The therapy of idiopathic pulmonary fibrosis: what is next?

Eur Respir Rev. 2019 Sep 4;28(153):190021. doi: 10.1183/16000617.0021-2019. Print 2019 Sep 30.


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.

Publication types

  • Review

MeSH terms

  • Animals
  • Comorbidity
  • Disease Progression
  • Humans
  • Idiopathic Pulmonary Fibrosis / mortality
  • Idiopathic Pulmonary Fibrosis / pathology
  • Idiopathic Pulmonary Fibrosis / physiopathology
  • Idiopathic Pulmonary Fibrosis / therapy*
  • Indoles / therapeutic use
  • Lung / drug effects*
  • Lung / pathology
  • Lung / physiopathology
  • Lung / surgery*
  • Lung Transplantation* / adverse effects
  • Lung Transplantation* / mortality
  • Molecular Targeted Therapy
  • Palliative Care*
  • Pyridones / therapeutic use
  • Respiratory System Agents / adverse effects
  • Respiratory System Agents / therapeutic use*
  • Respiratory Therapy* / adverse effects
  • Respiratory Therapy* / mortality
  • Risk Factors
  • Treatment Outcome


  • Indoles
  • Pyridones
  • Respiratory System Agents
  • pirfenidone
  • nintedanib