Idiopathic pulmonary fibrosis: Current and future treatment

Clin Respir J. 2022 Feb;16(2):84-96. doi: 10.1111/crj.13466. Epub 2022 Jan 10.


Objectives: Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease characterized by dry cough, fatigue, and progressive exertional dyspnea. Lung parenchyma and architecture is destroyed, compliance is lost, and gas exchange is compromised in this debilitating condition that leads inexorably to respiratory failure and death within 3-5 years of diagnosis. This review discusses treatment approaches to IPF in current use and those that appear promising for future development.

Data source: The data were obtained from the Randomized Controlled Trials and scientific studies published in English literature. We used search terms related to IPF, antifibrotic treatment, lung transplant, and management.

Results: Etiopathogenesis of IPF is not fully understood, and treatment options are limited. Pathological features of IPF include extracellular matrix remodeling, fibroblast activation and proliferation, immune dysregulation, cell senescence, and presence of aberrant basaloid cells. The mainstay therapies are the oral antifibrotic drugs pirfenidone and nintedanib, which can improve quality of life, attenuate symptoms, and slow disease progression. Unilateral or bilateral lung transplantation is the only treatment for IPF shown to increase life expectancy.

Conclusion: Clearly, there is an unmet need for accelerated research into IPF mechanisms so that progress can be made in therapeutics toward the goals of increasing life expectancy, alleviating symptoms, and improving well-being.

Keywords: idiopathic pulmonary fibrosis; lung transplantation; nintedanib; pentraxin; pirfenidone.

Publication types

  • Review

MeSH terms

  • Fibrosis
  • Humans
  • Idiopathic Pulmonary Fibrosis* / diagnosis
  • Idiopathic Pulmonary Fibrosis* / drug therapy
  • Lung / pathology
  • Lung Transplantation*
  • Quality of Life
  • Randomized Controlled Trials as Topic