Abstract
ObjectivesThis study was designed (a) to evaluate an improved quantitative lung fibrosis score based on a computer-aided diagnosis (CaM) system in patients with systemic sclerosis (SSc),—related interstitial lung disease (SSc-ILD), (b) to investigate the relationship between physiologic parameters (forced vital capacity [FVC] and single-breath diffusing capacity for carbon monoxide [DLCO]), patient-centred measures of dyspnea and functional disability and CaM and visual reader-based (CoVR) methods, and (c) to identify potential surrogate measures from quantitative and visual HRCT measurement.Methods126 patients with SSc underwent chest radiography, HRCT and PFTs. The following patient-centred measures were obtained: modified Borg Dyspnea Index (Borg score), VAS for breathing, and Health Assessment Questionnaire-Disability Index (HAQ-DI). HRCT abnormalities were scored according to the conventional visual reader-based score (CoVR) and by a CaM. The relationships among the HRCT scores, physiologic parameters (FVC and DLCO, % predicted) results and patient-centred measures, were calculated using linear regression analysis and Pearson’s correlation. Multivariate regression models were performed to identify the predictor variables on severity of pulmonary fibrosis.ResultsSubjects with limited cutaneous SSc had lower HAQ-DI scores than subjects with diffuse cutaneous SSc (p <0.001). CaM and CoVR scores were similar in the 2 groups. In univariate analysis, a strong correlation between CaM and CoVR was observed (p <0.0001). In multivariate analysis the CaM and CoVR scores were predicted by DLco, FVC, Borg score and HAQ-DI. Age, sex, disease duration, anti-topoisomerase antibodies and mRSS were not significantly associated with severity of pulmonary fibrosis on CaM- and CoVR methods.ConclusionsAlthough a close correlation between CaM score results and CoVR total score was found, CaM analysis showed a more significant correlation with DLco (more so than the FVC), patient-centred measures of perceived dyspnea and functional disability. Computer-aided tomographic analysis is computationally efficient, and in combination with physiologic and patient-centred measures, it could allow a means for accurately assessing and monitoring the disease progression or response to therapy.
Highlights
Systemic sclerosis (SSc) is a heterogeneous complex of diseases characterized by multiorgan involvement, endothelial dysfunction, excessive collagen production and immune system abnormalities [1]
computer-aided method (CaM) and conventional visual reader-based score (CoVR) scores were similar in the 2 groups
In multivariate analysis the CaM and CoVR scores were predicted by DLco, forced vital capacity (FVC), Borg score and Health Assessment Questionnaire-Disability Index (HAQ-DI)
Summary
Systemic sclerosis (SSc) is a heterogeneous complex of diseases characterized by multiorgan involvement, endothelial dysfunction, excessive collagen production and immune system abnormalities [1]. Interstitial lung disease (ILD) is a devastating and significant cause of death in patients with SSc. In early autopsy studies, up to 100% of patients were found to have parenchymal involvement [1]. Parenchymal lung involvement often appears early after the diagnosis of SSc, with 25% of patients developing clinically significant lung disease within 3 years as defined by physiological, radiographic or bronchoalveolar lavage abnormalities [2]. Baseline diffusing capacity for carbon monoxide (DLco) and forced vital capacity (FVC) levels have traditionally been used as measures of disease severity and reductions in both parameters have been associated with increased mortality in the SSc-ILD
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