A comprehensive and practical approach to the management of idiopathic pulmonary fibrosis

ABSTRACT

Introduction: Idiopathic pulmonary fibrosis (IPF) is a debilitating, progressive, and fatal fibrotic pulmonary disease with a prognosis comparable to that of lung cancer. IPF management is a complex process that involves pharmacological and nonpharmacological interventions, extensive patient education, and addressing patient needs that change through the course of the illness.

Areas covered: This review summarizes the key aspects of a multifaceted, multidisciplinary, individualized approach to IPF care that incorporates available treatment options, strategies to improve compliance with antifibrotic therapies, pulmonary rehabilitation, and the integration of palliative care for symptom management. Aspects of care discussed include the use of antifibrotic therapy and nonpharmacological treatments, targeted education and psychosocial support, evaluation and management of comorbidities, and early integration of palliative care.

Expert opinion: By incorporating this comprehensive approach to disease management, physicians can address most aspects of care for a patient with IPF to optimize survival and quality of life.

KEYWORDS:

• Idiopathic pulmonary fibrosis
• interstitial lung disease
• disease management
• disease progression
• pirfenidone
• nintedanib

Article Highlights

• The management of IPF is complex due to the unpredictable rate of disease progression, adverse events associated with antifibrotic therapies, the burden of comorbidities, the significant symptom burden, and the changing care and education needs of patients throughout the course of the disease

• A multifaceted, multidisciplinary approach to IPF management, tailored to each patient’s individual needs is recommended to optimally manage a patient’s overall health and expectations

• Key aspects of a multifaceted approach to IPF management include extensive patient education; early initiation of antifibrotic therapy, supplemental oxygen therapy, and pulmonary rehabilitation; monitoring and management of IPF-associated comorbidities; early integration of palliative care for managing IPF symptoms, especially cough and dyspnea; psychosocial support and nutritional counseling; and dynamic conversations on advance care planning and to establish goals of care throughout the illness journey

Acknowledgments

The authors acknowledge Benjamin L. Ricca, PhD, of Health Interactions, Inc – third-party writing assistance funded by Hoffmann-La Roche Ltd.

Declaration of interest

Dr. Moran-Mendoza reports research funding, honoraria, clinical trial participation fees, and funding of editorial support from Hoffmann-La Roche Ltd. and consultancy fees from Boehringer Ingelheim and Hoffmann-La Roche Ltd. Dr. Colman reports honoraria from Boehringer Ingelheim and Hoffmann-La Roche Ltd. and funding of editorial support from Hoffmann-La Roche Ltd. Meena Kalluri declares research funding, honoraria, clinical trial participation fees, and funding of editorial support from Hoffmann-La Roche Ltd. and consultancy fees from Boehringer Ingelheim and Hoffmann-La Roche Ltd. Dr Cabalteja is an employee of Hoffmann-La Roche Ltd. Dr Harle reports funding of editorial support from Hoffmann-La Roche Ltd. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewers Disclosure

Peer reviewers on this manuscript have no relevant financial relationships or otherwise to disclose.

Additional information

Funding

This paper was sponsored by Hoffmann-La Roche Ltd.