Myriam Aburto, Ana Romero, Guillermo Suarez-Cuartin, Maria Molina-Molina, Vanesa Vicens-Zygmunt, Eva Balcells, Orlando Acosta, Claudia Valenzuela, José Antonio Rodríguez-Portal, Guadalupe Bermudo, Teresa González-Budiño, Diego Castillo, Amalia Moreno, Esteban Cano, Patricio Luburich, Jaume Sauleda, Ana Villar, Belén Núñez, Estrella Fernández-Fabrellas, Jessica Germaine Shull, Diana Badenes-Bonet, Virginia Leiro-Fernández, Lurdes Planas-Cerezales, Tomás Franquet, Rosalía Laporta, Karina Portillo, and Pilar Rivera-Ortega
Introduction Idiopathic 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. Methods Spanish 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. Results 225 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 6 min 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 Conclusions Patients 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.