Ruolo del Pirfenidone nel trattamento della Fibrosi Polmonare Idiopatica: esperienza del centro di riferimento regionale dell'AOUS.

The objective of the thesis was to describe the trend of patients suffering from Idiopathic Pulmonary Fibrosis being treated with Pirfenidone at the regional reference center for rare lung diseases of the AOUS. All patients with IPF treated with Pirfenidone were retrospectively enrolled at the Siena Regional Reference Center from 2011 to 2019. Serial examinations, performed every 12 months, of all clinical and functional parameters and, if available, the parameters of the pulmonary function test 1 year before the start of antifibrotic therapy. A systematic review was performed on survival, causes of mortality, and treatment-related side effects of patients with IPF. A decrease greater than or equal to 10% of the FVC was considered as functional loss. A total of 139 patients were included in the study. On average, lung function at baseline showed a restrictive deficit in lung volumes with a moderate reduction in Dlco, the latter available for 105 patients who were able to perform the test and not treated with oxygen therapy. 13 patients had advanced disease stage (FVC <50% and / or DCLO <35%). The retrospective study in a real setting shows that the drug reduces progression and improves life expectancy with acceptable tolerability even in subjects with severe functional impairment. [ABSTRACT FROM AUTHOR]