Abstract
The course of interstitial lung diseases associated with fibrotic remodelling of lung tissue is not uniform. In some patients with this type of lung disease (regardless of the cause and current treatment), scarring progression occurs over time.
The term progressive fibrosing interstitial lung disease thus defines the biological behaviour of the disease over time, it is not a new nosological unit. Recent studies indicate that taking into account the evolution of the fibrosing interstitial lung disease over time may be as important as the diagnosis.
Patients with this type of disease benefit from antifibrotic treatment the same way as patients with idiopathic pulmonary fibrosis. The current level of knowledge continues to support the need for the most accurate diagnosis, initiation of appropriate treatment, and monitoring of further disease progression over time.