A real life multicenter national study on the use of nintedanib in moderate to severe IPF patients

Background: pirfenidone and nintedanib are two drugs approved for the treatment of mild to moderate of IPF, however data about the efficacy of these drugs on more severe patients (pts) are scarce. Method: We conducted a retrospective multicenter national real life study in Italy to evaluate the efficacy of nintedanib for the treatment of pts with severe IPF. We considered 41 pts consecutive with at least 6 months pre and post nintedanib therapy and with FVC ≤ 50% and/or DlCO ≤ 35% and compared pulmonary functional trends pre and post therapy. Survival was also calculated starting from month 6 of therapy with nintedanib. Results: At time zero (start nintedanib therapy), pts had a FVC % of 60.63 (SD 14.57) and a DlCO of 26.54 (SD 5.70). Therapy with nintedanib slowed the fall of DlCO (p= 0.004) e and TlCO (p=0.03) compared to pre treatment period but no other significant changes in pulmonary function data, including FVC, was registered. One year survival starting from month 6 of therapy with nintedanib was 79%. Conclusions: In this national multicenter experience, nintedanib reduced in patients with severe IPF the rate of decline of DlCO and of TlCO without significant impact on FVC and on other PFT data. Our results suggest that the drug might also be effective in patients with more advanced disease.