Fibroblast activation protein and disease severity, progression, and survival in idiopathic pulmonary fibrosis.

IF 4.1 4区 医学 Q2 IMMUNOLOGY
Scandinavian Journal of Immunology Pub Date : 2024-09-01 Epub Date: 2024-06-07 DOI:10.1111/sji.13392
Thomas Skovhus Prior, Nils Hoyer, Jesper Rømhild Davidsen, Saher Burhan Shaker, Malthe Pallesgaard Hundahl, Søren Lomholt, Bent Winding Deleuran, Elisabeth Bendstrup, Tue Wenzel Kragstrup
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Abstract

Idiopathic pulmonary fibrosis (IPF) is characterized by progressive fibrosis in the lungs. Activated fibroblasts play a central role in fibrogenesis and express fibroblast activation protein α. A truncated, soluble form (sFAP) can be measured in blood and is a potential novel biomarker of disease activity. The aim was to study the association between sFAP and clinical, radiological, and histopathological measures of disease severity, progression, and survival in a prospective, multicentre, real-world cohort of patients with IPF. Patients with IPF were recruited from the tertiary interstitial lung disease centres in Denmark and followed for up to 3 years. Baseline serum levels of sFAP were measured by ELISA in patients with IPF and compared to healthy controls. Pulmonary function tests, 6-minute walk test and quality of life measures were performed at baseline and during follow-up. The study included 149 patients with IPF. Median sFAP in IPF was 49.6 ng/mL (IQR: 43.1-61.6 ng/mL) and in healthy controls 73.8 ng/mL (IQR: 62.1-92.0 ng/mL). Continuous sFAP was not associated with disease severity, progression or survival (p > 0.05). After dichotomization of sFAP below or above mean sFAP + 2 SD for healthy controls, higher levels of sFAP were associated with lower FVC % predicted during follow-up (p < 0.01). Higher than normal serum levels of sFAP were associated with longitudinal changes in FVC % predicted, but sFAP did not show clear associations with other baseline or longitudinal parameters. As such, sFAP has limited use as a biomarker of disease progression or survival in patients with IPF.

成纤维细胞活化蛋白与特发性肺纤维化的病情严重程度、发展和存活。
特发性肺纤维化(IPF)的特点是肺部进行性纤维化。活化的成纤维细胞在纤维形成过程中发挥着核心作用,并表达成纤维细胞活化蛋白α。截短的可溶性形式(sFAP)可在血液中测量,是一种潜在的新型疾病活动生物标志物。研究的目的是在一个前瞻性、多中心、真实世界的 IPF 患者队列中,研究 sFAP 与疾病严重程度、进展和存活率的临床、放射学和组织病理学指标之间的关联。我们从丹麦的三级间质性肺病中心招募了IPF患者,并对他们进行了长达3年的随访。通过酶联免疫吸附法测定了 IPF 患者血清中 sFAP 的基线水平,并与健康对照组进行了比较。在基线和随访期间进行了肺功能测试、6 分钟步行测试和生活质量测量。研究共纳入了 149 名 IPF 患者。IPF患者的sFAP中位数为49.6纳克/毫升(IQR:43.1-61.6纳克/毫升),健康对照组为73.8纳克/毫升(IQR:62.1-92.0纳克/毫升)。连续的 sFAP 与疾病严重程度、进展或存活率无关(P > 0.05)。在对健康对照组的 sFAP 进行低于或高于平均 sFAP + 2 SD 的二分法处理后,较高水平的 sFAP 与随访期间较低的 FVC 预测百分比相关(p
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来源期刊
CiteScore
7.70
自引率
5.40%
发文量
109
审稿时长
1 months
期刊介绍: This peer-reviewed international journal publishes original articles and reviews on all aspects of basic, translational and clinical immunology. The journal aims to provide high quality service to authors, and high quality articles for readers. The journal accepts for publication material from investigators all over the world, which makes a significant contribution to basic, translational and clinical immunology.
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