Berger Kristin, Kaner Robert J
Division of Pulmonary and Critical Care Medicine, Department of Medicine, Weill Cornell Medicine, New York, NY 10021, USA.
Department of Genetic Medicine, Weill Cornell Medicine, New York, NY 10021, USA.
Life (Basel). 2023 Feb 21;13(3):599. doi: 10.3390/life13030599.
Interstitial lung disease is an umbrella term that encompasses a spectrum of parenchymal lung pathologies affecting the gas exchanging part of the lung. While many of these disease entities are not fibrotic in nature, a number can lead to pulmonary fibrosis which may or may not progress over time. Idiopathic pulmonary fibrosis is the prototypical, progressive fibrotic interstitial lung disease, which can lead to worsening hypoxemic respiratory failure and mortality within a number of years from the time of diagnosis. The importance of an accurate and timely diagnosis of interstitial lung diseases, which is needed to inform prognosis and guide clinical management, cannot be overemphasized. Developing a consensus diagnosis requires the incorporation of a variety of factors by a multidisciplinary team, which then may or may not determine a need for tissue sampling. Clinical management can be challenging given the heterogeneity of disease behavior and the paucity of controlled trials to guide decision making. This review addresses current paradigms and recent updates in the diagnosis and pharmacologic management of these fibrotic interstitial lung diseases.
间质性肺疾病是一个统称，涵盖了一系列影响肺部气体交换部分的实质性肺部病变。虽然这些疾病实体中的许多本质上并非纤维化，但有一些会导致肺纤维化，其可能会随着时间推移而进展，也可能不会。特发性肺纤维化是典型的进行性纤维化间质性肺疾病，可导致低氧性呼吸衰竭恶化，并在诊断后的数年内导致死亡。准确及时诊断间质性肺疾病对于判断预后和指导临床管理至关重要，这一点无论如何强调都不为过。达成共识诊断需要多学科团队综合考虑多种因素，然后可能会也可能不会确定是否需要进行组织采样。鉴于疾病行为的异质性以及指导决策的对照试验匮乏，临床管理可能具有挑战性。本综述阐述了这些纤维化间质性肺疾病在诊断和药物治疗方面的当前模式及最新进展。
