Ty -jour t1-吡非替酮和nintedanib在特发性肺纤维化患者中的安全性和功效-2003198 au- Justet，AurélienAu -Klay，Dymph au -Porcher，RaphaëlAu -Cottin，Vincent au -Ahmad -Ahmad，Kais au -Molina Molina -Molina Molina，Maria au -Nunes，Nurio au -hilario au -Reynaud -gaubert，Martinaud -gaubert，Martine au -naccache，naccache，naccache，jean，Jean，Jean，Jean，Jean，Marc AU - Manali, Effrosyni AU - Froidure, Antoine AU - Jouneau, Stéphane AU - Wemeau, Lidwine AU - Andrejak, Claire AU - Gondouin, Anne AU - Hirschi, Sandrine AU - Blanchard, Elodie AU - Bondue, Benjamin AU - Bonniaud,Philippe au -Tromeur，Cécileau -Prévot，Grégoireau -Marchand -Adam，Sylvain au -Funke -Chambour，Manuela au -Gamez- Gamez，Anne Sophie au -ba -ba，ibrahima au -Papiris，Spyridon au- spyridon au- grutters，Jan au -crestestani，crestestani，Crestesani，Crestesani，Bruno au -van Moorsel，Coline au -Kannengiesser，Caroline au -Borie，RaphaëlA2-，Y1- 2021/02/01 UR-http：//www.qdcxjkg.com/content/57/2/2/2003198.abstract N2-习惯性肺纤维化（IPF）是一种慢性，进步和致命的间质肺疾病（ILD）。在过去的十年中，IPF的家族发生导致了遗传易感性特征的鉴定[1]。端粒相关基因（TRG）的种系致病性变异，例如TERT，TERC TINF2，DKC1，RTEL1，PARN，NAF1，ZCCHC8，NHP2和NOP10，在20-30％的家族性肺纤维化（FPF）患者中，有20-30％的患者中有20-30％1–5％的零星IPF [2-4]。与IPF患者相比，TRG突变的载体显着年轻，并且表现出强迫生命力的加速下降（FVC）[5-7]。 Two drugs, pirfenidone and nintedanib, have been shown to reduce the decline of FVC in IPF patients [8, 9]. So far, two studies have reported on the safety and effectiveness of pirfenidone in patients with a TRG mutation [6, 7] whereas no study has investigated nintedanib in this specific population. Thus, the aim of this retrospective study was to assess safety and efficacy of nintedanib and pirfenidone in IPF patients with a TRG mutation.This study suggests that pirfenidone and nintedanib can be used safely in IPF patients with a telomerase related gene mutation and that both drugs reduce FVC decline. These results should be confirmed in a larger prospective study. https://bit.ly/3k7b4ZxWe thank Camille Taille, Clairelyne Dupin (Bichat Hospital, Paris) and Julie Traclet (Louis Pradel Hospital, Lyon) for their efficient collaboration and for their help in collecting the data. V. Cottin is a member of ERN-LUNG. ER -