Key messages
- •
Progressive fibrosing interstitial lung disease is a phenotype in which patients continue to progress despite conventional treatment directed at the underlying diagnosis; the definition of progressive fibrosis should integrate combinations of deteriorating lung function, CT appearances, and patient symptoms
- •
It is important to make an accurate diagnosis to ensure that patients are treated optimally before progressive fibrosis can be ascertained
- •
The multidisciplinary team should have a central role in establishing an underlying diagnosis and then assessing longitudinal disease behaviour on conventional therapy
- •
Antifibrotic therapy for non-idiopathic pulmonary fibrosis interstitial lung disease could be considered as a second-line therapy after demonstration of progressive fibrosis despite conventional treatment
- •
In the absence of clinical trial data, the decision to initiate combination treatment with antifibrotic therapy and immunosuppression should be made on a case-by-case basis by a multidisciplinary team, taking into account a number of patient and disease factors
- •
A range of approaches including epidemiological studies, randomised controlled trials, and deep learning algorithms will be needed to address key uncertainties in the identification and management of the progressive fibrotic phenotype