TY - JOUR T1 - An Open-label Study With Pirfenidone on Chronic Hypersensitivity Pneumonitis JO - Archivos de Bronconeumología T2 - AU - Mateos-Toledo,Heidegger AU - Mejía-Ávila,Mayra AU - Rodríguez-Barreto,Óscar AU - Mejía-Hurtado,José Guillermo AU - Rojas-Serrano,Jorge AU - Estrada,Andrea AU - Castillo-Pedroza,Jonathan AU - Castillo-Castillo,Kelly AU - Gaxiola,Miguel AU - Buendía-Roldan,Ivette AU - Selman,Moises SN - 03002896 M3 - 10.1016/j.arbres.2019.08.019 DO - 10.1016/j.arbres.2019.08.019 UR - https://www.archbronconeumol.org/en-an-open-label-study-with-pirfenidone-articulo-S0300289619303813 AB - BackgroundChronic hypersensitivity pneumonitis (cHP) represents a severe lung disease often evolving to fibrosis with the subsequent destruction of the lung parenchyma. There are no approved therapies with confirmed efficacy to deal with this disease. MethodsWe performed an open-label, proof of concept study, to evaluate the efficacy and safety of pirfenidone added to immunosuppressive drugs on the treatment of cHP. We included 22 patients assigned to two groups: Group 1, nine patients that received prednisone plus azathioprine and Group 2, thirteen patients, received prednisone plus azathioprine and pirfenidone (ClinicalTrials.gov identifier NCT02496182). There were no significant imbalances in clinically relevant baseline characteristics between two study groups. ResultsAfter 1 year of treatment, inclusion of pirfenidone was not associated with improved forced vital capacity (primary end-point). A not significant tendency to show higher improvement of diffusion capacity of the lung for carbon monoxide (DLCO) was observed in the group receiving pirfenidone (p=0.06). Likewise, a significant improvement in the total score on the SGRQ was found in the group 2 (p=0.02) without differences in other two questionnaires related to quality of life (ATAQ-IPF and EQ-5D-3L). HRCT showed a decrease of the ground glass attenuation without changes in the fibrotic lesions and without differences between both groups. ConclusionsThese findings suggest that the addition of pirfenidone to the anti-inflammatory treatment in patients with chronic HP may improve the outcome with acceptable safety profile. However, prospective randomized double-blind, placebo-controlled trials in largest cohorts are needed to validate its efficacy. ER -