大血管参与抗中性粒细胞质抗体相关的血管炎
Mahmut S Kaymakci1, Mohanad M Elfishawi1, Hannah E Langenfeld2
1Division of Rheumatology, Department of Medicine, Mayo Clinic, Rochester, MN, USA.
Rheumatology (Oxford, England)
|September 6, 2023
概括
与ANCA相关的血管炎 (AAV) 可以影响大动脉,而不仅仅是小血管. 这项研究描述了大血管AAV (L-AAV) 患者,发现它可以涉及大动脉和动脉,并建议针对AAV的治疗.
科学领域:
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
- 血管医学 血管医学
背景情况:
- 与ANCA相关的血管炎 (AAV) 主要归类为小血管血管炎.
- 关于大血管参与AAV (L-AAV),包括动脉 (TA-AAV),大动脉 (A-AAV) 和大动脉周软组织 (PA-AAV) 的知识有限,通常基于病例报告.
- 这项研究旨在描述被诊断患有L-AAV.患者的临床特征.
研究的目的:
- 描述ANCA相关血管炎 (L-AAV) 大血管参与患者的临床特征和治疗.
- 为了解L-AAV做出贡献,这种L-AAV在医学文献中很少被描述.
- 为 L-AAV. 的临床管理和治疗考虑提供信息.
主要方法:
- 在2000年至2021年期间在梅奥诊所诊断出TA-AAV,A-AAV或PA-AAV的患者的回顾性鉴定.
- 包括标准包括年龄>18岁,满足2022年ACR/EULAR对GPA,MPA或EGPA的分类标准,阳性ANCA测试,以及多次门诊/住院访问.
- 收集的数据包括患者人口统计,ANCA类型,受影响的血管,诊断确认和接受的治疗.
主要成果:
- 该队列包括36名L-AAV患者 (平均年龄63.4岁,56%是男性).
- 17名患者有TA-AAV,10名患者有A-AAV,9名患者有PA-AAV. 大多数 (69%) 在一年内被诊断出L-AAV和AAV.
- 在69%的患者中,非大血管部位的AAV在组织病理学上得到确认. 常见的治疗方法包括葡萄糖皮质类药物,rituximab和甲状腺素.
结论:
- 这项研究呈现了迄今为止最大的L-AAV患者单中心队列.
- 与ANCA相关的血管炎可以表现为大动脉参与,尽管很少发生.
- 在诊断为L-AAV的患者的治疗中,应考虑针对AAV的治疗方法.
更多相关视频
09:16Supervised Machine Learning for Semi-Quantification of Extracellular DNA in Glomerulonephritis
Published on: June 18, 2020
6.9K
11:22Quantitative In vitro Assay to Measure Neutrophil Adhesion to Activated Primary Human Microvascular Endothelial Cells under Static Conditions
Published on: August 23, 2013
17.3K
相关概念视频
Peripheral Artery Disease I: Introduction
9
Peripheral artery disease (PAD) predominantly results from atherosclerosis, which involves the accumulation of fatty deposits, or plaques, within the walls of arteries. This causes them to narrow and harden, significantly reducing blood flow. PAD predominantly affects the legs but also impacts other areas, such as the arms, thereby impairing overall circulation and organ function.Etiology of PAD:The principal cause of PAD is atherosclerosis, which results from fatty deposits inside the arterial...
9
Peripheral Arterial Disease II: Clinical Manifestations and Diagnostic Evaluation
17
Clinical manifestationsPeripheral Arterial Disease (PAD) manifests through a range of symptoms, from the characteristic intermittent claudication to atypical presentations and severe complications in advanced stages. Intermittent claudication, a hallmark symptom of PAD, presents as exercise-induced muscle pain that typically resolves within minutes of rest. This pain is reproducible and stems from inadequate blood flow, leading to the accumulation of lactic acid produced during anaerobic...
17
