Kastriot Kastrati, Svitlana Pochepnia, Oana C Kulterer, Thomas S Nakuz, Daniel Mrak, Irina Gessl, Elisabeth Simader, Florian Prayer, Helmut Prosch, Lukas Nics, Stefan Schmitl, Daniel Aletaha, Helga Lechner-Radner, Marcus Hacker, Peter Mandl
{"title":"FAPI PET/CT在肌炎相关间质性肺疾病中的应用","authors":"Kastriot Kastrati, Svitlana Pochepnia, Oana C Kulterer, Thomas S Nakuz, Daniel Mrak, Irina Gessl, Elisabeth Simader, Florian Prayer, Helmut Prosch, Lukas Nics, Stefan Schmitl, Daniel Aletaha, Helga Lechner-Radner, Marcus Hacker, Peter Mandl","doi":"10.1016/j.jaut.2025.103471","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Interstitial lung disease (ILD) is associated with morbidity and mortality in idiopathic inflammatory myopathies (IIM). Predicting ILD progression remains a significant challenge, as conventional diagnostic tools such as pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) have limited prognostic accuracy. This study evaluated whether <sup>68</sup>Ga-labelled inhibitor of Fibroblast-Activation-Protein (FAPI) based PET/CT at baseline predicts ILD evolution over two years.</p><p><strong>Material and methods: </strong>In this prospective observational study, n = 19 individuals with IIM (n = 14 with ILD) underwent [<sup>68</sup>Ga] Ga-FAPI PET/CT at baseline. ILD progression was defined by three criteria: (1) FVC decline ≥10 % or FVC 5-9 % plus DLCO decline ≥15 %, (2) INBUILD criteria, and (3) a composite endpoint including INBUILD plus therapy escalation, hospitalization, or mortality. Pulmonary tracer uptake was quantified by calculating the maximum and mean target-to-background ratios across the whole lung (wlTBR<sub>max</sub> and wlTBR<sub>mean</sub>, respectively), derived from standardized uptake values corrected for blood pool activity, and their predictive value was analysed.</p><p><strong>Results: </strong>Over two years, n = 4 (28.6 %) patients met PFT-based progression criteria, while n = 6 (42.9 %) fulfilled INBUILD criteria, and n = 8 (57.1 %) reached the composite endpoint. Baseline wlTBR<sub>max</sub> was significantly higher in INBUILD progressors compared to non-progressors (2.68 ± 1.06 vs. 1.59 ± 0.80, p = 0.04), as was wlTBR<sub>mean</sub> (0.58 ± 0.22 vs. 0.34 ± 0.10, p = 0.04). Similarly, patients meeting the composite endpoint had higher wlTBR<sub>max</sub> (2.63 ± 1.04 vs. 1.30 ± 0.31; p < 0.01) and wlTBR<sub>mean</sub> (0.55 ± 0.20 vs. 0.31 ± 0.09; p = 0.01). Logistic regression analysis showed that incorporating pulmonary wlTBR<sub>max</sub> and wlTBR<sub>mean</sub> enhanced the predictive accuracy over PFT and HRCT alone.</p><p><strong>Conclusion: </strong>FAPI PET/CT may serve as a non-invasive biomarker for early prediction of ILD progression in IIM, supporting personalized disease management. However, given the small, single-centre cohort, these findings should be considered as preliminary and require validation in larger, multi-centre studies.</p>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"156 ","pages":"103471"},"PeriodicalIF":7.0000,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"FAPI PET/CT for tracking disease trajectory in myositis-related interstitial lung disease.\",\"authors\":\"Kastriot Kastrati, Svitlana Pochepnia, Oana C Kulterer, Thomas S Nakuz, Daniel Mrak, Irina Gessl, Elisabeth Simader, Florian Prayer, Helmut Prosch, Lukas Nics, Stefan Schmitl, Daniel Aletaha, Helga Lechner-Radner, Marcus Hacker, Peter Mandl\",\"doi\":\"10.1016/j.jaut.2025.103471\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Interstitial lung disease (ILD) is associated with morbidity and mortality in idiopathic inflammatory myopathies (IIM). Predicting ILD progression remains a significant challenge, as conventional diagnostic tools such as pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) have limited prognostic accuracy. This study evaluated whether <sup>68</sup>Ga-labelled inhibitor of Fibroblast-Activation-Protein (FAPI) based PET/CT at baseline predicts ILD evolution over two years.</p><p><strong>Material and methods: </strong>In this prospective observational study, n = 19 individuals with IIM (n = 14 with ILD) underwent [<sup>68</sup>Ga] Ga-FAPI PET/CT at baseline. ILD progression was defined by three criteria: (1) FVC decline ≥10 % or FVC 5-9 % plus DLCO decline ≥15 %, (2) INBUILD criteria, and (3) a composite endpoint including INBUILD plus therapy escalation, hospitalization, or mortality. Pulmonary tracer uptake was quantified by calculating the maximum and mean target-to-background ratios across the whole lung (wlTBR<sub>max</sub> and wlTBR<sub>mean</sub>, respectively), derived from standardized uptake values corrected for blood pool activity, and their predictive value was analysed.</p><p><strong>Results: </strong>Over two years, n = 4 (28.6 %) patients met PFT-based progression criteria, while n = 6 (42.9 %) fulfilled INBUILD criteria, and n = 8 (57.1 %) reached the composite endpoint. Baseline wlTBR<sub>max</sub> was significantly higher in INBUILD progressors compared to non-progressors (2.68 ± 1.06 vs. 1.59 ± 0.80, p = 0.04), as was wlTBR<sub>mean</sub> (0.58 ± 0.22 vs. 0.34 ± 0.10, p = 0.04). Similarly, patients meeting the composite endpoint had higher wlTBR<sub>max</sub> (2.63 ± 1.04 vs. 1.30 ± 0.31; p < 0.01) and wlTBR<sub>mean</sub> (0.55 ± 0.20 vs. 0.31 ± 0.09; p = 0.01). Logistic regression analysis showed that incorporating pulmonary wlTBR<sub>max</sub> and wlTBR<sub>mean</sub> enhanced the predictive accuracy over PFT and HRCT alone.</p><p><strong>Conclusion: </strong>FAPI PET/CT may serve as a non-invasive biomarker for early prediction of ILD progression in IIM, supporting personalized disease management. However, given the small, single-centre cohort, these findings should be considered as preliminary and require validation in larger, multi-centre studies.</p>\",\"PeriodicalId\":15245,\"journal\":{\"name\":\"Journal of autoimmunity\",\"volume\":\"156 \",\"pages\":\"103471\"},\"PeriodicalIF\":7.0000,\"publicationDate\":\"2025-09-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of autoimmunity\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1016/j.jaut.2025.103471\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/8/7 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q1\",\"JCRName\":\"IMMUNOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of autoimmunity","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.jaut.2025.103471","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/8/7 0:00:00","PubModel":"Epub","JCR":"Q1","JCRName":"IMMUNOLOGY","Score":null,"Total":0}
引用次数: 0
摘要
背景：间质性肺疾病（ILD）与特发性炎性肌病（IIM）的发病率和死亡率相关。预测ILD进展仍然是一个重大挑战，因为传统的诊断工具，如肺功能测试（pft）和高分辨率计算机断层扫描（HRCT）的预后准确性有限。本研究评估了68ga标记的纤维母细胞激活蛋白（FAPI）抑制剂在PET/CT基线时是否能预测2年内ILD的演变。材料和方法：在这项前瞻性观察性研究中，n = 19例IIM患者（n = 14例ILD患者）在基线时接受了[68Ga] Ga-FAPI PET/CT检查。ILD进展由三个标准定义：(1)FVC下降≥10%或FVC 5- 9%加DLCO下降≥15%，(2)INBUILD标准，(3)包括INBUILD加治疗升级、住院或死亡率的复合终点。通过计算整个肺的最大和平均目标-背景比（分别为wlTBRmax和wlTBRmean）来量化肺示踪剂的摄取，这些摄取来自校正了血池活性的标准化摄取值，并分析了它们的预测值。结果：两年内，n = 4（28.6%）例患者满足PFT-based进展标准，n = 6（42.9%）例患者满足INBUILD标准，n = 8（57.1%）例患者达到复合终点。INBUILD进展者的基线wlTBRmax显著高于非进展者（2.68±1.06 vs 1.59±0.80,p = 0.04）， wlTBRmean（0.58±0.22 vs 0.34±0.10,p = 0.04）。同样，达到复合终点的患者wlTBRmax更高(2.63±1.04 vs 1.30±0.31；P均值（0.55±0.20∶0.31±0.09）；p = 0.01)。Logistic回归分析显示，合并肺wlTBRmax和wlTBRmean比单独PFT和HRCT的预测准确性更高。结论：FAPI PET/CT可作为一种无创生物标志物，早期预测IIM中ILD的进展，支持个性化疾病管理。然而，考虑到小的单中心队列，这些发现应该被认为是初步的，需要在更大的多中心研究中进行验证。
FAPI PET/CT for tracking disease trajectory in myositis-related interstitial lung disease.
Background: Interstitial lung disease (ILD) is associated with morbidity and mortality in idiopathic inflammatory myopathies (IIM). Predicting ILD progression remains a significant challenge, as conventional diagnostic tools such as pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) have limited prognostic accuracy. This study evaluated whether 68Ga-labelled inhibitor of Fibroblast-Activation-Protein (FAPI) based PET/CT at baseline predicts ILD evolution over two years.
Material and methods: In this prospective observational study, n = 19 individuals with IIM (n = 14 with ILD) underwent [68Ga] Ga-FAPI PET/CT at baseline. ILD progression was defined by three criteria: (1) FVC decline ≥10 % or FVC 5-9 % plus DLCO decline ≥15 %, (2) INBUILD criteria, and (3) a composite endpoint including INBUILD plus therapy escalation, hospitalization, or mortality. Pulmonary tracer uptake was quantified by calculating the maximum and mean target-to-background ratios across the whole lung (wlTBRmax and wlTBRmean, respectively), derived from standardized uptake values corrected for blood pool activity, and their predictive value was analysed.
Results: Over two years, n = 4 (28.6 %) patients met PFT-based progression criteria, while n = 6 (42.9 %) fulfilled INBUILD criteria, and n = 8 (57.1 %) reached the composite endpoint. Baseline wlTBRmax was significantly higher in INBUILD progressors compared to non-progressors (2.68 ± 1.06 vs. 1.59 ± 0.80, p = 0.04), as was wlTBRmean (0.58 ± 0.22 vs. 0.34 ± 0.10, p = 0.04). Similarly, patients meeting the composite endpoint had higher wlTBRmax (2.63 ± 1.04 vs. 1.30 ± 0.31; p < 0.01) and wlTBRmean (0.55 ± 0.20 vs. 0.31 ± 0.09; p = 0.01). Logistic regression analysis showed that incorporating pulmonary wlTBRmax and wlTBRmean enhanced the predictive accuracy over PFT and HRCT alone.
Conclusion: FAPI PET/CT may serve as a non-invasive biomarker for early prediction of ILD progression in IIM, supporting personalized disease management. However, given the small, single-centre cohort, these findings should be considered as preliminary and require validation in larger, multi-centre studies.
期刊介绍：
The Journal of Autoimmunity serves as the primary publication for research on various facets of autoimmunity. These include topics such as the mechanism of self-recognition, regulation of autoimmune responses, experimental autoimmune diseases, diagnostic tests for autoantibodies, as well as the epidemiology, pathophysiology, and treatment of autoimmune diseases. While the journal covers a wide range of subjects, it emphasizes papers exploring the genetic, molecular biology, and cellular aspects of the field.
The Journal of Translational Autoimmunity, on the other hand, is a subsidiary journal of the Journal of Autoimmunity. It focuses specifically on translating scientific discoveries in autoimmunity into clinical applications and practical solutions. By highlighting research that bridges the gap between basic science and clinical practice, the Journal of Translational Autoimmunity aims to advance the understanding and treatment of autoimmune diseases.
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