Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis

Rachel Strykowski; Ayodeji Adegunsoye

doi:10.1016/j.iac.2023.01.010

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.

Authors

Rachel Strykowski¹, Ayodeji Adegunsoye²

Affiliations

¹ Section of Pulmonary and Critical Care, Department at University of Chicago, 5841 South Maryland Avenue, MC 6076, Chicago, IL 60637, USA.
² Interstitial Lung Disease Program, Section of Pulmonary/Critical Care, University of Chicago, Chicago, IL, USA. Electronic address: deji@uchicago.edu.

PMID: 37055085
DOI: 10.1016/j.iac.2023.01.010

Abstract

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

Grants and funding

K23 HL146942/HL/NHLBI NIH HHS/United States