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Prevalence and prognosis of unclassifiable interstitial lung disease

Open access
Ryerson, Christopher J., Urbania, Thomas H., Richeldi, Luca, Mooney, Joshua J., Lee, Joyce S., Jones, Kirk D., Elicker, Brett M., Koth, Laura L., King, Talmadge E., Wolters, Paul J., Collard, Harold R.·2013·European Respiratory Journal

Abstract

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.

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Metadata

Authors
Ryerson, C. J., Urbania, T. H., Richeldi, L., Mooney, J. J., Lee, J. S., Jones, K. D., Elicker, B. M., Koth, L. L., King, T. E., Wolters, P. J., & Collard, H. R.
Venue
European Respiratory Journal
Published
2012-12-06
Volume
42
Issue
3
Pages
750-757
ISSN
  • 0903-1936 (ISSN-L)
  • 1399-3003
OpenAlex
W2157572334
Cited by
310
References
21
FWCI
10.43
Type
journal article
Language
en
Open access
bronze

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CoChat. (2026). Citation check: Prevalence and prognosis of unclassifiable interstitial lung disease. https://app.cochat.ai/papers/10.1183/09031936.00131912
The paper
Ryerson, C. J., Urbania, T. H., Richeldi, L., Mooney, J. J., Lee, J. S., Jones, K. D., Elicker, B. M., Koth, L. L., King, T. E., Wolters, P. J., & Collard, H. R. (2013). Prevalence and prognosis of unclassifiable interstitial lung disease. European Respiratory Journal. https://doi.org/10.1183/09031936.00131912

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.