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Background <p>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.</p> Methods <p>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).</p> Results <p>We analysed 143 patients (106 men, 74%), with a median age of 70&#xa0;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 &lt; 0.05). Patients with short TL experienced a greater decline in lung function over 24&#xa0;months compared to those with normal TL (−&#xa0;4% vs + 3% FVC, p &lt; 0.05; −&#xa0;7% vs −&#xa0;3% DLCO, p &lt; 0.05). Patients with very short TL were younger at diagnosis and tended to have a more pronounced FVC decline (−&#xa0;5% vs −&#xa0;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%).</p> Conclusion <p>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.</p>

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Use of Telomere Length as a Biomarker in Idiopathic Pulmonary Fibrosis

  • 期刊论文

  • 发表日期：

    2025年7月9日

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  • Caroline Dahlqvist,
  • Thomas Planté-Bordeneuve,
  • Trejsi Muca,
  • Anne de Leener,
  • Benoît Ghaye,
  • Emmanuel Coche,
  • Anabelle Decottignies,
  • Marie-Astrid van Dievoet,
  • Antoine Froidure

摘要

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.