Idiopathic pulmonary fibrosis: early detection and referral

Respir Med. 2014 Jun;108(6):819-29. doi: 10.1016/j.rmed.2014.03.008. Epub 2014 Apr 4.


Idiopathic pulmonary fibrosis (IPF), a devastating progressive interstitial lung disease (ILD) with no known cause or cure, is the most common and deadly of the idiopathic interstitial pneumonias. With a median survival of 3-5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. Vigilance among clinicians in recognizing IPF early in the disease course remains critical to properly caring for these patients, as this provides the widest range of management options. When IPF is suspected, a multidisciplinary evaluation (MDE) by a clinician, radiologist and pathologist with ILD expertise should occur, as this improves diagnostic agreement in both community and academic settings. When community MDE is not possible, or diagnostic doubt exists, referral to an ILD center should be considered. ILD center referral may also provide access specialized care, including clinical trials and lung transplantation, and should be considered for any patient with an established diagnosis of IPF.

Keywords: ILD; IPF; Lung transplant; Pulmonary fibrosis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Aged
  • Biopsy / methods
  • Bronchoscopy / methods
  • Clinical Laboratory Techniques / methods
  • Early Diagnosis
  • Humans
  • Idiopathic Pulmonary Fibrosis / complications
  • Idiopathic Pulmonary Fibrosis / diagnosis*
  • Idiopathic Pulmonary Fibrosis / therapy
  • Lung / pathology
  • Lung Transplantation / methods
  • Middle Aged
  • Oxygen / therapeutic use
  • Patient Care Team
  • Physical Examination / methods
  • Referral and Consultation*
  • Respiratory Function Tests
  • Respiratory System Agents / therapeutic use
  • Tomography, X-Ray Computed / methods


  • Respiratory System Agents
  • Oxygen