Models of disease behavior in idiopathic pulmonary fibrosisReport as inadecuate

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BMC Medicine

, 13:165

First Online: 24 September 2015Received: 15 April 2015Accepted: 19 June 2015DOI: 10.1186-s12916-015-0403-7

Cite this article as: Johannson, K.A., Ley, B. & Collard, H.R. BMC Med 2015 13: 165. doi:10.1186-s12916-015-0403-7


Idiopathic pulmonary fibrosis is a diffuse parenchymal lung disease of unknown cause. The natural history of disease can vary considerably, making it difficult to predict the clinical trajectory for an individual patient. Accurate prognostication is desirable for clinical management as well as for cohort enrichment in clinical trials of therapeutics. Clinical and biomarker models of disease behavior have been developed to improve prognostication in idiopathic pulmonary fibrosis, with moderate predictive capabilities. Integrated prediction models that combine both clinical and biomarker variables will improve prognostication for patients and improved cohort enrichment strategies for clinical trials. This goal may be best achieved through collaborative patient registries with prospectively collected biological samples that allow for characterization of disease behavior in idiopathic pulmonary fibrosis.

KeywordsBiological markers Idiopathic pulmonary fibrosis Interstitial lung disease Prognosis AbbreviationsDLCODiffusion capacity of the lung for carbon monoxide

FVCForced vital capacity

IPFIdiopathic pulmonary fibrosis

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Author: Kerri A. Johannson - Brett Ley - Harold R. Collard


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