Chronic interstitial lung disease (ILD) is considered a heterogeneous group of pulmonary diseases usually classified on the basis of clinical, radiological and histological aspects, characterized by a variable degree of inflammation and fibrosis. The most diagnosed ILD are idiopathic pulmonary fibrosis (IPF), characterized by the presence of serious changes in the normal architecture of the pulmonary parenchyma, resulting in a progressive and often rapid respiratory functional decline. Although considered a rare disease, the IPF has seen a considerable increase in prevalence and mortality, over the last 10 years. Moreover, it has a worse outcome than most malignant tumours resulting in an average. IPF should be considered in all adult patients with unexplained chronic exertional dyspnoea, and commonly presents with cough, bibasilar inspiratory crackles and finger clubbing. The diagnosis of IPF requires exclusion of other known causes of interstitial lung disease (ILD) and the presence of a usual interstitial pneumoniae (UIP) pattern on high-resolution computed tomography (HRCT) in patients not subjected to surgical lung biopsy. The functional respiratory pattern is characterized by restrictive type of ventilatory compromise, reduction of the DLCO diffusion capacity, hypoxaemia during exercise and, then, at rest too. Despite the efforts made in scientific research and the therapeutic progress achieved, there are currently no drugs capable of achieving the remission and healing of this pathology. To date, there are only two drugs, pirfenidone and nintedanib, although not usable by all patients, able to drastically reduce symptoms and exacerbations, significantly improve the quality of life and, above all, stop the decline in respiratory function.
