Development and progress in interstitial lung diseases

In the field of interstitial lung disease (ILD) there have been very significant advances in the diagnostics and treatment in recent years. This includes an improved understanding of interstitial lung abnormalities (ILA). An important focus is the establishment of prognostic and predictive biomarkers. Regarding imaging modalities, the use of magnetic resonance imaging (MRI) as well as fibroblast-activation protein inhibitor/positron emission tomography-computed tomography (FAPI/PET-CT) in ILD is currently under study and appears to be promising. Confocal laser endoscopy could serve as a complementary navigation tool, increasing the diagnostic yield of transbronchial cryobiopsy. Progress has also been made with respect to nonpharmacologic treatment and a prognostic significance of pulmonary rehabilitation has been described for the first time. Major developments have been made in the area of drug treatment. For example, antifibrotic treatment has been shown to be effective even in progressively fibrosing phenotypes. Nintedanib is also effective in systemic scleroderma-associated ILD (SSc-ILD); in this indication, the FDA has also approved tocilizumab as the first biologic based on data from the FocuSSced trial. Following the recent termination of a phase III program on the autotaxin inhibitor GLPG1690 due to ineffectiveness, the promising new therapeutics for the treatment of idiopathic pulmonary fibrosis, pentraxin and pamrevlumab, are being tested in phase III trials.