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

Idiopathic pulmonary fibrosis: early detection and referral.

Author information

1
The University of Chicago, Section of Pulmonary and Critical Care Medicine, 5841 S. Maryland Ave, MC 6076, Chicago, IL 60637, USA. Electronic address: justin.oldham@uchospitals.edu.
2
The University of Chicago, Section of Pulmonary and Critical Care Medicine, 5841 S. Maryland Ave, MC 6076, Chicago, IL 60637, USA.

Abstract

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

PMID:
24746629
PMCID:
PMC4785031
DOI:
10.1016/j.rmed.2014.03.008
[Indexed for MEDLINE]
Free PMC Article

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