TY - JOUR T1 - Different Faces of Idiopathic Pulmonary Fibrosis With Preserved Forced Vital Capacity JO - Archivos de Bronconeumología T2 - AU - Bermudo,Guadalupe AU - Suarez-Cuartin,Guillermo AU - Rivera-Ortega,Pilar AU - Rodriguez-Portal,Jose Antonio AU - Sauleda,Jaume AU - Nuñez,Belen AU - Castillo,Diego AU - Aburto,Myriam AU - Portillo,Karina AU - Balcells,Eva AU - Badenes-Bonet,Diana AU - Valenzuela,Claudia AU - Fernandez-Fabrellas,Estrella AU - González-Budiño,Teresa AU - Cano,Esteban AU - Acosta,Orlando AU - Leiro-Fernández,Virginia AU - Romero,Ana AU - Planas-Cerezales,Lurdes AU - Villar,Ana AU - Moreno,Amalia AU - Laporta,Rosalia AU - Vicens-Zygmunt,Vanesa AU - Shull,Jessica AU - Franquet,Tomàs AU - Luburich,Patricio AU - Molina-Molina,Maria SN - 03002896 M3 - 10.1016/j.arbres.2021.03.018 DO - 10.1016/j.arbres.2021.03.018 UR - https://archbronconeumol.org/en-different-faces-idiopathic-pulmonary-fibrosis-articulo-S0300289621001162 AB - IntroductionIdiopathic pulmonary fibrosis (IPF) is progressive and irreversible. Some discrepancies about IPF staging exists, especially in mild phases. Forced vital capacity (FVC) higher than 80% has been considered early or mild IPF even for the design of clinical trials. MethodsSpanish multicentre, observational, retrospective study of IPF patients diagnosed between 2012 and 2016, based on the ATS/ERS criteria, which presented FVC greater or equal 80% at diagnosis. Clinical and demographic characteristics, lung function, radiological pattern, treatment, and follow-up were analyzed. Results225 IPF patients were included, 72.9% were men. The mean age was 69.5 years. The predominant high-resolution computed tomography (HRCT) pattern was consistent usual interstitial pneumonia (UIP) (51.6%). 84.7% of patients presented respiratory symptoms (exertional dyspnea and/or cough) and 33.33% showed oxygen desaturation below 90% in the 6min walking test (6MWT). Anti-fibrotic treatment was initiated at diagnosis in 55.11% of patients. Median FVC was 89.6% (IQR 17) and 58.7% of patients had a decrease of diffusion lung capacity for carbon monoxide (DLCO) below 60% of theoretical value; most of them presented functional progression (61.4%) and higher mortality at 3 years (20.45%). A statistically significant correlation with the 3-years mortality was observed between DLCO <60% and consistent UIP radiological pattern. ConclusionsPatients with preserved FVC but presenting UIP radiological pattern and moderate–severe DLCO decrease at diagnosis associate an increased risk of progression, death or lung transplantation. Therefore, in these cases, preserved FVC would not be representative of early or mild IPF. ER -