% 0期刊文章%瑞尔森,Christopher j . % Urbania, Thomas H. %A Richeldi, Luca %A Mooney, Joshua J. %A Lee, Joyce S. %A Jones, Kirk D. %A Elicker, Brett M. %A Koth, Laura L. %A King, Talmadge E. %A Wolters, Paul J. %A Collard, Harold R. %T Prevalence and prognosis of unclassifiable interstitial lung disease %D 2013 %R 10.1183/09031936.00131912 %J European Respiratory Journal %P 750-757 %V 42 %N 3 %X 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. Unclassifiable ILD has a heterogeneous clinical course that can be predicted using clinical and radiological variables http://ow.ly/mdjwg %U //www.qdcxjkg.com/content/erj/42/3/750.full.pdf