Idiopathic Pulmonary Fibrosis: Best Practice in Monitoring and Managing a Relentless Fibrotic Disease

Abstract

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease that is, by definition, progressive. Progression of IPF is reflected by a decline in lung function, worsening of dyspnea and exercise capacity, and deterioration in health-related quality of life. In the short term, the course of disease for an individual patient is impossible to predict. A period of relative stability in forced vital capacity (FVC) does not mean that FVC will remain stable in the near future. Frequent monitoring using multiple assessments, not limited to pulmonary function tests, is important to evaluate disease progression in individual patients and ensure that patients are offered appropriate care. Optimal management of IPF requires a multidimensional approach, including both pharmacological therapy to slow decline in lung function and supportive care to preserve patients' quality of life.

Keywords: Interstitial lung disease; Mortality; Nintedanib; Pirfenidone; Therapeutics; Treatment.

Fig. 1

Lung function decline in patients with IPF treated with placebo in Phase II and III clinical trials [16]. Red dots denote the mean or median change from baseline in FVC or VC in the placebo groups of Phase II and III clinical trials in patients with IPF. The black line denotes the mean decline in FVC in healthy subjects aged 60 years based on FVC measurements taken between 1987–1989, 1990–1992, and 2011–2013 [87]. Reproduced with permission of the ^© ERS 2019 [16]. This material has not been reviewed prior to release; therefore, the European Respiratory Society may not be responsible for any errors, omissions, or inaccuracies, or for any consequences arising there from, in the content. FVC, forced vital capacity; IPF, idiopathic pulmonary fibrosis.

Fig. 2

Risk of mortality in patients treated with placebo in the TOMORROW, INPULSIS, CAPACITY, and ASCEND trials in subgroups by baseline variables, decline in FVC, and acute exacerbations (adapted from [17]). Comparisons were made to reference levels: age <65 years, never smoker, FVC >85% predicted, DLco >50% predicted, FVC decline <5% predicted, no acute exacerbation. Reprinted with permission of the American Thoracic Society. Copyright ^© 2019 American Thoracic Society [17]. FVC, forced vital capacity.

Fig. 3

Trajectory of FVC following stability or decline in previous year (adapted from [19]). Mixed-models analysis of trend in FVC. Solid lines indicate mean FVC; dashed lines indicate 95% CI. Reprinted from [19]. Copyright (2019) with permission from the American College of Chest Physicians; permission conveyed through Copyright Clearance Center, Inc. FVC, forced vital capacity.

Fig. 4

A multifaceted approach to managing patients with IPF (adapted from [88]). Reprinted from [88]. Copyright (2019) with permission from Elsevier; permission conveyed through Copyright Clearance Center, Inc.