PT - JOURNAL ARTICLE AU - Ana Belén Llanos-González AU - Sara García Gil AU - Juan Alfonso Bonilla Arjona AU - Sonia García Hernández AU - Verónica S. Hernández García AU - Paula Pérez De Armas AU - Nelson Mesa León AU - Orlando Acosta Fernández TI - Experience with antifibrotics treatment in progressive interstitial lung diseases non-IPF AID - 10.1183/13993003.congress-2021.PA2375 DP - 2021 Sep 05 TA - European Respiratory Journal PG - PA2375 VI - 58 IP - suppl 65 4099 - http://erj.ersjournals.com/content/58/suppl_65/PA2375.short 4100 - http://erj.ersjournals.com/content/58/suppl_65/PA2375.full SO - Eur Respir J2021 Sep 05; 58 AB - Introduction and aim: Progressive fibrosing interstitial lung diseases (ILD) other than idiopathic pulmonary fibrosis (IPF) can have a similar course, and clinical trials have shown the efficacy and safety of antifibrotic treatments (pirfenidone/nintedanib) in progressive ILD non-IPF patients. In this study, we aimed to analyse the evolution and characteristics of these patients in clinical practice.Methods: Prospective study of 27 patients with progressive ILD non-IPF treated with off-label antifibrotics drugs, from January 2015 to January 2021. Epidemiological, clinical and therapeutic characteristics were evaluated.Results: Mean age was 71±8 years, 63% were male. They were:33% unclassifiable pulmonary fibrosis (UPF),26% chronic hypersensitivity pneumonitis (CHP),26% combination of pulmonary fibrosis and emphysema (CPFE),11% pulmonary fibrosis associated with systemic autoimmune disease (CTD-ILD) and 4% smoking-related interstitial fibrosis (SRIF).67% had non usual interstitial pneumonia HRCT pattern. Lung biopsy was performed in 7 cases. Fifteen (56%) patients received pirfenidone and twelve (44%) nintedanib. Mean FVC was 67%±21 and DLCO 43%±16. Functional stability after a year of antifibrotic therapy was observed in all groups, except in 4 patients who had a FVC decrease of 5-10%. Digestive mild-moderate adverse effects where in 81%. Thus, in 9 of the cases a dose decrease of antifibrotics was maintained and in 3 it was suspended.22% have died.Conclusion: After one year antifibrotic therapy, patients with progressive ILD non-IPF achieve functional stability. Further clinical trials with these drugs are warranted to evaluate their efficacy as combined therapeutic approach in treating these diseases.FootnotesCite this article as: European Respiratory Journal 2021; 58: Suppl. 65, PA2375.This abstract was presented at the 2021 ERS International Congress, in session “Prediction of exacerbations in patients with COPD”.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).