Wijsenbeek Marlies, Suzuki Atsushi, Maher Toby M
Center for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC, University Medical Center Rotterdam, Rotterdam, Netherlands.
Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Aichi, Japan.
Lancet. 2022 Sep 3;400(10354):769-786. doi: 10.1016/S0140-6736(22)01052-2. Epub 2022 Aug 11.
Over 200 interstitial lung diseases, from ultra rare to relatively common, are recognised. Most interstitial lung diseases are characterised by inflammation or fibrosis within the interstitial space, the primary consequence of which is impaired gas exchange, resulting in breathlessness, diminished exercise tolerance, and decreased quality of life. Outcomes vary considerably for each of the different interstitial lung diseases. In some conditions, spontaneous reversibility or stabilisation can occur, but unfortunately in many people with interstitial lung disease, especially in those manifesting progressive pulmonary fibrosis, respiratory failure and death are a sad reality. Over the past 3 years, the field of interstitial lung disease has had important advances, with the approval of drugs to treat systemic sclerosis-associated interstitial lung disease, interstitial lung disease-associated pulmonary hypertension, and different forms of progressive pulmonary fibrosis. This Seminar provides an update on epidemiology, pathogenesis, presentation, diagnosis, disease course, and management of the interstitial lung diseases that are most frequently encountered in clinical practice. Furthermore, we describe how developments have led to a shift in the classification and treatment of interstitial lung diseases that exhibit progressive pulmonary fibrosis and summarise the latest practice-changing guidelines. We conclude with an outline of controversies, uncertainties, and future directions.
已确认有200多种间质性肺疾病，从极其罕见的到相对常见的都有。大多数间质性肺疾病的特征是间质空间内存在炎症或纤维化，其主要后果是气体交换受损，导致呼吸困难、运动耐量下降和生活质量降低。不同的间质性肺疾病，其预后差异很大。在某些情况下，可能会出现自发逆转或病情稳定，但不幸的是，许多间质性肺疾病患者，尤其是那些表现为进行性肺纤维化的患者，呼吸衰竭和死亡是残酷的现实。在过去3年里，间质性肺疾病领域取得了重要进展，治疗系统性硬化症相关间质性肺疾病、间质性肺疾病相关肺动脉高压以及不同形式的进行性肺纤维化的药物已获批准。本次研讨会提供了临床实践中最常遇到的间质性肺疾病的流行病学、发病机制、临床表现、诊断、病程及管理方面的最新信息。此外，我们描述了这些进展如何导致表现为进行性肺纤维化的间质性肺疾病在分类和治疗上的转变，并总结了最新的改变临床实践的指南。我们最后概述了争议、不确定性及未来方向。
