Dept of Medicine, University of British Columbia and Centre for Heart Lung Innovation, St Paul's Hospital, Vancouver, BC, Canada
Dept of Respiratory Medicine, Royal Prince Alfred Hospital, University of Sydney, Centre of Research Excellence for Pulmonary Fibrosis, Sydney, Australia.
Eur Respir J. 2021 Dec 16;58(6). doi: 10.1183/13993003.00276-2021. Print 2021 Dec.
Fibrotic interstitial lung diseases (ILDs) frequently have nonspecific and overlapping clinical and radiological features, resulting in ∼10-20% of patients with ILD lacking a clear diagnosis and thus being labelled with unclassifiable ILD. The objective of this review is to describe how patients with unclassifiable ILD should be evaluated, and what impact specific clinical, radiological and histopathological features may have on management decisions, focusing on patients with a predominantly fibrotic phenotype. We highlight recent data that have suggested an increasing role for antifibrotic medications in a variety of fibrotic ILDs, but justify the ongoing importance of making an accurate ILD diagnosis given the benefit of immunomodulatory therapies in many patient populations. We provide a practical approach to support management decisions that can be used by clinicians and tested by clinical researchers, and further identify the need for additional research to support a rational and standardised approach to the management of patients with unclassifiable ILD.
纤维性间质性肺疾病（ILDs）常具有非特异性和重叠的临床及影像学特征，导致约 10-20%的ILD 患者缺乏明确的诊断，因此被归类为无法分类的ILD。本综述的目的在于描述如何评估无法分类的ILD 患者，以及特定的临床、影像学和组织病理学特征可能对管理决策产生何种影响，重点关注以纤维化为主要表型的患者。我们强调了最近的数据，这些数据表明在各种纤维性ILD 中，抗纤维化药物的作用越来越大，但鉴于免疫调节疗法在许多患者群体中的益处，仍需做出准确的ILD 诊断。我们提供了一种实用的方法来支持临床医生做出管理决策，并由临床研究人员进行测试，进一步确定需要进一步研究以支持对无法分类的ILD 患者进行合理和标准化的管理。
