Abstract

BackgroundThe 2011 idiopathic pulmonary fibrosis (IPF) guidelines are based on the diagnosis of IPF using only high-resolution computed tomography (HRCT). However, few studies have thus far reviewed the usefulness of the HRCT scoring system based on the grading scale provided in the guidelines. We retrospectively studied 98 patients with respect to assess the prognostic value of changes in HRCT findings using a new HRCT scoring system based on the grading scale published in the guidelines.MethodsConsecutive patients with IPF who were diagnosed using HRCT alone between January 2008 and January 2012 were evaluated. HRCT examinations and pulmonary function tests were performed at six-month intervals for the first year after diagnosis. The HRCT findings were evaluated using the new HRCT scoring system (HRCT fibrosis score) over time. The findings and survival rates were analyzed using a Kaplan-Meier analysis.ResultsThe HRCT fibrosis scores at six and 12 months after diagnosis were significantly increased compared to those observed at the initial diagnosis (p < 0.001). The patients with an elevated HRCT fibrosis score at six months based on a receiver operating characteristic (ROC) curves analysis had a poor prognosis (log-rank, hazard ratio [HR] 2.435, 95% CI 1.196-4.962; p = 0.0142). Furthermore, among the patients without marked changes in %FVC, those with an elevated score above the cut-off value had a poor prognosis (HR 2.192, 95% CI 1.003-4.791; p = 0.0491).ConclusionsOur data demonstrate that the HRCT scoring system based on the grading scale is useful for predicting the clinical outcomes of IPF and identifying patients with an adverse prognosis when used in combination with spirometry.

Highlights

  • Idiopathic pulmonary fibrosis (IPF) is a progressive and generally fatal disease

  • These results indicate that the new high-resolution computed tomography (HRCT) scoring system is an appropriate and subjective method for monitoring idiopathic pulmonary fibrosis (IPF) patients

  • The HRCT scoring system-based grading scale is useful for inferring the prognosis in patients with IPF and, in particular, facilitates the extraction of patients with a poor prognosis who cannot be identified using only pulmonary function examinations

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Summary

Introduction

The 2011 IPF guidelines provide updated and simplified IPF diagnostic criteria proposed by the ATS/ERS/JRS/ ALAT [6]. This may result in HRCT scanning playing a the 2011 IPF guidelines state that disease progression manifests as worsening respiratory symptoms, worsening pulmonary functions, the presence of progressive fibrosis on HRCT and acute respiratory decline and that monitoring patients with IPF is necessary to detect the development of the disease and proactively identify those with progressive disease. We retrospectively studied 98 patients with respect to assess the prognostic value of changes in HRCT findings using a new HRCT scoring system based on the grading scale published in the guidelines

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