IPF is currently an underdiagnosed disease due to difficulties in the interpretation of HRCT findings and also due to the initially silent clinic. It is a progressive, irreversible, fatal, fibrosing lung disease of unknown cause, that carries with a survival rate lower than some cancer types.

Current hypothesis to the emergence of IPF:

• An endogenous/environmental stimulus disrupts the balance of alveolar epithelial cells, resulting in cellular activation and anomalous epithelial repair
•  The exposure of a susceptible person to a specific agent may lead to alveolar damage, later triggering the fibrosis process.

Eventual external predisposing factors

• Tobacco smoke
• Environmental pollutants
• Viral Infections 
• Gastroesophageal reflux
• Chronic micro aspirations 

Eventual intrinsic predisposing factors

• E2 prostaglandin deficit
• Genetic factors
• Caveolin-1 deficit

The use of HRCT as a diagnostic tool for IPF has given the radiologist an additional responsibility for recognizing this entity. Although there is no cure, the use of antifibrotic drugs is already approved to slow the decline in lung function. While respiratory function tests are validated tools to monitor lung function in these patients, imaging features expected in the follow-up HRCT after the introduction of these drugs is still not still well studied.