RT Journal Article
SR Electronic
T1 Prevalence and prognosis of unclassifiable interstitial lung disease
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 750
OP 757
DO 10.1183/09031936.00131912
VO 42
IS 3
A1 Christopher J. Ryerson
A1 Thomas H. Urbania
A1 Luca Richeldi
A1 Joshua J. Mooney
A1 Joyce S. Lee
A1 Kirk D. Jones
A1 Brett M. Elicker
A1 Laura L. Koth
A1 Talmadge E. King, Jr
A1 Paul J. Wolters
A1 Harold R. Collard
YR 2013
UL http://erj.ersjournals.com/content/42/3/750.abstract
AB 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