Clinical course and management of idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal interstitial lung disease (ILD) with an unpredictable clinical course. Although IPF is rare, healthcare professionals should consider IPF as a potential cause of unexplained chronic dyspnea and/or cough in middle-aged/elderly patients and refer patients to a pulmonologist for evaluation. Making a diagnosis of IPF requires specialist expertise. Multidisciplinary discussion, involving at minimum a pulmonologist and a radiologist with expertise in the differential diagnosis of ILDs, is required to ensure the most accurate diagnosis. Prompt diagnosis of IPF is important to enable patients to receive appropriate care from an early stage. Optimal management of IPF involves the use of antifibrotic drugs, as well as the provision of supportive care to alleviate symptoms and preserve patients' quality of life. Antifibrotic drugs have been shown to slow lung function decline seen in patients with IPF. Patients' symptoms and functional capacity can be improved through participation in pulmonary rehabilitation programs and the use of supplemental oxygen. Patient education is essential to help patients understand and manage their disease. The identification and management of comorbidities, such as obstructive sleep apnea, pulmonary hypertension, and emphysema, is also an important element of the overall care of patients with IPF. Patients with IPF should be evaluated for lung transplantation at an early stage to maximize their chances of meeting eligibility criteria. In this review, we describe the clinical course and impact of IPF and best practice in its management, highlighting the importance of taking a patient-centered approach.

Keywords: Dyspnea; Fibrosis; Idiopathic pulmonary fibrosis; Interstitial lung disease.

Fig. 1

Examples of disease course in patients with IPF. Daily FVC measurements for subjects with (a) inexorably progressive disease, (b) rapidly progressive disease, and (c) an acute exacerbation. Each point represents a single FVC measurement. From: Russell et al., 2016 [8]. Reprinted with permission of the American Thoracic Society. Copyright© 2018 American Thoracic Society. Russell AM, et al./2016/Daily home spirometry: an effective tool for detecting progression in idiopathic pulmonary fibrosis/Am J Respir Crit Care Med/194/989–997. The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society

Fig. 2

The patient journey in idiopathic pulmonary fibrosis

Fig. 3

Effect of antifibrotic therapies on lung function decline [24]. Reprinted from The Lancet, Vol. 389, Richeldi L, et al., Idiopathic pulmonary fibrosis, Page No. 1941–1952., Copyright (2017), with permission from Elsevier

Fig. 4

Photosensitivity in pirfenidone-treated patients [41]. Reprinted by permission from RightsLink®: Springer, Advances in Therapy, 31(4):375–91, Pirfenidone in idiopathic pulmonary fibrosis: expert panel discussion on the management of drug-related adverse events, Costabel U, et al., Copyright® 2014

Fig. 5

A holistic approach to the management of patients with IPF