Zou Richard H, Kass Daniel J, Gibson Kevin F, Lindell Kathleen O
Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA.
Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease at UPMC, University of Pittsburgh, Pittsburgh, PA, USA.
Pulm Ther. 2020 Jun;6(1):35-46. doi: 10.1007/s41030-019-00108-2. Epub 2020 Jan 4.
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with a median survival of 3-4 years from time of initial diagnosis, similar to the time course of many malignancies. A hallmark of IPF is its unpredictable disease course, ranging from long periods of clinical stability to acute exacerbations with rapid decompensation. As the disease progresses, patients with chronic cough and progressive exertional dyspnea become oxygen dependent. They may experience significant distress due to concurrent depression, anxiety, and fatigue, which often lead to increased symptom burden and decreased quality of life. Despite these complications, palliative care is an underutilized, and often underappreciated, resource before end-of-life care in this population. While there is growing recognition about early palliative care in IPF, current data suggest referral patterns vary widely based on institutional practices. In addition to focusing on symptom management, there is emphasis on supplemental oxygen use, pulmonary rehabilitation, quality of life, and end-of-life care. Importantly, increased use of support groups and national foundation forums have served as venues for further disease education, communication, and advanced care planning outside of the hospital settings. The purpose of this review article is to discuss the clinical features of IPF, the role of palliative care in chronic disease management, current data supporting benefits of palliative care in IPF, its role in symptom management, and practices to help patients and their caregivers achieve their best quality of life.
特发性肺纤维化（IPF）是一种进行性纤维化性肺病，从初次诊断起的中位生存期为3至4年，与许多恶性肿瘤的病程相似。IPF的一个特点是其疾病进程不可预测，从长期临床稳定到急性加重并迅速失代偿。随着疾病进展，患有慢性咳嗽和进行性劳力性呼吸困难的患者会出现氧依赖。他们可能因并发的抑郁、焦虑和疲劳而遭受极大痛苦，这些常常导致症状负担加重和生活质量下降。尽管存在这些并发症，但在该人群临终关怀之前，姑息治疗是一种未得到充分利用且常被低估的资源。虽然人们对IPF早期姑息治疗的认识不断提高，但目前的数据表明，转诊模式因机构做法而异。除了关注症状管理外，还强调补充氧气的使用、肺康复、生活质量和临终关怀。重要的是，越来越多地使用支持小组和国家基金会论坛，为医院环境之外的进一步疾病教育、交流和高级护理规划提供了场所。这篇综述文章的目的是讨论IPF的临床特征、姑息治疗在慢性病管理中的作用、支持姑息治疗对IPF有益的当前数据、其在症状管理中的作用，以及帮助患者及其护理人员实现最佳生活质量的做法。
