Observational studyThe Lancet. Respiratory medicine2014
Effect of telomere length on survival in patients with idiopathic pulmonary fibrosis: an observational cohort study with independent validation.
Observational study in The Lancet. Respiratory medicine, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 182 papers, 2 of them syntheses that pooled it.
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Who cites it
182 citing papers in PubMed, 2 syntheses or guidelines pooled it, 300 citations in OpenAlex.
- Approach to the Evaluation and Management of Interstitial Lung Abnormalities: An Official American Thoracic Society Clinical Statement.American journal of respiratory and critical care medicine · 2025Guideline
- Guideline
- TELO-SCOPE study: a randomised, double-blind, placebo-controlled, phase 2 trial of danazol for short telomere related pulmonary fibrosis.BMJ open respiratory research · 2021Trial
- Telomere Length and Use of Immunosuppressive Medications in Idiopathic Pulmonary Fibrosis.American journal of respiratory and critical care medicine · 2019Trial
- Performance of age-adjusted whole genome sequencing telomere length in idiopathic pulmonary fibrosis.American journal of respiratory and critical care medicine · 2026Article
- The telomeric DNA damage response as a therapeutic target in idiopathic pulmonary fibrosis.EMBO molecular medicine · 2026Article
- Short leukocyte telomere length across fibrotic interstitial lung disease phenotypes: An Indian pilot study.Lung India : official organ of Indian Chest Society · 2026Article
- Clonal hematopoiesis is associated with progression of idiopathic pulmonary fibrosis.JCI insight · 2026Article
- From ideal respiratory health to chronic lung disease: indicators of impaired respiratory health.Expert review of respiratory medicine · 2026Review
- Telomere-associated non-malignant pulmonary diseases: Pathogenic mechanisms and therapeutic strategies.Acta pharmaceutica Sinica. B · 2026Review
- A Proposed Clinical Diagnostic Framework for Short Telomere Syndrome.Clinical genetics · 2026Review
- Current Clinical Perspectives of Biomarkers in Respiratory Diseases: A Narrative Review.Journal of clinical medicine · 2026Review
- Pleuroparenchymal fibroelastosis-like findings identify an idiopathic pulmonary fibrosis subgroup with shortened telomeres and poor prognosis.ERJ open research · 2026Article
- Integrated phenotyping identifies reproducible prognostic subgroups in idiopathic pleuroparenchymal fibroelastosis.Respiratory research · 2026Observational
- Polygenic risk and rare variants in endotypes of idiopathic pulmonary fibrosis: a genetic analysis of population-based and case-control cohorts.The Lancet. Respiratory medicine · 2026Article
- The Fibrotic-Cancer Continuum in IPF: Shared Mechanisms, Clinical Implications and Therapeutic Challenges.Life (Basel, Switzerland) · 2026Review
- Interstitial lung abnormalities: a mechanistic window into early fibrotic lung disease.Frontiers in medicine · 2026Review
- Shortened Telomere Length as a Risk Factor for Idiopathic Pulmonary Fibrosis: A Meta-Analysis.The open respiratory medicine journal · 2026Review
- Interstitial lung abnormalities: to treat or not to treat? The hamlet dilemma.Frontiers in medicine · 2026Review
- Telomerase-active urine-derived stem cells: Regenerative solutions for aging.Ageing research reviews · 2026Review
122 more citing papers are in PubMed but not listed here.
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Authors and funding
16 authors at 4 institutions in 1 country.
Funding
Abstract
backgroundShort telomere lengths are found in a subset of patients with idiopathic pulmonary fibrosis, but their clinical significance is unknown. Our aim was to investigate whether patients with various blood leucocyte telomere lengths had different overall survival.
methodsIn this observational cohort study, we enrolled patients with interstitial lung disease from Dallas, TX (primary cohort), and from Chicago, IL, and San Francisco, CA (replication cohorts). We obtained genomic DNA samples from unrelated healthy controls in Dallas, TX, and spouses of patients were also enrolled as an independent control group. Telomere lengths were measured in genomic DNA samples isolated from peripheral blood obtained at the time of the initial enrolment assessment. The primary endpoint was transplant-free survival (ie, time to death or lung transplantation) in the Dallas cohort. Findings were validated in the two independent idiopathic pulmonary fibrosis cohorts (Chicago and San Francisco).
findings370 patients were enrolled into the Dallas cohort between June 17, 2003, and Aug 25, 2011. The 149 patients with idiopathic pulmonary fibrosis had shorter telomere lengths than did the 195 healthy controls (mean age-adjusted log-transformed ratio of telomere to single copy gene was -0.16 [SD 0.23] vs 0.00 [0.18]; p<0.0001); however, telomere lengths of the Dallas patients with idiopathic pulmonary fibrosis (1.33 [SD 0.25]) were similar to the 221 patients with other interstitial lung disease diagnoses (1.46 [0.24]) after adjusting for age, sex, and ethnicity (p=0.47). Telomere length was independently associated with transplant-free survival time for patients with idiopathic pulmonary fibrosis (HR 0.22 [95% CI 0.08-0.63]; p=0.0048), but not for patients with interstitial lung disease diagnoses other than idiopathic pulmonary fibrosis (HR 0.73 [0.16-3.41]; p=0.69). The association between telomere length and survival in patients with idiopathic pulmonary fibrosis was independent of age, sex, forced vital capacity, or diffusing capacity of carbon monoxide, and was replicated in the two independent idiopathic pulmonary fibrosis replication cohorts (Chicago cohort, HR 0.11 [0.03-0.39], p=0.00066; San Francisco cohort, HR 0.25 [0.07-0.87], p=0.029).
interpretationShorter leucocyte telomere lengths are associated with worse survival in idiopathic pulmonary fibrosis. Additional studies will be needed to establish clinically relevant thresholds for telomere length and how this biomarker might affect risk stratification of patients with idiopathic pulmonary fibrosis.
fundingUS National Heart, Lung, and Blood Institute, National Center for Advancing Translational Sciences, Harroun Family Foundation, and Nina Ireland Lung Disease Program.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.