Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis

Immunol Allergy Clin North Am. 2023 May;43(2):209-228. doi: 10.1016/j.iac.2023.01.010. Epub 2023 Mar 3.


Idiopathic pulmonary fibrosis (IPF), a common interstitial lung disease (ILD), is a chronic, progressive fibrosing interstitial pneumonia, with an unknown cause. IPF has been linked to several genetic and environmental risk factors. Disease progression is common and associated with worse outcomes. Management often encompasses pharmacotherapy, supportive interventions, addressing comorbidities when present, and treating hypoxia with ambulatory O2. Consideration for antifibrotic therapy and lung transplantation evaluation should occur early. Patients with ILD other than IPF, and who have radiological evidence of pulmonary fibrosis, may have progressive pulmonary fibrosis.

Keywords: Diffuse parenchymal lung disease; Idiopathic pulmonary fibrosis; Interstitial lung disease; Progressive pulmonary fibrosis.

Publication types

  • Review
  • Research Support, N.I.H., Extramural

MeSH terms

  • Disease Progression
  • Fibrosis
  • Humans
  • Idiopathic Pulmonary Fibrosis* / diagnosis
  • Idiopathic Pulmonary Fibrosis* / etiology
  • Idiopathic Pulmonary Fibrosis* / therapy
  • Lung Diseases, Interstitial* / diagnosis
  • Lung Diseases, Interstitial* / etiology
  • Lung Diseases, Interstitial* / therapy
  • Tomography, X-Ray Computed