Lurdes Planas-Cerezales1,2, Elena G Arias-Salgado3,4, Ivette Buendia-Roldán5, Ana Montes-Worboys1,2, Cristina Esquinas López1,6, Vanesa Vicens-Zygmunt2, Patricio Luburich Hernaiz7, Roger Llatjós Sanuy8, Virginia Leiro-Fernandez9,10, Eva Balcells Vilarnau1,11, Ernest Sala Llinás1,12, Jordi Dorca Sargatal1,2, Rosario Perona Abellón4, Moisés Selman5, Maria Molina-Molina1,2. 1. Centro de Investigación en Red de Enfermedades Respiratorias (CIBERES), Madrid, Spain. 2. Unidad Funcional de Intersticio Pulmonar, Servicio Neumología, Hospital Universitario de Bellvitge, Instituto de Investigación Biomédica de Bellvitge (IDIBELL), Hospitalet de Llobregat, Barcelona, Spain. 3. Advanced Medical Projects, Madrid, Spain. 4. Instituto de Investigaciones Biomédicas, Consejo Superior de Investigaciones Científicas (CSIC)/Universidad Autónoma de Madrid (UAM), Centro de Investigación en Red de Enfermedades Raras (CIBERER), Madrid, Spain. 5. Instituto Nacional de Enfermedades Respiratorias, "Ismael Cosío Villegas", México City, Mexico. 6. Servicio de Neumología, Hospital Vall d'Hebron, Barcelona, Spain. 7. Unidad Funcional de Intersticio Pulmonar. Servicio Radiodiagnóstico, Hospital Universitario de Bellvitge, Instituto de Investigación Biomédica de Bellvitge (IDIBELL), Hospitalet de Llobregat, Barcelona, Spain. 8. Unidad Funcional de Intersticio Pulmonar, Servicio de Anatomía Patológica, Hospital Universitario de Bellvitge, Instituto de Investigación Biomédica de Bellvitge (IDIBELL), Hospitalet de Llobregat, Barcelona, Spain. 9. Servicio de Neumología, Complexo Hospitalario Universitario de Vigo (CHUVI), Vigo, Spain. 10. Grupo de Investigación en Respiratorio, Instituto de Investigación Biomédica de Vigo, Vigo, Spain. 11. Servicio de Neumología, Hospital del Mar, Instituto Hospital del Mar de Investigaciones Médicas (IMIM), Universidad Pompeu Fabra (UPF), Barcelona, Spain. 12. Servicio de Neumología, Hospital Son Espases, Instituto de Investigación Sanitaria Islas Baleares (IdISBa), Palma de Mallorca, Spain.
Abstract
BACKGROUND AND OBJECTIVE: The abnormal shortening of telomeres is a mechanism linking ageing to idiopathic pulmonary fibrosis (IPF) that could be useful in the clinical setting. The objective of this study was to identify the IPF patients with higher risk for telomere shortening and to investigate the outcome implications. METHODS: Consecutive Spanish patients were included at diagnosis and followed up for 3 years. DNA blood samples from a Mexican cohort were used to validate the results found in Spanish sporadic IPF. Prior to treatment, telomere length was measured through quantitative polymerase chain reaction (qPCR) and Southern blot. Outcome was assessed according to mortality or need for lung transplantation. A multivariate regression logistic model was used for statistical analysis. RESULTS: Family aggregation, age of <60 years and the presence of non-specific immunological or haematological abnormalities were associated with a higher probability of telomere shortening. Overall, 66.6% of patients younger than 60 years with telomere shortening died or required lung transplantation, independent of functional impairment at diagnosis. By contrast, in patients older than 60 years with telomere shortening, the negative impact of telomere shortening in outcome was not significant. CONCLUSION: Our data indicate that young sporadic IPF patients (<60 years) with some non-specific immunological or haematological abnormalities had higher risk of telomere shortening, and furthermore, they presented a poorer prognosis.
BACKGROUND AND OBJECTIVE: The abnormal shortening of telomeres is a mechanism linking ageing to idiopathic pulmonary fibrosis (IPF) that could be useful in the clinical setting. The objective of this study was to identify the IPF patients with higher risk for telomere shortening and to investigate the outcome implications. METHODS: Consecutive Spanish patients were included at diagnosis and followed up for 3 years. DNA blood samples from a Mexican cohort were used to validate the results found in Spanish sporadic IPF. Prior to treatment, telomere length was measured through quantitative polymerase chain reaction (qPCR) and Southern blot. Outcome was assessed according to mortality or need for lung transplantation. A multivariate regression logistic model was used for statistical analysis. RESULTS: Family aggregation, age of <60 years and the presence of non-specific immunological or haematological abnormalities were associated with a higher probability of telomere shortening. Overall, 66.6% of patients younger than 60 years with telomere shortening died or required lung transplantation, independent of functional impairment at diagnosis. By contrast, in patients older than 60 years with telomere shortening, the negative impact of telomere shortening in outcome was not significant. CONCLUSION: Our data indicate that young sporadic IPF patients (<60 years) with some non-specific immunological or haematological abnormalities had higher risk of telomere shortening, and furthermore, they presented a poorer prognosis.
Authors: Ana Merino; Martin J Hoogduijn; Maria Molina-Molina; Elena G Arias-Salgado; Sander S Korevaar; Carla C Baan; Ana Montes-Worboys Journal: PLoS One Date: 2021-03-17 Impact factor: 3.240
Authors: Alicia Usategui; Cristina Municio; Elena G Arias-Salgado; María Martín; Beatriz Fernández-Varas; Manuel J Del Rey; Patricia Carreira; Antonio González; Gabriel Criado; Rosario Perona; José L Pablos Journal: Immun Ageing Date: 2022-01-27 Impact factor: 6.400
Authors: Josep Mercader-Barceló; Joan Truyols-Vives; Carlos Río; Nora López-Safont; Ernest Sala-Llinàs; Alice Chaplin Journal: Int J Mol Sci Date: 2020-08-22 Impact factor: 5.923