Raghu Ganesh, Richeldi Luca
University of Washington, Seattle, WA, USA.
Catholic University of the Sacred Heart, Rome, Italy.
Respir Med. 2017 Aug;129:24-30. doi: 10.1016/j.rmed.2017.05.017. Epub 2017 May 30.
Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal lung disease associated with dyspnoea, cough and impaired quality of life. Currently, the aims of patient care are to improve outcomes for patients by slowing the progression of the disease, extending life, and improving quality of life. A prompt, accurate diagnosis is important to enable patients to receive treatment early in the course of the disease and to be considered for lung transplantation. Two anti-fibrotic drugs, nintedanib and pirfenidone, have been shown to reduce decline in lung function in patients with IPF. In addition to pharmacological therapy, optimal management of IPF includes treatment of comorbidities, symptom relief, pulmonary rehabilitation, and palliative care. Patient education is important to enable patients to make decisions about their care and to help them manage their disease and the side-effects of anti-fibrotic drugs. Research continues into new treatments and combinations of treatments that may improve outcomes for patients with this devastating disease.
特发性肺纤维化（IPF）是一种进行性且最终致命的肺部疾病，与呼吸困难、咳嗽及生活质量受损相关。目前，患者护理的目标是通过减缓疾病进展、延长寿命及改善生活质量来提高患者的治疗效果。及时、准确的诊断对于使患者能够在疾病进程早期接受治疗并被考虑进行肺移植至关重要。两种抗纤维化药物，尼达尼布和吡非尼酮，已被证明可减少IPF患者的肺功能下降。除药物治疗外，IPF的最佳管理包括合并症的治疗、症状缓解、肺康复及姑息治疗。患者教育对于使患者能够就其护理做出决策并帮助他们管理疾病及抗纤维化药物的副作用很重要。针对可能改善这种毁灭性疾病患者治疗效果的新治疗方法及联合治疗方法的研究仍在继续。
