Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial
lung disease, characterised by progressive scarring of the lung and associated with
a high burden of disease and early death. The pathophysiological understanding, clinical
diagnostics and therapy of IPF have significantly evolved in recent years. While the
recent introduction of the two antifibrotic drugs pirfenidone and nintedanib led to
a significant reduction in lung function decline, there is still no cure for IPF;
thus, new therapeutic approaches are needed. Currently, several clinical phase I-III
trials are focusing on novel therapeutic targets. Furthermore, new approaches in nonpharmacological
treatments in palliative care, pulmonary rehabilitation, lung transplantation, management
of comorbidities and acute exacerbations aim to improve symptom control and quality
of life. Here we summarise new therapeutic attempts and potential future approaches
to treat this devastating disease.
