Non-pharmacological therapy for IPF: Is respiratory care actually effective?

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease characterized by a poor prognosis and the lack of proven effective treatments. The symptoms and complications of IPF such as dyspnea, exercise intolerance, and depression severely impair patients' quality of life (QOL) and decrease social participation. Although evidence for the benefit of pulmonary rehabilitation (PR) in IPF is limited, it has recently been reported that PR can improve dyspnea and exercise tolerance. Furthermore, exercise training and educational programs may also be effective means of addressing low mood and depression in selected patients with IPF. Long-term oxygen therapy (LTOT) is also considered to improve QOL in patients with IPF. Although LTOT may have no survival benefit, patients with resting hypoxemia, pulmonary hypertension, exercise-induced hypoxemia, or nocturnal hypoxemia should be treated with LTOT to improve QOL. Noninvasive ventilation and nasal high-flow oxygen therapy have been recently used to manage acute respiratory failure complicating IPF. Early use of these techniques might afford the opportunity to avoid tracheal intubation and reduce the high incidence of mortality associated with acute deterioration of respiratory function in IPF. In conclusion, the evidence base informing management strategies for IPF has been growing gradually. It appears that PR may have an important role to play in improving the QOL of patients with IPF, although further research is still needed.