Prevalence and prognosis of unclassifiable interstitial lung disease
Prevalence and prognosis of unclassifiable interstitial lung disease
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DOI：
10.1183/09031936.00131912
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发表时间：
2013-09-01
影响因子：
24.3
通讯作者：
Collard, Harold R.
中科院分区：
文献类型：
--
作者：
Ryerson, Christopher J.;Urbania, Thomas H.;Collard, Harold R.
本研究的目的是确定未分类间质性肺病（ILD）患者的患病率、特征和结局，并开发一种预测疾病行为的简单方法。在多学科审查后诊断为无法分类的ILD，但未确定具体的ILD诊断。比较了特发性肺纤维化（IPF）和非IPF ILD的临床特征和结局。采用考克斯比例风险分析确定死亡率的独立预测因子，以确定具有不同疾病行为的亚组。无法分类的最常见原因是由于手术肺活检风险高而缺失组织病理学评估。无法分类的ILD的人口统计学和生理学特征介于IPF和非IPF疾病对照之间。在校正分析中，与IPF相比，不可分类ILD的生存率更长（风险比0.62，p=0.04），与非IPF ILD相比，生存率相似（风险比1.54，p=0.12）。不可分类的ILD患者生存率的独立预测因子包括肺一氧化碳弥散量（p=0.001）和放射学纤维化评分（p=0.02）。不可分类的ILD约占ILD病例的10%，并且具有异质性的临床病程，可以使用临床和放射学变量进行预测。
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.