Elsevier

Respiratory Medicine

Volume 108, Issue 6, June 2014, Pages 819-829
Respiratory Medicine

Review
Idiopathic pulmonary fibrosis: Early detection and referral

https://doi.org/10.1016/j.rmed.2014.03.008Get rights and content
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Summary

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

IPF
Pulmonary fibrosis
ILD
Lung transplant

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