Abstract
BackgroundProgression of the disease in idiopathic pulmonary fibrosis (IPF) is difficult to predict, due to its variable and heterogenous course. The relationship between radiographic progression and functional decline in IPF is unclear. We sought to confirm that a simple HRCT fibrosis visual score is a reliable predictor of mortality in IPF, when longitudinally followed; and to ascertain which pulmonary functional variables best reflect clinically significant radiographic progression.MethodsOne-hundred-twenty-three consecutive patients with IPF from 2 centers were followed for an average of 3 years. Longitudinal changes of HRCT fibrosis scores, forced vital capacity (FVC), total lung capacity and diffusing lung capacity for carbon monoxide were considered. HRCTs were scored by 2 chest radiologists. The primary outcome was lung transplant (LTx)-free survival after the follow-up HRCT.ResultsDuring the follow-up period, 43 deaths and 11 LTx occurred. On average, the HRCT fibrosis score increased significantly, and a longitudinal increase > 7% predicted LTx-free survival significantly, with good specificity, but limited sensitivity. The correlation between radiographic and functional progression was moderately significant. HRCT progression and FVC decline predicted LTx-free survival independently and significantly, with better sensitivity, but worse specificity for a ≥ 5% decline of FVC. However, the area under the curve towards LTx-survival were only 0.61 and 0.62, respectively.ConclusionsThe HRCT fibrosis visual score is a reliable and responsive tool to detect clinically meaningful disease progression. Although no individual pulmonary function test closely reflects radiographic progression, a longitudinal FVC decline improves sensitivity in the detection of clinically significant disease progression. However, the accuracy of these methods remains limited, and better prognostication models need to be found.
Highlights
Progression of the disease in idiopathic pulmonary fibrosis (IPF) is difficult to predict, due to its variable and heterogenous course
We previously demonstrated that baseline high-resolution chest CT scan (HRCT) fibrosis visual score predicted 3-year survival in a cohort of patients newly diagnosed with IPF significantly [25]
Variables considered at baseline and follow-up assessment included an HRCT fibrosis visual score and measures of lung function, including forced vital capacity (FVC), total lung capacity (TLC), and diffusing lung capacity for carbon monoxide (DLCO)
Summary
Progression of the disease in idiopathic pulmonary fibrosis (IPF) is difficult to predict, due to its variable and heterogenous course. We sought to confirm that a simple HRCT fibrosis visual score is a reliable predictor of mortality in IPF, when longitudinally followed; and to ascertain which pulmonary functional variables best reflect clinically significant radiographic progression. One of the major challenges in the management of idiopathic pulmonary fibrosis (IPF) is predicting the clinical course of the disease in an individual patient, given its variability and heterogeneity [1]. The decline in the FVC has been the driving parameter for the approval of anti-fibrotic drugs in IPF [7, 8] This has been preferred over the use of HRCT as an endpoint, given the concerns over exposure to radiation and cost
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