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Review
. 2018 Sep 21:9:321-328.
doi: 10.2147/PROM.S144425. eCollection 2018.

Impact of novel antifibrotic therapy on patient outcomes in idiopathic pulmonary fibrosis: patient selection and perspectives

Affiliations
Review

Impact of novel antifibrotic therapy on patient outcomes in idiopathic pulmonary fibrosis: patient selection and perspectives

Bridget A Graney et al. Patient Relat Outcome Meas. .

Abstract

Patients with idiopathic pulmonary fibrosis, an incurable, progressive fibrotic interstitial lung disease, suffer an impaired quality of life due to symptoms, resultant functional limitations, and the constraints of supplemental oxygen. Two antifibrotic medications, nintedanib and pirfenidone, are approved for the treatment of idiopathic pulmonary fibrosis. Both medications slow the rate of decline of lung function, but their effect on patient-reported outcomes is not yet fully understood. Nintedanib may slow the decline in health-related quality of life for treated patients. Pirfenidone may slow the progression of dyspnea and improve cough. Patients and providers should participate in shared decision-making when starting antifibrotic therapy, taking into consideration the benefits of treatment in addition to drug-related side effects and dosing schedules. Although antifibrotic therapy may have an impact on health-related quality of life, providers should also focus on comprehensive care of the patient to improve health-related outcomes. This includes a multidisciplinary evaluation, diagnosis and treatment of comorbid medical conditions, and referral to and participation in a pulmonary rehabilitation program.

Keywords: idiopathic pulmonary fibrosis; interstitial lung disease; nintedanib; patient-reported outcome measures; pirfenidone; quality of life.

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Conflict of interest statement

Disclosure JSL is supported by National Institutes of Health/National Heart, Lung, and Blood Institute K23-HL138131. The authors report no conflicts of interest in this work.

Figures

Figure 1
Figure 1
Positive and negative influences on HRQL in IPF. Notes: Symptoms, and dyspnea in particular, are the most influential drivers of impaired HRQL in IPF. Other drivers of impaired HRQL include the resultant physical functional limitations due to deconditioning, mood disorders and comorbidities, and the constraints of supplemental oxygen. Pulmonary rehabilitation is associated with improved HRQL in IPF. Pharmacologic therapies, management of comorbid conditions, and a multidisciplinary approach to diagnosis and management may also improve HRQL for some patients. Abbreviations: HRQL, health-related quality of life; IPF, idiopathic pulmonary fibrosis.

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