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Prevalence and prognosis of unclassifiable interstitial lung disease

Prevalence and prognosis of unclassifiable interstitial lung disease
Prevalence and prognosis of unclassifiable interstitial lung disease
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.
0903-1936
750-757
Ryerson, Christopher J.
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Urbania, Thomas H.
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Richeldi, Luca
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Mooney, Joshua J.
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Lee, Joyce S.
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Jones, Kirk D.
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Elicker, Brett M.
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Koth, Laura L.
affd9c40-0a8f-4121-92bf-a61a4a851930
King, Talmadge E.
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Wolters, Paul J.
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Collard, Harold R.
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Ryerson, Christopher J.
d7904e80-fa84-4514-95b3-4f24acbcce1f
Urbania, Thomas H.
a84e65db-4370-4e3d-95d2-9dfca4cc90eb
Richeldi, Luca
47177d9c-731a-49a1-9cc6-4ac8f6bbbf26
Mooney, Joshua J.
fa51b2d0-251b-4e5d-a35c-9b94201a876c
Lee, Joyce S.
76fedf8e-1be0-45b9-8f0c-14a4cd32273f
Jones, Kirk D.
22fb3000-abeb-4cb0-bf2f-4906230a76c0
Elicker, Brett M.
0fd41517-e990-46ad-941f-ed0cd82af30d
Koth, Laura L.
affd9c40-0a8f-4121-92bf-a61a4a851930
King, Talmadge E.
4514ccf1-980f-4216-bfe9-26f6f3c96638
Wolters, Paul J.
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Collard, Harold R.
6eee2ce5-3016-4c13-ac58-f30a77f7d141

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. and Collard, Harold R. (2013) Prevalence and prognosis of unclassifiable interstitial lung disease. European Respiratory Journal, 42 (3), 750-757. (doi:10.1183/09031936.00131912). (PMID:23222877)

Record type: Article

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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Published date: September 2013
Organisations: Clinical & Experimental Sciences

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Local EPrints ID: 369040
URI: http://eprints.soton.ac.uk/id/eprint/369040
ISSN: 0903-1936
PURE UUID: 7ee4035b-828d-41b7-af8f-97cedd719c55

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Date deposited: 23 Sep 2014 12:50
Last modified: 14 Mar 2024 17:57

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Contributors

Author: Christopher J. Ryerson
Author: Thomas H. Urbania
Author: Luca Richeldi
Author: Joshua J. Mooney
Author: Joyce S. Lee
Author: Kirk D. Jones
Author: Brett M. Elicker
Author: Laura L. Koth
Author: Talmadge E. King
Author: Paul J. Wolters
Author: Harold R. Collard

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