Idiopathic pulmonary fibrosis: spread and mortality

Idiopathic pulmonary fibrosis is a particular form of pulmonary fibrosis, due to unknown causes.

Pulmonary fibrosis is a severe and incurable respiratory disease, which occurs when the normal lung tissue is replaced by scar tissue (or fibrotic). The scar tissue "crushes" the pulmonary alveoli, preventing the latter from oxygenating the blood.

According to recent estimates, each year idiopathic pulmonary fibrosis would affect between 14 and 43 individuals per 100, 000, predominantly male.

Furthermore, it seems that the diagnosis generally occurs very late (about one or two years after the first symptoms) and that death occurs, in 50% of cases, two / three years after diagnosis.

Although the precise triggers are not known, it is suspected that they may have a particular influence: cigarette smoke, inhalation of metal or carbon dust, infections with Epstain-Barr virus or hepatitis C virus, some forms of gastroesophageal reflux and, finally, a certain genetic predisposition.