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Takayasu 动脉炎:瑞士的患病率和临床表现。

Takayasu arteritis: Prevalence and clinical presentation in Switzerland.

机构信息

Department of Rheumatology, Immunology and Allergology, University Hospital (Inselspital) and, University of Bern, Bern, Switzerland.

Department of Diagnostic and Interventional Radiology, University Hospital (Inselspital) and, University of Bern, Bern, Switzerland.

出版信息

PLoS One. 2021 Jun 18;16(6):e0250025. doi: 10.1371/journal.pone.0250025. eCollection 2021.

DOI:10.1371/journal.pone.0250025
PMID:34143786
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8213155/
Abstract

OBJECTIVE

Takayasu arteritis (TAK) is a rare immune-mediated vasculitis of the aorta and its branches. Aims were to calculate prevalence and incidence in Switzerland, to assess disease activity and performance of MR-Angiography (MRA).

METHODS

31 patients were recorded in a database, 27 were followed prospectively up to 3 years. Prevalence was calculated based on data of the national statistical bureau. Disease activity was defined using the revised EULAR criteria. MRA depicted stenotic changes and aortic wall enhancement.

RESULTS

A disease prevalence of 14.5/1.000.000 inhabitants and an incidence of 0.3/1.000.000 per year was calculated. Aortic wall enhancement was found in 10 patients while in clinical and serological remission. EULAR criteria missed 5 patients with disease activity with isolated elevations of ESR/CRP. Arterial stenosis did not change over time in 5 cases, it improved in 2 and increased in 7. At follow-up 16 patients were treated with tocilizumab, 11/16 in monotherapy, 5 patients were treatment-free, 25/27 stayed in remission.

CONCLUSION

In addition to prevalence and incidence, our data show that MRA qualifies to detect subclinical disease activity, but, on the other hand, that EULAR criteria may miss disease activity in case of isolated elevation of ESR/CRP.

摘要

目的

Takayasu 动脉炎(TAK)是一种罕见的主动脉及其分支的免疫介导性血管炎。目的是计算瑞士的患病率和发病率,评估疾病活动度和磁共振血管造影(MRA)的表现。

方法

在数据库中记录了 31 名患者,其中 27 名患者前瞻性随访了 3 年。患病率根据国家统计局的数据计算。疾病活动度使用修订后的 EULAR 标准定义。MRA 描绘了狭窄性改变和主动脉壁增强。

结果

计算出疾病的患病率为 14.5/1000000 居民,发病率为 0.3/1000000 人/年。在临床和血清学缓解的情况下,发现 10 名患者存在主动脉壁增强。EULAR 标准遗漏了 5 名仅出现 ESR/CRP 升高的活动期患者。5 例动脉狭窄在随访期间无变化,2 例改善,7 例增加。16 名患者接受托珠单抗治疗,11 例为单药治疗,5 例为无治疗,27 例患者均处于缓解状态。

结论

除了患病率和发病率外,我们的数据还表明,MRA 有资格检测亚临床疾病活动度,但另一方面,在 ESR/CRP 孤立升高的情况下,EULAR 标准可能会遗漏疾病活动度。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dc7/8213155/32f02fc28252/pone.0250025.g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dc7/8213155/8b2e42b8f278/pone.0250025.g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dc7/8213155/367874c71dbb/pone.0250025.g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dc7/8213155/32f02fc28252/pone.0250025.g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dc7/8213155/8b2e42b8f278/pone.0250025.g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dc7/8213155/367874c71dbb/pone.0250025.g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dc7/8213155/32f02fc28252/pone.0250025.g003.jpg

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