Abstract
Interstitial iung processes are a heterogeneous group of diseases with different etiopathogeneses. Progressive pulmonary fibrosis includes diverse nosological units, the common feature of which is a progressive phenotype.
The progressive pulmonary (PPF) fibrosis phenotype is associated with poor prognosis, higher risk of acute exacerbations, and increased mortality. Idiopathic pulmonary fibrosis (IPF) is a typical prototype of PPF with a poor prognosis and high mortality.
Among the treatment options available to patients with IPF and PPF is antifibrotic therapy, which slows the progression of the disease. However, despite all available treatment options, high mortality rate remains.
A comprehensive approach to patients is necessary, keeping in mind timely referrals centers for Interstitial lung processes, precise diagnostics, and consultations within multidisciplinary teams.