{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T03:25:51Z","timestamp":1780370751583,"version":"3.54.1"},"reference-count":90,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T00:00:00Z","timestamp":1740355200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Telomeropathies, or telomere biology disorders (TBDs), are syndromes that can cause a number of medical conditions, including interstitial lung disease (ILD), bone marrow failure, liver fibrosis, and other diseases. They occur due to genetic mutations to the telomerase complex enzymes that result in premature shortening of telomeres, the caps on the ends of cellular DNA that protect chromosome length during cell division, leading to early cell senescence and death. Idiopathic pulmonary fibrosis (IPF) is the most common manifestation of the telomere biology disorders, although it has been described in other interstitial lung diseases as well, such as rheumatoid arthritis-associated ILD and chronic hypersensitivity pneumonitis. Telomere-related mutations can be inherited or can occur sporadically. Identifying these patients and offering genetic counseling is important because telomerapathies have been associated with poorer outcomes including death, lung transplantation, hospitalization, and FVC decline. Additionally, treatment with immunosuppressants has been shown to be associated with worse outcomes. Currently, there is no specific treatment for TBD except to transplant the organ that is failing, although there are a number of promising treatment strategies currently under investigation. Shortened telomere length is routinely discovered in patients undergoing lung transplantation for IPF. Testing to detect early TBD in patients with suggestive signs or symptoms can allow for more comprehensive treatment and multidisciplinary care pre- and post-transplant. Patients with TBD undergoing lung transplantation have been reported to have both pulmonary and extrapulmonary complications at a higher frequency than other lung transplant recipients, such as graft-specific complications, increased infections, and complications related to immunosuppressive therapy.<\/jats:p>","DOI":"10.3390\/jcm14051496","type":"journal-article","created":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T05:36:47Z","timestamp":1740375407000},"page":"1496","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Telomeropathies in Interstitial Lung Disease and Lung Transplant Recipients"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2983-5741","authenticated-orcid":false,"given":"Brian D.","family":"Southern","sequence":"first","affiliation":[{"name":"Integrated Hospital-Care Institute, Department of Pulmonary Medicine, Cleveland Clinic, Cleveland, OH 44195, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shruti K.","family":"Gadre","sequence":"additional","affiliation":[{"name":"Integrated Hospital-Care Institute, Department of Pulmonary Medicine, Cleveland Clinic, Cleveland, OH 44195, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,2,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Turner, K.J., Vasu, V., and Griffin, D.K. (2019). Telomere Biology and Human Phenotype. Cells, 8.","DOI":"10.3390\/cells8010073"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1038\/305189a0","article-title":"Construction of artificial chromosomes in yeast","volume":"305","author":"Murray","year":"1983","journal-title":"Nature"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1016\/0092-8674(85)90170-9","article-title":"Identification of a specific telomere terminal transferase activity in Tetrahymena extracts","volume":"43","author":"Greider","year":"1985","journal-title":"Cell"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2664","DOI":"10.1056\/NEJMcpc1408595","article-title":"CASE RECORDS of the MASSACHUSETTS GENERAL HOSPITAL. Case 41-2015. A 14-Year-Old Boy with Immune and Liver Abnormalities","volume":"373","author":"Walter","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"2449","DOI":"10.1016\/j.chest.2020.07.025","article-title":"Synonymous Mutation in DKC1 Causes Telomerase RNA Insufficiency Manifesting as Familial Pulmonary Fibrosis","volume":"158","author":"Gaysinskaya","year":"2020","journal-title":"Chest"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1056\/NEJMoa066157","article-title":"Telomerase mutations in families with idiopathic pulmonary fibrosis","volume":"356","author":"Armanios","year":"2007","journal-title":"N. Engl. J. Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"827","DOI":"10.2147\/CIA.S256425","article-title":"Shelterin Complex at Telomeres: Implications in Ageing","volume":"15","author":"Mir","year":"2020","journal-title":"Clin. Interv. Aging"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1441","DOI":"10.1261\/rna.078953.121","article-title":"Dyskerin: An essential pseudouridine synthase with multifaceted roles in ribosome biogenesis, splicing, and telomere maintenance","volume":"27","author":"Garus","year":"2021","journal-title":"RNA"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1016\/j.tcb.2023.07.002","article-title":"The many faces of the helicase RTEL1 at telomeres and beyond","volume":"34","author":"Hourvitz","year":"2024","journal-title":"Trends Cell Biol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1482","DOI":"10.1038\/ng.3423","article-title":"Poly(A)-specific ribonuclease (PARN) mediates 3\u2032-end maturation of the telomerase RNA component","volume":"47","author":"Moon","year":"2015","journal-title":"Nat. Genet."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"7552","DOI":"10.1073\/pnas.0701009104","article-title":"Adult-onset pulmonary fibrosis caused by mutations in telomerase","volume":"104","author":"Tsakiri","year":"2007","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1361","DOI":"10.1378\/chest.14-1947","article-title":"Exome sequencing identifies mutant TINF2 in a family with pulmonary fibrosis","volume":"147","author":"Alder","year":"2015","journal-title":"Chest"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1183\/09031936.00040115","article-title":"Heterozygous RTEL1 mutations are associated with familial pulmonary fibrosis","volume":"46","author":"Kannengiesser","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1038\/ng.3278","article-title":"Exome sequencing links mutations in PARN and RTEL1 with familial pulmonary fibrosis and telomere shortening","volume":"47","author":"Stuart","year":"2015","journal-title":"Nat. Genet."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1517","DOI":"10.3324\/haematol.2018.215699","article-title":"DKC1 is a transcriptional target of GATA1 and drives upregulation of telomerase activity in normal human erythroblasts","volume":"105","author":"Richards","year":"2020","journal-title":"Haematologica"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1186\/s12967-021-02827-0","article-title":"Inhibition of DKC1 induces telomere-related senescence and apoptosis in lung adenocarcinoma","volume":"19","author":"Kan","year":"2021","journal-title":"J. Transl. Med."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"3764","DOI":"10.1093\/nar\/gkac179","article-title":"Telomerase RNA TERC and the PI3K-AKT pathway form a positive feedback loop to regulate cell proliferation independent of telomerase activity","volume":"50","author":"Wu","year":"2022","journal-title":"Nucleic Acids Res."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"17119","DOI":"10.1073\/pnas.0505318102","article-title":"The effect of TERC haploinsufficiency on the inheritance of telomere length","volume":"102","author":"Goldman","year":"2005","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"818","DOI":"10.1093\/hmg\/ddae015","article-title":"Clinical mutations in the TERT and TERC genes coding for telomerase components induced oxidative stress, DNA damage at telomeres and cell apoptosis besides decreased telomerase activity","volume":"33","author":"Calatayud","year":"2024","journal-title":"Human. Mol. Genet."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1212","DOI":"10.1158\/2159-8290.CD-16-1050","article-title":"TERT Promoter Mutations Enhance Telomerase Activation by Long-Range Chromatin Interactions","volume":"6","author":"Min","year":"2016","journal-title":"Cancer Discov."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1016\/j.redox.2017.10.004","article-title":"Acute telomerase components depletion triggers oxidative stress as an early event previous to telomeric shortening","volume":"14","year":"2018","journal-title":"Redox Biol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"8073","DOI":"10.1073\/pnas.0800042105","article-title":"Mutations in the telomerase component NHP2 cause the premature ageing syndrome dyskeratosis congenita","volume":"105","author":"Vulliamy","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"e61235","DOI":"10.7554\/eLife.61235","article-title":"TINF2 is a haploinsufficient tumor suppressor that limits telomere length","volume":"9","author":"Schmutz","year":"2020","journal-title":"eLife"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"608","DOI":"10.1182\/blood.2021013750","article-title":"Editing TINF2 as a potential therapeutic approach to restore telomere length in dyskeratosis congenita","volume":"140","author":"Choo","year":"2022","journal-title":"Blood"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1016\/j.cell.2004.05.026","article-title":"Regulation of Murine Telomere Length by Rtel: An Essential Gene Encoding a Helicase-like Protein","volume":"117","author":"Ding","year":"2004","journal-title":"Cell"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1016\/j.cell.2012.03.030","article-title":"RTEL1 Dismantles T Loops and Counteracts Telomeric G4-DNA to Maintain Telomere Integrity","volume":"149","author":"Vannier","year":"2012","journal-title":"Cell"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"e1735","DOI":"10.1002\/cnr2.1735","article-title":"Regulator of telomere elongation helicase 1 gene and its association with malignancy","volume":"6","author":"Hassani","year":"2023","journal-title":"Cancer Rep. (Hoboken)"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"e10201","DOI":"10.15252\/emmm.201810201","article-title":"Impaired telomere integrity and rRNA biogenesis in PARN-deficient patients and knock-out models","volume":"11","author":"Benyelles","year":"2019","journal-title":"EMBO Mol. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1016\/j.molcel.2019.04.031","article-title":"A-Tail of Telomerase RNA Maturation","volume":"74","author":"Corbett","year":"2019","journal-title":"Mol. Cell"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"2775","DOI":"10.1182\/blood-2014-05-526285","article-title":"Bone marrow failure and the telomeropathies","volume":"124","author":"Townsley","year":"2014","journal-title":"Blood"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"S447","DOI":"10.1513\/AnnalsATS.201609-718AW","article-title":"Telomerase and the Genetics of Emphysema Susceptibility. Implications for Pathogenesis Paradigms and Patient Care","volume":"13","author":"Stanley","year":"2016","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1038\/s41575-019-0165-3","article-title":"The role of telomeres and telomerase in cirrhosis and liver cancer","volume":"16","author":"Nault","year":"2019","journal-title":"Nat. Rev. Gastroenterol. Hepatol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"2925","DOI":"10.1111\/jocd.15992","article-title":"The role of telomerase in hair growth and relevant disorders: A review","volume":"22","author":"Wang","year":"2023","journal-title":"J. Cosmet. Dermatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"2947","DOI":"10.1182\/blood-2022-169845","article-title":"Avascular Necrosis and Minimal Trauma Bone Fractures in Patients with Dyskeratosis Congenita","volume":"140","author":"Niknafs","year":"2022","journal-title":"Blood"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"833","DOI":"10.1002\/JPER.20-0269","article-title":"Association of periodontitis with leukocyte telomere length in US adults: A cross-sectional analysis of NHANES 1999 to 2002","volume":"92","author":"Song","year":"2021","journal-title":"J. Periodontol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1038\/nrm.2016.171","article-title":"Telomeres in cancer: Tumour suppression and genome instability","volume":"18","author":"Maciejowski","year":"2017","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1038\/s41576-022-00527-z","article-title":"Genetics of human telomere biology disorders","volume":"24","author":"Revy","year":"2023","journal-title":"Nat. Rev. Genet."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1038\/ng1346","article-title":"Disease anticipation is associated with progressive telomere shortening in families with dyskeratosis congenita due to mutations in TERC","volume":"36","author":"Vulliamy","year":"2004","journal-title":"Nat. Genet."},{"key":"ref_39","doi-asserted-by":"crossref","unstructured":"Alder, J.K., Cogan, J.D., Brown, A.F., Anderson, C.J., Lawson, W.E., Lansdorp, P.M., Phillips, J.A., Loyd, J.E., Chen, J.J., and Armanios, M. (2011). Ancestral mutation in telomerase causes defects in repeat addition processivity and manifests as familial pulmonary fibrosis. PLoS Genet., 7.","DOI":"10.1371\/journal.pgen.1001352"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.1111\/resp.14145","article-title":"Pulmonary fibrosis in non-mutation carriers of families with short telomere syndrome gene mutations","volume":"26","author":"Goldschmeding","year":"2021","journal-title":"Respirology"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/S0092-8674(01)80006-4","article-title":"Telomere shortening and tumor formation by mouse cells lacking telomerase RNA","volume":"91","author":"Blasco","year":"1997","journal-title":"Cell"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"2369","DOI":"10.1128\/MCB.05312-11","article-title":"Phenotypes in mTERT+\/\u2212 and mTERT\u2212\/\u2212 mice are due to short telomeres, not telomere-independent functions of telomerase reverse transcriptase","volume":"31","author":"Strong","year":"2011","journal-title":"Mol. Cell. Biol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1016\/j.mayocp.2018.03.020","article-title":"Short Telomere Syndromes in Clinical Practice: Bridging Bench and Bedside","volume":"93","author":"Mangaonkar","year":"2018","journal-title":"Mayo Clin. Proc."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"2365","DOI":"10.1038\/nprot.2006.263","article-title":"Flow cytometry and FISH to measure the average length of telomeres (flow FISH)","volume":"1","author":"Baerlocher","year":"2006","journal-title":"Nat. Protoc."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1183\/09031936.00185114","article-title":"Global incidence and mortality of idiopathic pulmonary fibrosis: A systematic review","volume":"46","author":"Hutchinson","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1046\/j.1365-2141.2000.02109.x","article-title":"Dyskeratosis congenita in all its forms","volume":"110","author":"Dokal","year":"2000","journal-title":"Br. J. Haematol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1097\/MCP.0000000000000506","article-title":"Clinical implications of telomere dysfunction in lung fibrosis","volume":"24","author":"Borie","year":"2018","journal-title":"Curr. Opin. Pulm. Med."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1164\/rccm.200804-550OC","article-title":"Telomere shortening in familial and sporadic pulmonary fibrosis","volume":"178","author":"Cronkhite","year":"2008","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"13051","DOI":"10.1073\/pnas.0804280105","article-title":"Short telomeres are a risk factor for idiopathic pulmonary fibrosis","volume":"105","author":"Alder","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"636","DOI":"10.1001\/jamaoncol.2017.2316","article-title":"Association Between Telomere Length and Risk of Cancer and Non-Neoplastic Diseases: A Mendelian Randomization Study","volume":"3","author":"Haycock","year":"2017","journal-title":"JAMA Oncol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1710","DOI":"10.1183\/13993003.00308-2016","article-title":"Telomere-related lung fibrosis is diagnostically heterogeneous but uniformly progressive","volume":"48","author":"Newton","year":"2016","journal-title":"Eur. Respir. J."},{"key":"ref_52","doi-asserted-by":"crossref","unstructured":"\u0160elb, J., Osolnik, K., Kern, I., Koro\u0161ec, P., and Rijavec, M. (2022). Utility of Telomerase Gene Mutation Testing in Patients with Idiopathic Pulmonary Fibrosis in Routine Practice. Cells, 11.","DOI":"10.3390\/cells11030372"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1016\/S2213-2600(18)30135-8","article-title":"Analysis of protein-altering variants in telomerase genes and their association with MUC5B common variant status in patients with idiopathic pulmonary fibrosis: A candidate gene sequencing study","volume":"6","author":"Dressen","year":"2018","journal-title":"Lancet Respir. Med."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1164\/rccm.201809-1646OC","article-title":"Telomere Length and Use of Immunosuppressive Medications in Idiopathic Pulmonary Fibrosis","volume":"200","author":"Newton","year":"2019","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1513\/AnnalsATS.201808-508CME","article-title":"Telomeres in Interstitial Lung Disease: The Short and the Long of It","volume":"16","author":"Courtwright","year":"2019","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"1602314","DOI":"10.1183\/13993003.02314-2016","article-title":"Shared genetic predisposition in rheumatoid arthritis-interstitial lung disease and familial pulmonary fibrosis","volume":"49","author":"Juge","year":"2017","journal-title":"Eur. Respir. J."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"2002872","DOI":"10.1183\/13993003.02872-2020","article-title":"Leukocyte telomere length and mycophenolate therapy in chronic hypersensitivity pneumonitis","volume":"57","author":"Adegunsoye","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1016\/S2213-2600(17)30216-3","article-title":"The MUC5B promoter polymorphism and telomere length in patients with chronic hypersensitivity pneumonitis: An observational cohort-control study","volume":"5","author":"Ley","year":"2017","journal-title":"Lancet Respir. Med."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"1801641","DOI":"10.1183\/13993003.01641-2018","article-title":"Telomere length and genetic variant associations with interstitial lung disease progression and survival","volume":"53","author":"Newton","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"2000268","DOI":"10.1183\/13993003.00268-2020","article-title":"Telomere length in patients with unclassifiable interstitial lung disease: A cohort study","volume":"56","author":"Ley","year":"2020","journal-title":"Eur. Respir. J."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1922","DOI":"10.1056\/NEJMoa1515319","article-title":"Danazol Treatment for Telomere Diseases","volume":"374","author":"Townsley","year":"2016","journal-title":"N. Engl. J. Med."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"00131","DOI":"10.1183\/23120541.00131-2023","article-title":"No effect of danazol treatment in patients with advanced idiopathic pulmonary fibrosis","volume":"9","author":"Hoffman","year":"2023","journal-title":"ERJ Open Res."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"896","DOI":"10.1016\/j.stem.2020.03.016","article-title":"Small-Molecule PAPD5 Inhibitors Restore Telomerase Activity in Patient Stem Cells","volume":"26","author":"Nagpal","year":"2020","journal-title":"Cell Stem Cell"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"908","DOI":"10.1056\/NEJMoa1310523","article-title":"A Pilot Study of the Telomerase Inhibitor Imetelstat for Myelofibrosis","volume":"373","author":"Tefferi","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1093\/annonc\/mdu550","article-title":"A randomized phase II study of the telomerase inhibitor imetelstat as maintenance therapy for advanced non-small-cell lung cancer","volume":"26","author":"Chiappori","year":"2015","journal-title":"Ann. Oncol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"e31299","DOI":"10.7554\/eLife.31299","article-title":"Therapeutic effects of telomerase in mice with pulmonary fibrosis induced by damage to the lungs and short telomeres","volume":"7","author":"Povedano","year":"2018","journal-title":"eLife"},{"key":"ref_67","doi-asserted-by":"crossref","unstructured":"Pi\u00f1eiro-Hermida, S., Autilio, C., Mart\u00ednez, P., Bosch, F., P\u00e9rez-Gil, J., and Blasco, M.A. (2020). Telomerase treatment prevents lung profibrotic pathologies associated with physiological aging. J. Cell Biol., 219.","DOI":"10.1083\/jcb.202002120"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"2083","DOI":"10.1056\/NEJMoa1402582","article-title":"A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis","volume":"370","author":"King","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"2071","DOI":"10.1056\/NEJMoa1402584","article-title":"Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis","volume":"370","author":"Richeldi","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1718","DOI":"10.1056\/NEJMoa1908681","article-title":"Nintedanib in Progressive Fibrosing Interstitial Lung Diseases","volume":"381","author":"Flaherty","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"2003198","DOI":"10.1183\/13993003.03198-2020","article-title":"Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation","volume":"57","author":"Justet","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"1701875","DOI":"10.1183\/13993003.01875-2017","article-title":"Safety and efficacy of pirfenidone in patients carrying telomerase complex mutation","volume":"51","author":"Justet","year":"2018","journal-title":"Eur. Respir. J."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"654","DOI":"10.1016\/j.healun.2021.11.008","article-title":"Lung transplantation for idiopathic pulmonary fibrosis enriches for individuals with telomere-mediated disease","volume":"41","author":"Alder","year":"2022","journal-title":"J. Heart Lung Transplant."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1016\/j.healun.2017.02.005","article-title":"Telomere length in patients with pulmonary fibrosis associated with chronic lung allograft dysfunction and post-lung transplantation survival","volume":"36","author":"Newton","year":"2017","journal-title":"J. Heart Lung Transplant."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"1318","DOI":"10.1016\/j.healun.2015.05.002","article-title":"Clinical outcomes of lung transplant recipients with telomerase mutations","volume":"34","author":"Tokman","year":"2015","journal-title":"J. Heart Lung Transplant."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1016\/j.healun.2014.11.010","article-title":"Severe hematologic complications after lung transplantation in patients with telomerase complex mutations","volume":"34","author":"Borie","year":"2015","journal-title":"J. Heart Lung Transplant."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1164\/rccm.201805-0825OC","article-title":"Impaired Cytomegalovirus Immunity in Idiopathic Pulmonary Fibrosis Lung Transplant Recipients with Short Telomeres","volume":"199","author":"Popescu","year":"2019","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1183\/09031936.00060014","article-title":"Lung transplantation in telomerase mutation carriers with pulmonary fibrosis","volume":"44","author":"Silhan","year":"2014","journal-title":"Eur. Respir. J."},{"key":"ref_79","doi-asserted-by":"crossref","unstructured":"Courtwright, A.M., Lamattina, A.M., Takahashi, M., Trindade, A.J., Hunninghake, G.M., Rosas, I.O., Agarwal, S., Raby, B.A., Goldberg, H.J., and El-Chemaly, S. (2020). Shorter telomere length following lung transplantation is associated with clinically significant leukopenia and decreased chronic lung allograft dysfunction-free survival. ERJ Open Res., 6.","DOI":"10.1183\/23120541.00003-2020"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"1666","DOI":"10.1016\/j.healun.2023.08.001","article-title":"Impact of age and telomere length on circulating T cells and rejection risk after lung transplantation for idiopathic pulmonary fibrosis","volume":"42","author":"Snyder","year":"2023","journal-title":"J. Heart Lung Transplant."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"e14552","DOI":"10.1111\/ctr.14552","article-title":"Airway complications in lung transplant recipients with telomere-related interstitial lung disease","volume":"36","author":"Choi","year":"2022","journal-title":"Clin. Transplant."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"894","DOI":"10.1016\/j.jtcvs.2013.04.030","article-title":"Comparative study of bronchial artery revascularization in lung transplantation","volume":"146","author":"Pettersson","year":"2013","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"S1821","DOI":"10.21037\/jtd.2019.09.09","article-title":"Lung transplant with bronchial arterial revascularization: Review of surgical technique and clinical outcomes","volume":"11","author":"Yun","year":"2019","journal-title":"J. Thorac. Dis."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"1590","DOI":"10.1016\/j.ajt.2023.06.014","article-title":"Lung transplant recipients with telomere-mediated pulmonary fibrosis have increased risk for hematologic complications","volume":"23","author":"Hannan","year":"2023","journal-title":"Am. J. Transplant."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1182\/hematology.2020000170","article-title":"Extrahematopoietic manifestations of the short telomere syndromes","volume":"2020","author":"Schratz","year":"2020","journal-title":"Hematol. Am. Soc. Hematol. Educ. Program."},{"key":"ref_86","doi-asserted-by":"crossref","unstructured":"Kapse, B., Budev, M.M., Singer, J.P., and Greenland, J.R. (2024). Immune aging: Biological mechanisms, clinical symptoms, and management in lung transplant recipients. Front. Transplant., 3.","DOI":"10.3389\/frtra.2024.1356948"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1016\/j.healun.2021.12.005","article-title":"Cytomegalovirus prevention in thoracic organ transplantation: A single-center evaluation of letermovir prophylaxis","volume":"41","author":"Saullo","year":"2022","journal-title":"J. Heart Lung Transplant."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"1439","DOI":"10.1111\/ajt.15756","article-title":"Idiopathic pulmonary fibrosis lung transplant recipients are at increased risk for EBV-associated posttransplant lymphoproliferative disorder and worse survival","volume":"20","author":"Iasella","year":"2020","journal-title":"Am. J. Transplant."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"1747","DOI":"10.1097\/TP.0000000000002198","article-title":"Multiple Solid Organ Transplantation in Telomeropathy: Case Series and Literature Review","volume":"102","author":"Lebeer","year":"2018","journal-title":"Transplantation"},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"840","DOI":"10.1002\/lt.25734","article-title":"Combined Lung and Liver Transplantation for Short Telomere Syndrome","volume":"26","author":"Moschouri","year":"2020","journal-title":"Liver Transpl."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/14\/5\/1496\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T16:41:03Z","timestamp":1760028063000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/14\/5\/1496"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,2,24]]},"references-count":90,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2025,3]]}},"alternative-id":["jcm14051496"],"URL":"https:\/\/doi.org\/10.3390\/jcm14051496","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,2,24]]}}}