Dept of Medicine, University of British Columbia, Vancouver, BC, Canada.
Eur Respir J. 2013 Sep;42(3):750-7. doi: 10.1183/09031936.00131912. Epub 2012 Dec 6.
The aim of this study was to determine the prevalence, characteristics and outcomes of patients with unclassifiable interstitial lung disease (ILD) and to develop a simple method of predicting disease behaviour. Unclassifiable ILD patients were identified from an ongoing longitudinal cohort. Unclassifiable ILD was diagnosed after a multidisciplinary review did not secure a specific ILD diagnosis. Clinical characteristics and outcomes were compared with idiopathic pulmonary fibrosis (IPF) and non-IPF ILDs. Independent predictors of mortality were determined using Cox proportional-hazards analysis to identify subgroups with distinct disease behaviour. Unclassifiable ILD was diagnosed in 10% of the ILD cohort (132 out of 1370 patients). The most common reason for being unclassifiable was missing histopathological assessment due to a high risk of surgical lung biopsy. Demographic and physiological features of unclassifiable ILD were intermediate between IPF and non-IPF disease controls. Unclassifiable ILD had longer survival rates when compared to IPF on adjusted analysis (hazard ratio 0.62, p = 0.04) and similar survival compared to non-IPF ILDs (hazard ratio 1.54, p = 0.12). Independent predictors of survival in unclassifiable ILD included diffusion capacity of the lung for carbon monoxide (p = 0.001) and a radiological fibrosis score (p = 0.02). Unclassifiable ILD represents approximately 10% of ILD cases and has a heterogeneous clinical course, which can be predicted using clinical and radiological variables.
本研究旨在确定无法分类的间质性肺疾病（ILD）患者的患病率、特征和结局，并开发一种简单的预测疾病行为的方法。无法分类的ILD 患者是从正在进行的纵向队列中确定的。在多学科审查未能确定特定的ILD 诊断后，诊断为无法分类的ILD。比较了无法分类的ILD 与特发性肺纤维化（IPF）和非 IPFILD 的临床特征和结局。使用 Cox 比例风险分析确定死亡率的独立预测因素，以确定具有不同疾病行为的亚组。在 1370 名ILD 患者中，10%（132 名）被诊断为无法分类的ILD。无法分类的最常见原因是由于手术肺活检风险高而缺少组织病理学评估。无法分类的ILD 的人口统计学和生理学特征在 IPF 和非 IPF 疾病对照之间处于中间位置。调整分析显示，与 IPF 相比，无法分类的ILD 的生存率更高（风险比 0.62，p = 0.04），与非 IPFILD 的生存率相似（风险比 1.54，p = 0.12）。无法分类的ILD 的生存独立预测因素包括肺一氧化碳弥散量（p = 0.001）和影像学纤维化评分（p = 0.02）。无法分类的ILD 占ILD 病例的 10%左右，具有异质性的临床过程，可使用临床和影像学变量进行预测。
