Idiopathic pulmonary fibrosis: early detection and referral

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.

Figure 1

Chest radiograph of a patient with IPF. Findings include asymmetric peripheral and basilar reticular opacity and lower lobe volume loss.

Figure 2

HRCT of the chest demonstrating classic UIP pattern. Findings include peripheral and basilar predominant reticulation with traction bronchiectasis and honeycombing.

Figure 3

Histopathologic UIP pattern. Low-power photomicrographs showing findings characteristic of UIP, including subpleural interstitial fibrosis (thin arrow) with temporal heterogeneity (normal lung at the lower right of each slide) and fibroblastic foci (thick arrow). (Courtesy of Aliya Husain, M.D., Department of Pathology, The University of Chicago).

Figure 4

Survival from the time of evaluation at a tertiary care center adjusted for age and FVC across quartiles of delay. Entry time into the cohort began at study enrollment. Reprinted from Ref. [72] with permission of the American Thoracic Society. Copyright © 2014 American Thoracic Society.