Pirfenidone and Nintedanib in idiopathic pulmonary fibrosis: Real-life experience in an Italian referral centre

Background: Idiopathic pulmonary fibrosis has a median survival time after diagnosis of 2-5 years. The main goal of treating IPF is to stabilize or reduce the rate of disease progression. Nintedanib and Pirfenidone have been a breakthrough in the management of IPF. Here we evaluated the effectiveness of Pirfenidone and Nintedanib in a population of IPF patients diagnosed in the last 12 months at Florence ILD Referral Centre. Methods: In the last 12 months, 82 IPF patients (66 male, mean age 78.3 +/- 23.8 years) were diagnosed and started antifibrotic therapy with Pirfenidone or Nintedanib. Their clinical and functional details were analyzed retrospectively at time 0 and after 6 and 12 months of therapy. Results: The median age of the patients treated with Nintedanib was higher than that of the Pirfenidone group (p < 0.0001). The most common symptoms at disease onset were exertional dyspnoea and dry cough with no differences between the two groups (p < 0.05). All IPF patients manifested bibasal crackles at the time of diagnosis. No significant differences in FVC, FEV1, TLC and DLCO were found at time 0 or after 6 months between patients treated with Pirfenidone and Nintedanib (p > 0.05). After 1 year, lung function test parameters of patients treated with Pirfenidone had remained stable from baseline. Discussion: This study emphasizes that both antifibrotic drugs appeared to be a good therapeutic choice in terms of functional stabilization, also in older patients. Published by Elsevier Espana, S.L.U. on behalf of Sociedade Portuguesa de Pneumologia.