维克萨斯综合征是ANCA相关血管炎的模仿者
Franco Murillo-Chavez1, Brendan Antiochos2
1Department of Medicine, Sinai Hospital of Baltimore, Baltimore, MD, USA.
Rheumatology advances in practice
|October 16, 2024
概括
维克萨斯综合征可以模仿ANCA相关的血管炎 (AAV),带来诊断挑战. 这项研究强调了VEXAS作为AAV模仿剂,强调了它在非典型血管炎病例中的考虑.
科学领域:
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
- 遗传学 遗传学 是一个
背景情况:
- 从临床上来说,区分VEXAS综合征与原发性血管炎,特别是ANCA相关性血管炎 (AAV),是具有挑战性的.
- 维克萨斯综合征的特征是真空孔,E1酶,X链自炎症和体质突变,可以呈现与血管炎重叠的特征.
研究的目的:
- 审查VEXAS综合征呈现为AAV模仿器的临床特征.
- 在现有的文献中添加三个具有AAV表型的VEXAS新病例.
- 将发现与之前报告的病例进行比较.
主要方法:
- 鉴定了三个具有AAV表型的VEXAS病例.
- 对类似案件进行全面的文献搜索.
- 分析临床特征,包括肺部,皮肤,眼睛和脏的参与.
- 通过阳性UBA1突变分析确认VEXAS.
主要成果:
- 在VEXAS模仿AAV的常见发现包括肺透 (67%),皮肤卷入 (55%) 和眼部表现 (44%).
- 在44%的病例中观察到脏干扰,有些病例呈现出AAV类脏病变.
- 呈现异常特征的患者,如呼吸无症状的肺部发现或缺少鼻疾病.
结论:
- 在非典型的AAV呈现时应考虑VEXAS综合征,特别是当耐治疗时.
- 需要进一步的研究,以了解维克萨斯的AAV类表型的免疫基础.
- 识别VEXAS作为AAV模仿器可以提高诊断准确性和患者管理.
更多相关视频
09:29Induction of Paralysis and Visual System Injury in Mice by T Cells Specific for Neuromyelitis Optica Autoantigen Aquaporin-4
Published on: August 21, 2017
11.4K
06:35An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
7.2K
相关概念视频
01:25Endocarditis II: Clinical features and Diagnostic Tests
2
Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
2
01:21Aneurysm II: Clinical Manifestations and Diagnostic Studies
3
Thoracic, aortic arch and abdominal aneurysms are significant vascular conditions that can present with various clinical manifestations and lead to serious complications. Understanding these manifestations and the appropriate diagnostic studies is essential for effective management and treatment.Thoracic Aortic AneurysmsThoracic aortic aneurysms often remain asymptomatic until they reach a size that impinges on adjacent structures. They typically cause deep, diffuse chest pain that radiates to...
3