Therapeutic target
Halting progression (progression of the disease).
Therapy recommendations
- Therapy depends on the underlying disease.
- Therapy of idiopathic pulmonary fibrosis (idiopathic pulmonary fibrosis, idiopathic pulmonary fibrosis (IPF)/idiopathic pulmonary fibrosis) is usually with prednisiolone (glucocorticoids); furthermore, immunosuppressants (e.g., azathioprine) are used. IPF guideline 2015: prednisone + azathioprine + N-acetylcysteine; strong recommendation against (PANTHER; IPF guideline 2017: prednisol, azathioprine and acetylcysteine is classified as not suitable).
- Treatment options are available for the first time for mild to moderate idiopathic pulmonary fibrosis:
- Pirfenidone (antifibrotic agent; ASCEND trial; see “Note” below) [IPF guideline 2015: conditional recommendation for this; confirmed in 2017] as well as.
- Nintedanib (multikinase inhibitor; INPULSIS trial) halt disease progression [IPF guideline 2015: conditional recommendation for this] .Nintedanip has been approved in the European Union since January 15, 2015, in adults for the treatment of idiopathic pulmonary fibrosis (IPF).
- In one study, patients took nintedanib or placebo twice daily for the study duration of 52 weeks; lung function (forced expiratory vital capacity (FVC)) was checked regularly during the course. The nintedanib group showed a markedly lower decline in lung function compared with the placebo group, resulting in a significant slowing of disease progression.
- Nintedanib can also in other forms of pulmonary fibrosis, slow the loss of lung function
- In 70-80% of all patients, the above drugs can be used in the long term.
Notice:
- The updated guideline (see below) advises against treating patients with idiopathic pulmonary fibrosis (IPF) with vitamin K antagonists (VKA), imatinib, abrisentan, bosentan, or macitentan.
- Also evaluated negatively are sildenafil and acetylcysteine monotherapy.
- Severe cases of drug-induced liver injury (DILI), including fatal cases, have been reported with the use of pirfenidone.