Pathobiology of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis is an alveolar epithelium disease. It results from severe and widespread dysfunctions of cells of the alveolar epithelium, which are favored by exposure to inhaled toxicants such as tobacco, by aging, and in some cases by genetic susceptibility. Inflammation would play a limited role, especially in the early stages of the disease, but could contribute to the maintenance of the vicious circle of fibrosis. The alveolar microbiome alterations have been described but their etiological role is uncertain. Two drugs (pirfenidone, nintedanib) demonstrated their ability to slow the progression of pulmonary fibrosis. The pathways inhibited by these drugs give indications as to the profibrosing pathways in the lung