Idiopathic pulmonary fibrosis (IPF) is one of the most common forms of interstitial lung disease, with a median survival time of two to five years. Most patients with IPF experience chronic breathlessness, which is closely linked to poor perceived quality of life and significant restriction of daily activities; therefore, effective management of this distressing symptom is a major goal of patient care. Areas covered: This report summarizes the physiology of IPF during rest and exercise, outlines current concepts of the mechanisms of breathlessness, and provides a physiological rationale for optimal management of individual patients. It also examines the evidence for efficacy of a number of therapeutic interventions currently at our disposal for the management of breathlessness in IPF, which aim to reduce respiratory neural drive, reduce worsening of mechanical load, and alter central perception. Expert commentary: The current evidence supporting general measures in relieving chronic breathlessness is weak; hence, more carefully designed prospective studies are required.
Keywords: Idiopathic pulmonary fibrosis; breathlessness; dyspnea; interstitial lung disease; symptom management.