Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI, USA
Both authors contributed equally.
Eur Respir Rev. 2018 Dec 21;27(150). doi: 10.1183/16000617.0078-2018. Print 2018 Dec 31.
There are over 200 interstitial lung diseases (ILDs). In addition to patients with idiopathic pulmonary fibrosis (IPF), a percentage of patients with other ILDs also develop progressive fibrosis of the lung during their disease course. Patients with progressive-fibrosing ILDs may show limited response to immunomodulatory therapy, worsening symptoms and lung function and, ultimately, early mortality. There are few data for ILDs that may present a progressive fibrosing phenotype specifically, but we believe the burden and healthcare costs associated with these conditions may be comparable to those reported in IPF. This review discusses the burden of ILDs that may present a progressive fibrosing phenotype and the factors impacting healthcare utilisation.
有超过 200 种间质性肺疾病 (ILDs)。除了特发性肺纤维化 (IPF) 患者外,一部分患有其他 ILD 的患者在疾病过程中也会出现肺部进行性纤维化。进行性纤维化ILD 患者的免疫调节治疗反应可能有限,症状和肺功能恶化,最终导致早期死亡。ILDs 中可能存在具有进行性纤维化表型的特定ILDs 的相关数据较少,但我们认为这些疾病的负担和医疗保健费用可能与 IPF 报告的情况相当。本文综述了具有进行性纤维化表型的ILDs 的负担,以及影响医疗保健利用的因素。