Abstract
ObjectivesTo evaluate interstitial lung disease associated with systemic sclerosis (SSc-ILD) and its changes during treatment by using quantitative analysis (QA) compared to semi-quantitative analysis (semiQA) of chest computed tomography (CT) scans. To assess the prognostic value of QA in predicting functional changes.Materials and methodsWe retrospectively selected 35 consecutive patients with SSc-ILD with complete pulmonary functional evaluation, Doppler-echocardiography, immunological tests, and chest CT scan at both baseline and follow-up after immunosuppressive therapy. CT images were analyzed by two chest radiologists for semiQA and by a computational platform for texture analysis of ILD patterns (CALIPER) for QA. Concordance between semiQA and QA was tested. Traction bronchiectasis severity was scored. Analysis of ROC curves was performed.ResultsSeventy CT scans were analyzed and QA failed in 4/70 scans. Thus, the final population included 31/35 patients (51.3±12.1 years). QA had a weak-to-good concordance with semiQA (ICC reticular:0.275; ICC ground-glass:0.667) and QA correlated better than semiQA (r = -0.3 to -0.74 vs r = -0.3 to -0.4) with functional parameters. Both methods correlated with traction bronchiectases score and pulmonary artery diameter at CT. A pulmonary artery diameter ≥29mm distinguished patients with lower lung volumes and ILD extent greater than 39% (p<0.001). Changes in QA patterns during treatment were not accurate (AUC: 0.50 to 0.70; p>0.05) in predicting disease progression as assessed by functional parameters, whereas variation in total lung volume at QA accurately predicted changes in the composite functional respiratory endpoint with FVC% and DLco% (AUC = 0.74; 95%CI: 0.54 to 0.93; p = 0.03).ConclusionsPulmonary QA of CT images can objectively quantify specific patterns of ILD changes during treatment in patients with SSc-ILD. Changes in QA patterns do not correlate with functional changes, but variation in total lung volume at QA accurately predicted changes in the composite functional respiratory endpoint with FVC% and DLco%. Pulmonary artery diameter at CT reflects the interstitial involvement, identifying patients with more severe prognosis.
Highlights
Systemic sclerosis (SSc) is a connective tissue disease with a variety of clinical presentations, ranging from involvement restricted to the skin and peripheral angiopathy to rapidly progressive forms affecting internal organs
Seventy computed tomography (CT) scans were analyzed and quantitative analysis (QA) failed in 4/70 scans
QA had a weak-to-good concordance with semi-quantitative analysis (semiQA) (ICC reticular:0.275; Intraclass correlation coefficient (ICC) ground-glass:0.667) and QA correlated better than semiQA (r = -0.3 to -0.74 vs r = -0.3 to -0.4) with functional parameters. Both methods correlated with traction bronchiectases score and pulmonary artery diameter at CT
Summary
Systemic sclerosis (SSc) is a connective tissue disease with a variety of clinical presentations, ranging from involvement restricted to the skin and peripheral angiopathy to rapidly progressive forms affecting internal organs. Goh et al proposed a composite functional end-point with FVC and DLco for predicting survival in patients with SSc-ILD [3] and a decade ago the same group proposed a radiological score together with FVC to distinguish “limited” versus “extensive” disease [4, 5]. This approach is restricted to evaluate patient prognosis and is limited to a semi-quantitative analysis (semiQA) of chest computed tomography (CT) images, susceptible to observer bias, inter-reader, and intra-reader variability [6]. These limits represent a critical obstacle especially when evaluating the response to therapy in daily practice, which is the real unmet need in the assessment of SSc population [7, 8]
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