2020
Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation
Justet A, Klay D, Porcher R, Cottin V, Ahmad K, Molina Molina M, Nunes H, Reynaud-Gaubert M, Naccache J, Manali E, Froidure A, Jouneau S, Wemeau L, Andrejak C, Gondouin A, Hirschi S, Blanchard E, Bondue B, Bonniaud P, Tromeur C, Prévot G, Marchand-Adam S, Funke-Chambour M, Gamez A, Ba I, Papiris S, Grutters J, Crestani B, van Moorsel C, Kannengiesser C, Borie R. Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation. European Respiratory Journal 2020, 57: 2003198. PMID: 33214205, DOI: 10.1183/13993003.03198-2020.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosis patientsIdiopathic pulmonary fibrosisTelomere-related genesDecline of forced vital capacityFamilial pulmonary fibrosisInterstitial lung diseaseForced Vital CapacityPulmonary fibrosisGene mutationsSporadic idiopathic pulmonary fibrosisEfficacy of nintedanibEfficacy of pirfenidoneEffect of pirfenidoneGermline pathogenic variationOccurrence of idiopathic pulmonary fibrosisFVC declineRetrospective studyProspective studyAssess safetyNintedanibLung diseasePirfenidoneFamilial occurrencePatientsGenetic susceptibility traits
2018
Safety and efficacy of pirfenidone in patients carrying telomerase complex mutation
Justet A, Thabut G, Manali E, Molina M, Kannengiesser C, Cadranel J, Cottin V, Gondouin A, Nunes H, Magois E, Tromeur C, Prevot G, Papiris S, Marchand-Adam S, Gamez A, Reynaud-Gaubert M, Wemeau L, Crestani B, Borie R. Safety and efficacy of pirfenidone in patients carrying telomerase complex mutation. European Respiratory Journal 2018, 51: 1701875. PMID: 29449422, DOI: 10.1183/13993003.01875-2017.Peer-Reviewed Original ResearchConceptsFamilial pulmonary fibrosisIdiopathic pulmonary fibrosisEfficacy of pirfenidoneMutation carriersForced Vital CapacityRetrospective studyPulmonary fibrosisTreatment of idiopathic pulmonary fibrosisReduced FVC declineProgression-free survivalMulticentre retrospective studyEffect of pirfenidoneLung function declineTERC mutationsFVC declineMultidisciplinary diagnosisAssociated with bloodPatient carriersFrequent mutationsPirfenidoneSynthetic androgenDiffusing capacitySkin disordersPatientsVital capacity
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