治疗。出院后,开始使用英夫利昔单抗,初始剂量为200mg,共三剂,然后每六周增加至300mg(5mg/kg)。第六次输注后,直立性症状明显改善。随访期间头晕未加重。每年的随访CTA和颈动脉超声检查显示动脉狭窄无进展。 本病例报告显示,尽管炎症标志物水平正常,但颈动脉和锁骨下动脉的血管狭窄可能导致头晕,而英夫利昔单抗治疗可改善这种症状。已有报道称,尽管炎症标志物水平正常,但TAK仍有明显进展的病例。虽然血管狭窄的可逆性尚不确定,但英夫利昔单抗治疗显著改善了头晕和直立性症状。对于原因不明的头晕和直立性症状患者,即使炎症标志物未升高,在鉴别诊断中也应考虑大动脉血管炎,如TAK。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9d4/12174477/7bce67b8085b/cureus-0017-00000084361-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9d4/12174477/97a9ba728ff3/cureus-0017-00000084361-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9d4/12174477/5c005d932012/cureus-0017-00000084361-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9d4/12174477/7bce67b8085b/cureus-0017-00000084361-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9d4/12174477/97a9ba728ff3/cureus-0017-00000084361-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9d4/12174477/5c005d932012/cureus-0017-00000084361-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9d4/12174477/7bce67b8085b/cureus-0017-00000084361-i03.jpg

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本文引用的文献

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Current Diagnosis and Management of Takayasu Arteritis.高安动脉炎的当前诊断与管理
Int Heart J. 2023;64(4):519-534. doi: 10.1536/ihj.23-195.
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Efficacy and safety of TNF-α antagonists and tocilizumab in Takayasu arteritis: multicentre retrospective study of 209 patients.TNF-α 拮抗剂和托珠单抗治疗大动脉炎的疗效和安全性:209 例患者的多中心回顾性研究。
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Cyclophosphamide could be a better choice than methotrexate as induction treatment for patients with more severe Takayasu's arteritis.环磷酰胺可能是比甲氨蝶呤更好的选择,作为诱导治疗严重的多发性大动脉炎患者的药物。
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