Mullholand Jon B, Grossman Catherine E, Perelas Apostolos
Division of Pulmonary Disease and Critical Care Medicine, Virginia Commonwealth University, Richmond, VA 23298, USA.
J Clin Med. 2025 Feb 17;14(4):1317. doi: 10.3390/jcm14041317.
Idiopathic pulmonary fibrosis (IPF) is a relatively common progressive fibrotic interstitial lung disease associated with significant morbidity and mortality. The available medications for IPF only slow down the disease process, with lung transplantation the only option for a cure. Non-pharmacological therapies are significant adjuncts that can improve symptom burden and quality of life with minimal or no side effects. Supplemental oxygen can improve exercise capacity and the sensation of dyspnea in a significant portion of patients with resting or exertional hypoxemia and has been supported by several professional societies. Pulmonary rehabilitation is a comprehensive program that includes education and therapeutic exercises to improve patient stamina and strength. It is one of the few interventions that have been shown to produce a meaningful increase in a patient's exercise capacity, but its wide adoption is limited by availability, especially in rural areas. Sleep optimization with supplemental oxygen and positive airway pressure therapy should actively be investigated for all patients diagnosed with IPF. Although gastroesophageal reflux control with non-pharmacological means is still controversial as an intervention to reduce the rate of lung function decline, it can help control reflux symptoms and improve cough intensity. IPF patients should be educated on the importance of balanced nutrition and the potential benefits of screening for lung transplantation. Palliative medicine can help with symptom control and should be considered for all patients regardless severity, but especially in those in the later stages of disease.
特发性肺纤维化(IPF)是一种相对常见的进行性纤维化间质性肺疾病,具有较高的发病率和死亡率。现有的IPF治疗药物只能减缓疾病进程,肺移植是唯一的治愈选择。非药物疗法是重要的辅助手段,可减轻症状负担、提高生活质量,且副作用极小或无副作用。对于很大一部分静息或运动性低氧血症患者,补充氧气可提高运动能力并缓解呼吸困难感,这已得到多个专业学会的支持。肺康复是一个综合项目,包括教育和治疗性锻炼,以提高患者的耐力和力量。它是少数已被证明能显著提高患者运动能力的干预措施之一,但其广泛应用受到可用性的限制,尤其是在农村地区。对于所有诊断为IPF的患者,应积极研究通过补充氧气和气道正压通气疗法来优化睡眠。尽管通过非药物手段控制胃食管反流作为降低肺功能下降速率的干预措施仍存在争议,但它有助于控制反流症状并减轻咳嗽强度。应向IPF患者宣传均衡营养的重要性以及肺移植筛查的潜在益处。姑息治疗有助于控制症状,所有患者无论病情严重程度如何都应考虑接受姑息治疗,尤其是疾病晚期患者。