Department of Medicine, University of British Columbia.
Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, British Columbia, Canada.
Curr Opin Pulm Med. 2018 Sep;24(5):461-468. doi: 10.1097/MCP.0000000000000509.
Accurate diagnosis of interstitial lung diseases (ILDs) can be challenging, and a substantial percentage of ILD patients remain unclassifiable even after thorough assessment by an experienced multidisciplinary team. In this review, we summarize the recent literature on the definition, prevalence, diagnosis, treatment, and prognosis of unclassifiable ILD, and also discuss important current issues and provide future perspectives on the classification of ILD.
Approximately 12% of patients with ILD are considered unclassifiable, with large variability across previous studies that is in part secondary to inconsistent definitions of unclassifiable ILD and other ILD subtypes. A recent International Working Group suggested that unclassifiable ILD should be defined by the absence of a leading diagnosis that is considered more likely than not after multidisciplinary discussion of all available information. Clinical features and outcomes of unclassifiable ILD are intermediate between idiopathic pulmonary fibrosis and nonidiopathic pulmonary fibrosis ILD cohorts, and choices for pharmacotherapy should be considered on a case-by-case basis.
Recent studies have provided additional data on the clinical features and prognosis of unclassifiable ILD, but also highlight the many uncertainties that still exist in ILD diagnosis and classification. New tools are needed to more accurately characterize patients with unclassifiable ILD.
准确诊断间质性肺疾病（ILDs）具有挑战性，即使在经验丰富的多学科团队进行全面评估后，仍有相当一部分 ILD 患者无法分类。在这篇综述中，我们总结了最近关于无法分类的 ILD 的定义、患病率、诊断、治疗和预后的文献，并讨论了当前的重要问题，并对 ILD 的分类提供了未来的观点。
大约 12%的ILD 患者被认为是无法分类的，这在以前的研究中有很大的差异，部分原因是无法分类的 ILD 和其他 ILD 亚型的定义不一致，以及其他因素。最近的一个国际工作组建议，无法分类的 ILD 应该定义为在对所有可用信息进行多学科讨论后，没有一个被认为更有可能的主要诊断。无法分类的 ILD 的临床特征和结局介于特发性肺纤维化和非特发性肺纤维化 ILD 队列之间，药物治疗的选择应根据具体情况考虑。
最近的研究提供了更多关于无法分类的 ILD 的临床特征和预后的数据，但也强调了ILD 诊断和分类中仍然存在许多不确定因素。需要新的工具来更准确地描述无法分类的 ILD 患者。
