Short Telomere Syndrome and Interstitial Lung Diseases: An Updated Clinical Review (2025)

Short telomere syndrome (STS) is a telomere biology disorder increasingly recognized across interstitial lung diseases, particularly idiopathic pulmonary fibrosis, pleuroparenchymal fibroelastosis, and fibrotic hypersensitivity pneumonitis (fHP). STS accelerates disease onset, worsens survival, and influences tolerance to immunosuppressive and transplant therapies. Diagnosis is guided by Flow-FISH-based telomere length <10 th percentile and variants in telomerase reverse transcriptase, telomerase RNA component, regulator of telomere elongation helicase 1, and poly(A)-specific ribonuclease. This review summarizes telomere biology, clinical spectrum, diagnostic pathways, and management, with emphasis on practical algorithms, transplant considerations, and emerging therapies. It also addresses challenges in resource-limited settings and highlights priorities for future research.