Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL), Heidelberg, Germany.
Dept of Pulmonology, Semmelweis University, Budapest, Hungary.
Eur Respir Rev. 2019 Sep 4;28(153). doi: 10.1183/16000617.0021-2019. Print 2019 Sep 30.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial lung disease, characterised by progressive scarring of the lung and associated with a high burden of disease and early death. The pathophysiological understanding, clinical diagnostics and therapy of IPF have significantly evolved in recent years. While the recent introduction of the two antifibrotic drugs pirfenidone and nintedanib led to a significant reduction in lung function decline, there is still no cure for IPF; thus, new therapeutic approaches are needed. Currently, several clinical phase I-III trials are focusing on novel therapeutic targets. Furthermore, new approaches in nonpharmacological treatments in palliative care, pulmonary rehabilitation, lung transplantation, management of comorbidities and acute exacerbations aim to improve symptom control and quality of life. Here we summarise new therapeutic attempts and potential future approaches to treat this devastating disease.
特发性肺纤维化（IPF）是一种慢性、进行性、纤维性间质性肺疾病，其特征是肺部进行性瘢痕形成，并伴有疾病负担沉重和早期死亡。近年来，IPF 的病理生理学认识、临床诊断和治疗有了显著进展。虽然最近两种抗纤维化药物吡非尼酮和尼达尼布的引入显著减缓了肺功能下降，但 IPF 仍然无法治愈；因此，需要新的治疗方法。目前，几项临床 I-III 期试验都集中在新的治疗靶点上。此外，姑息治疗、肺康复、肺移植、合并症和急性加重管理中在非药物治疗方面的新方法旨在改善症状控制和生活质量。在这里，我们总结了治疗这种毁灭性疾病的新治疗尝试和潜在的未来方法。
