Novel management strategies for idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) treatment was revolutionized by the advent of two novel antifibrotics, nintedanib and pirfenidone. However, neither is a panacea and other agents are still sorely needed. This review presents on-going efforts to improve outcomes for patients with IPF by targeting novel pharmacologic pathways, improving comorbidity management, and aiming for improved quality of life. Areas covered: We provide an overview of on-going basic and clinical science efforts focused on development of additional drug therapies for patients with IPF. Known and emerging pathogenic pathways such as the microbiome and pulmonary vasculature hold promise as targets for therapy. While the focus remains on pharmacologic intervention, the impact of comorbidities and their management may also impact patient outcomes significantly. Supportive care with pulmonary rehabilitation, oxygen therapy, and palliative care remain integral at various stages of the disease course. Finally, lung transplant is the only lifesaving intervention for patients with end-stage fibrosis. Expert commentary: Future investigation should aim to prevent the initial insult or injury that engages the multiple pathways associated with the development and progression of IPF. Targeted therapies represent just one management aspect with a multidisciplinary approach necessary for the global holistic care of these complex patients.