RT Journal Article
SR Electronic
T1 Increase in post-transplant survival and quality of life in pulmonary fibrosis with and without telomere dysfunction
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 5255
DO 10.1183/13993003.congress-2020.5255
VO 56
IS suppl 64
A1 Lurdes Planas Cerezales
A1 Elena G Arias-Salgado
A1 Cristina Berastegui Garcia
A1 Ana Montes Worboys
A1 Rafaela Gonzalez Montelongo
A1 Jose M Lorenzo Salazar
A1 Vanesa Vicens-Zygmunt
A1 Marta Garcia Moyano
A1 Jordi Dorca
A1 Carlos Flores
A1 Rosario Perona
A1 Antonio Román
A1 Maria Molina-Molina
YR 2020
UL http://erj.ersjournals.com/content/56/suppl_64/5255.abstract
AB Telomere dysfunction has been related to post-transplant morbi-mortality.Study aim´s: post-transplant survival and morbidity evaluation in fibrotic ILD with and without telomeropathyMethods: Prospective observational study of fibrotic ILD cases from Bellvitge Hospital that underwent lung transplant(LT) in V.Hebrón Hospital. Telomere length was measured from lymphocytes DNA, through quantitative PCR. Whole-exome sequencing for telomere maintenance genes was performed in case of telomere shortening(TS)Results: 20 patients(IPF 40%, FPF 40%, hypersensitivity pneumonitis 5%, CPFE 5%, descamative interstitial pneumonia 5%, CTD-ILD 5%). Twelve(60%) patients had TS and mutations in RTEL1, TERT, DKC1,PARN. A similar pre-LT functional status was reported regardless of TS. LT morbidity: patients with TS had a greater need for extracorporeal circulation, longer ICU admission period and higher number of hospital admissions(p=0.033) with 2.9 times higher risk for readmission[(CI 1.11-7.59)(p=0.029)]; being the only ones with hematological complications during the first month. At long term, no differences in morbidity were found regardless of TS.Survival: 17/20(85%) patients,[10/12(83.3%) with TS and 7/8(87.5%) without]. Quality of life: overall mean FVC post-LT increase of 0.55 liters(p=0.0003)[CI95%: 0.21;0.89]. Oxygen withdrawal in 16/17(94.1%) cases with independence for daily life activities[15/17(88.2%)] and active life[11/17(64.7%)] regardless of TS(p=1.000)Conclusions: Post-LT morbidity differs accoding to elapsed time from LT and genetic susceptibility.However, a positive impact in post-LT survival and quality of life was shown for fibrotic ILD regardless of telomeropathyFootnotesCite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 5255.This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).