Validation of a semi-quantitative method to assess interstitial lung disease severity and progression in systemic sclerosis by standard and low-dose HRCT scans.

Abstract

Background: While the presence of distinct imaging abnormalities by high-resolution CT (HRCT) defines interstitial lung disease (ILD), there is a relative lack of validated methods to quantify these abnormalities in clinical practice, limiting ILD severity and progression assessments. We aimed to validate a semi-quantitative method for lung fibrosis assessment in patients with systemic sclerosis associated ILD (SSc-ILD) by standard and low-dose HRCT, considering lung structure and function as integral components of ILD evaluation.

Methods: SSc patients from Oslo and Zurich with HRCT images, pulmonary function tests, including forced vital capacity (FVC), diffusing capacity for carbon monoxide (DLCO) and the 6-minute walk test with oxygen (O₂) desaturation were enrolled. We validated the semi-quantitative fibrosis extent method by HRCT using criteria for content and construct validity, discrimination, sensitivity to change and feasibility, as well as inter- and intra-rater variability.

Results: 65 SSc patients from Zurich and 90 from Oslo were included. Significant correlations were observed between the extent of fibrosis on HRCT and FVC (r=-0.517, p<0.001), DLCO (r=-0.400, p<0.001) and O₂ desaturation (r=-0.500, p<0.001), indicating content, construct and criterion validity. Discrimination and sensitivity to change assessments showed moderate correlation with DLCO (r=-0.377, p=0.003) but not with FVC or O₂ desaturation. Inter- and intra-rater variability demonstrated excellent reliability (κ=0.891 and κ=0.996, respectively), with HRCT quantification averaging 9-15 min, indicating high feasibility.

Conclusion: This study confirms that semi-quantitative fibrosis assessment of HRCT for SSc-ILD meets most validation criteria, supporting its use in clinical practice and showing additive value of structural to functional ILD assessment.