Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Multicenter Study
. 2025 Jul 9;203(1):78.
doi: 10.1007/s00408-025-00830-6.

Use of Telomere Length as a Biomarker in Idiopathic Pulmonary Fibrosis

Caroline Dahlqvist #  1   2 , Thomas Planté-Bordeneuve #  1   2 , Trejsi Muca  3 , Anne de Leener  2   4 , Benoît Ghaye  2   5 , Emmanuel Coche  2   5 , Anabelle Decottignies  6 , Marie-Astrid van Dievoet  7 , Antoine Froidure  8   9
Affiliations
Multicenter Study

Use of Telomere Length as a Biomarker in Idiopathic Pulmonary Fibrosis

Caroline Dahlqvist et al. Lung. .

Abstract

Background: Telomere shortening, a hallmark of cellular aging, is associated with poor outcomes in idiopathic pulmonary fibrosis (IPF). This study aimed to explore the relationships between telomere length (TL), pulmonary function tests, and telomere-related gene (TRG) mutations in a real-world IPF population.

Methods: We included IPF patients from two Belgian academic hospitals, collecting demographic and clinical data. TL was measured using Flow-FISH and expressed as a percentile. Short TL was defined as below the 10th percentile (P10), and very short TL as below the 1st percentile (P1).

Results: We analysed 143 patients (106 men, 74%), with a median age of 70 years. Thirty patients (21%) met the European Respiratory Society (ERS) criteria for familial pulmonary fibrosis (FPF). Short TL was found in 74 patients (50%), predominantly in men (p < 0.05). Patients with short TL experienced a greater decline in lung function over 24 months compared to those with normal TL (- 4% vs + 3% FVC, p < 0.05; - 7% vs - 3% DLCO, p < 0.05). Patients with very short TL were younger at diagnosis and tended to have a more pronounced FVC decline (- 5% vs - 1%, p = 0.06). TRG variants were identified in 16 individuals, occurring more frequently in those with short (14/27, 52%) or very short TL (10/20, 50%).

Conclusion: Short TL is common in both sporadic and familial IPF and serves as a predictive biomarker for accelerated lung function decline. Additionally, the presence of short TL is indicative of an underlying TRG mutation.

Keywords: Biomarker; Pulmonary fibrosis; Telomeres.

PubMed Disclaimer

Conflict of interest statement

Declarations. Competing Interests: The authors declare no competing interests.

References

    1. Richeldi L, Collard HR, Jones MG (2017) Idiopathic pulmonary fibrosis. Lancet 389:1941–1952 - DOI - PubMed
    1. Borie R, Kannengiesser C, Antoniou K, Bonella F, Crestani B, Fabre A et al (2023) European respiratory society statement on familial pulmonary fibrosis. Eur Respir J 61(3):2201383 - DOI - PubMed
    1. Zhang K, Xu L, Cong YS (2021) Telomere dysfunction in idiopathic pulmonary fibrosis. Front Med (Lausanne) 8:739810 - DOI - PubMed
    1. Armanios MY, Chen JJ, Cogan JD, Alder JK, Ingersoll RG, Markin C et al (2007) Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med 356(13):1317–1326 - DOI - PubMed
    1. Courtwright AM, El-Chemaly S (2019) Telomeres in interstitial lung disease: the short and the long of it. Ann Am Thorac Soc 16(2):175–181 - DOI - PubMed - PMC

Publication types

LinkOut - more resources