What is the treatment for pulmonary fibrosis

Pulmonary fibrosis: therapy

The active ingredient pirfenidone received EU approval for the treatment of idiopathic pulmonary fibrosis in March 2011. It is a so-called "small molecule" which, among other things, inhibits the transforming growth factor beta1.

Studies had shown that the drug slowed the deterioration of lung function in people with IPF. It was therefore considered to be the bearer of hope in the therapy of idiopathic pulmonary fibrosis.

Clinical studies on those affected have shown that pirfenidone slows down the decline in lung function (vital capacity). This can be seen as a measure of the scarring of the lungs. There are also positive effects

  • physical performance measured as 6-minute walk, and
  • on overall survival at one year.

However, a positive influence on breathing difficulties and quality of life could not be proven.

Side effects of pirfenidone included gastrointestinal problems and skin rashes and sensitivity to sunlight. So that those affected who take pirfenidone have to apply a sun protection factor 50 even in normal daylight.

After weighing up the effects shown and the side effects, the authors of the currently valid medical guidelines for the treatment of IPF came to the recommendation that IPF patients should be treated with pirfenidone. However, those affected must be informed in detail about the effects and possible side effects. Based on these studies, pirfenidone has been approved in the EU since 2011 for the treatment of patients with mild to moderate IPF.