Idiopathic Pulmonary Fibrosis: A Review of Disease, Pharmacological, and Nonpharmacological Strategies With a Focus on Symptoms, Function, and Health-Related Quality of Life

Abstract

Despite several advances in treatment, idiopathic pulmonary fibrosis (IPF) remains a progressive, symptomatic, and terminal disease in patients not suitable for lung transplantation. With disease progression, IPF often leads to a constellation of symptoms, including dyspnea, cough, anxiety, and depression. Palliative care is appropriate to support these patients. However, traditional curriculum in palliative care has often focused on supporting patients with malignant disease, and clinicians are not universally trained to manage patients with progressive nonmalignant diseases such as IPF. Current antifibrotic therapies aim to slow disease progression but are not able to reduce symptoms or improve daily function and health-related quality of life (HRQL). Palliative care in this patient group requires an understanding of the clinical characteristics of IPF, comorbidities, common medications used, and nonpharmacological strategies that can be undertaken to improve daily function and HRQL. This review focuses on IPF management strategies and their effects on symptoms, exercise tolerance, HRQL, and survival. Pharmacological interactions and considerations related to commonly used palliative care medications are also reviewed. This review highlights the needs of patients with IPF and caregivers, psychosocial function, patient-reported assessment tools, and topics related to advance care planning.

Keywords: Pulmonary fibrosis; drug interactions; function; health-related quality of life; palliative care; symptom.