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Expert Rev Respir Med. 2019 Jul;13(7):601-614. doi: 10.1080/17476348.2019.1627204. Epub 2019 Jun 8.

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

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a Division of Respiratory Medicine, Department of Medicine , Queen's University , Kingston , ON , Canada.
b Division of Respirology, Department of Medicine , University Health Network , Toronto , ON , Canada.
c Division of Pulmonary Medicine, Department of Medicine , University of Alberta , Edmonton , AB , Canada.
d Hoffmann-La Roche Ltd , Mississauga , ON , Canada.
e Division of Palliative Medicine, Department of Medicine and Department of Oncology , Queen's University , Kingston , ON , Canada.


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.


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

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