Summary: Idiopathic pulmonary fibrosis (IPF) is an advancing and fatal lung disease with increasing incidence and prevalence. The average life expectancy of untreated patients with IPF is only about three years.
After many drugs failed in clinical trials, improvements in understanding the pathogenesis of IPF led to the approval of two drugs that slow down the progression of the disease. Nintedanib and pirfenidone were approved by the FDA for the treatment of IPF in 2014 based on positive phase 3 trials, and both of these antifibrotic drugs are conditionally recommended in the 2015 ATS/ERS/JRS/ALAT Clinical Practice Guideline. However, the prognosis for patients with IPF remains poor. The search continues for drugs that inhibit the pathogenic pathways active in IPF to reduce or even stop the progression of the disease.
The paper presents a summary of the recommendations for IPF treatment, a short characterization of the new drugs used in the treatment of the disease, non-pharmacological methods of treatment and new directions for clinical trials.