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

Thomas Skovhus Prior; Nils Hoyer; Jesper Rømhild Davidsen; Saher Burhan Shaker; Malthe Pallesgaard Hundahl; Søren Lomholt; Bent Winding Deleuran; Elisabeth Bendstrup; Tue Wenzel Kragstrup

doi:10.1111/sji.13392

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

Thomas Skovhus Prior^*, Nils Hoyer, Jesper Rømhild Davidsen, Saher Burhan Shaker, Malthe Pallesgaard Hundahl, Søren Lomholt, Bent Winding Deleuran, Elisabeth Bendstrup, Tue Wenzel Kragstrup

^*Corresponding author af dette arbejde

Publikation: Bidrag til tidsskrift/Konferencebidrag i tidsskrift /Bidrag til avis › Tidsskriftartikel › Forskning › peer review

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.

Originalsprog	Engelsk
Artikelnummer	e13392
Tidsskrift	Scandinavian Journal of Immunology
Vol/bind	100
Nummer	3
ISSN	0300-9475
DOI	https://doi.org/10.1111/sji.13392
Status	Udgivet - sep. 2024

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title = "Fibroblast activation protein and disease severity, progression, and survival in idiopathic pulmonary fibrosis",

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.",

keywords = "fibroblast activation protein, idiopathic pulmonary fibrosis, prognosis",

author = "Prior, {Thomas Skovhus} and Nils Hoyer and Davidsen, {Jesper R{\o}mhild} and Shaker, {Saher Burhan} and Hundahl, {Malthe Pallesgaard} and S{\o}ren Lomholt and Deleuran, {Bent Winding} and Elisabeth Bendstrup and Kragstrup, {Tue Wenzel}",

note = "Publisher Copyright: {\textcopyright} 2024 The Author(s). Scandinavian Journal of Immunology published by John Wiley & Sons Ltd on behalf of The Scandinavian Foundation for Immunology.",

year = "2024",

month = sep,

doi = "10.1111/sji.13392",

language = "English",

volume = "100",

journal = "Scandinavian Journal of Immunology",

issn = "0300-9475",

publisher = "Wiley-Blackwell Publishing Ltd",

number = "3",

}

Fibroblast activation protein and disease severity, progression, and survival in idiopathic pulmonary fibrosis. / Prior, Thomas Skovhus; Hoyer, Nils; Davidsen, Jesper Rømhild et al.
I: Scandinavian Journal of Immunology, Bind 100, Nr. 3, e13392, 09.2024.

Publikation: Bidrag til tidsskrift/Konferencebidrag i tidsskrift /Bidrag til avis › Tidsskriftartikel › Forskning › peer review

TY - JOUR

T1 - Fibroblast activation protein and disease severity, progression, and survival in idiopathic pulmonary fibrosis

AU - Prior, Thomas Skovhus

AU - Hoyer, Nils

AU - Davidsen, Jesper Rømhild

AU - Shaker, Saher Burhan

AU - Hundahl, Malthe Pallesgaard

AU - Lomholt, Søren

AU - Deleuran, Bent Winding

AU - Bendstrup, Elisabeth

AU - Kragstrup, Tue Wenzel

PY - 2024/9

Y1 - 2024/9

N2 - 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.

AB - 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.

KW - fibroblast activation protein

KW - idiopathic pulmonary fibrosis

KW - prognosis

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U2 - 10.1111/sji.13392

DO - 10.1111/sji.13392

M3 - Journal article

C2 - 38849304

AN - SCOPUS:85195413500

SN - 0300-9475

VL - 100

JO - Scandinavian Journal of Immunology

JF - Scandinavian Journal of Immunology

IS - 3

M1 - e13392

ER -

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

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