Automated lung texture analysis for assessing interstitial lung disease in systemic sclerosis: Diagnostic accuracy in photon-counting-detector and conventional energy-integrating-detector CT.

This study assessed the potential of ultra-high resolution (UHR) and a 1024-matrix in photon-counting-detector CT (PCD-CT) for evaluating interstitial lung disease (ILD) in systemic sclerosis (SSc) patients. Sixty-six SSc patients who underwent ILD-CT screening on a first-generation PCD-CT were retrospectively included. Scans were performed in UHR mode at 100 kVp with two different matrix sizes (512×512 and 1024x1024) and reconstructed at slice thicknesses of 1.5 and 0.2 mm. Image noise, subjective image quality, and ILD changes (ground glass opacities and reticulations) were evaluated on a 5-point Likert-scale by two independent readers. Interreader agreement for subjective image quality ranged from fair to almost perfect (Krippendorff-Alpha: 0.258-0.862). Overall image quality was highest for 1.5 mm/1024 matrix images [(reader 1: 4(4.4), reader 2: 5(4.5)]. Image sharpness was rated significantly better in 0.2 mm images (P < .001). Regarding ILD changes, 0.2 mm slice thickness outperformed 1.5 mm slice thickness significantly (P < .001), while there was no significant difference between the two matrix sizes. A 1024-matrix size demonstrated superiority in evaluating coarse reticulations compared to 512-matrix size. UHR mode with a 0.2 mm slice thickness showed enhanced image sharpness and improved visibility of ILD changes compared to standard reconstructions. This has the potential to enable the early detection of subtle disease manifestations. With the invention of PCD-CT different reconstruction algorithms need to be evaluated for specific pathologies. In our study ILD UHR mode with 0.2 mm slice thickness showed to be beneficial in the detection of parenchymal changes in patients with scleroderma.

The alveolar concentration of exhaled nitric oxide (CA,(NO)) is increased in patients with systemic sclerosis (SSc), but whether this increase is related to the severity of interstitial lung disease (ILD) in SSc has not yet been investigated. In total, 58 SSc patients prospectively underwent pulmonary function tests (PFTs), echocardiogram and fibrosis scoring on pulmonary computed tomography (CT). Patients were divided into two groups according to the presence (or not) of ILD. Measurements of CA,(NO) were assessed in all SSc patients and compared with those obtained in 19 healthy volunteers. Relationships were sought between CA,(NO) PFTs and CT scan fibrosis scores. Overall, CA,(NO) was significantly increased in SSc patients (median (range) 6.2 (3.8-9.9) ppb) as compared with controls (2.0 (1.2-3.0) ppb). Among SSc patients, CA,(NO) was significantly higher in patients with ILD compared with patients without ILD (n = 33, 7.5 (5.2-11.9) ppb versus n = 25, 4.9 (3.1-7.0) ppb, respectively). CA,(NO) was inversely related to total lung capacity (r = -0.34) and the diffusing capacity of the lung for carbon monoxide (r = -0.37) and was directly related to CT scan fibrosis scores (r = 0.36). An increased alveolar concentration of exhaled nitric oxide could, at least in part, either reflect or contribute to the severity of lung disease and could be used to noninvasively assess the extent of interstitial lung disease in systemic sclerosis.

Incidence, prevalence and long-term progression of Goh algorithm rated interstitial lung disease in systemic sclerosis in two independent cohorts in flanders: A retrospective cohort study

Squamous cell carcinoma antigen-IgM (SCCA-IgM) is associated with interstitial lung disease in systemic sclerosis

Background:Lung transplantation (LT) shows promise for managing advanced interstitial lung disease (ILD) in systemic sclerosis (SSc) patients. However, concerns remain among surgeons due to SSc multiorgan involvement, complexities related to...

Association Between Immunosuppressive Therapy and Incident Risk of Interstitial Lung Disease in Systemic Sclerosis

The objective of this study was to identify those SSc patients with concurrent pulmonary hypertension (PH) (SSc-PH patients) and interstitial lung disease (ILD) and determine their disease severity, therapeutic approach, and survival. Consecutive SSc patients enrolled in the Australian Scleroderma Cohort Study (ASCS) who were diagnosed with pulmonary hypertension (via right-heart catherization) were included in the study. Logistic regression was used to determine the associations of ILD with PH hemodynamic parameters and therapeutic approach. Kaplan-Meier survival curves were used to estimate survival rates. Of 1883 SSc patients, 164 (8.7%) developed incident PH over a median follow-up of 4.3 (1.7-7.9) years. Of these, 43.9% had concurrent ILD at PH diagnosis (PH-ILD) and 56.1% had group 1 pulmonary arterial hypertension (PAH). Extensive ILD was present at PH diagnosis in 40.3%. Despite these distinct PH cohorts, a similar frequency of each PH cohort was treated with vasodilatory therapy at PH diagnosis, regardless of the presence or severity of ILD. The majority (87.5%) of those patients with extensive ILD and PH received upfront vasodilatory therapy at PH diagnosis, with no difference in its tolerability or therapy cessation being observed compared with the patients with group 1 PAH. Although vasodilator therapy was not associated with a survival advantage in those with extensive ILD, its use was associated with an amelioration of symptoms, and an improvement in physical function, and quality of life (QoL). Despite vasodilator therapy, survival in SSc-PH was found to be poor, with the presence of concurrent ILD being associated with worse survival. Although vasodilator therapy commenced at PH diagnosis was not shown to be associated with an improved survival in PH with extensive ILD, it appeared to be well tolerated and may improve symptoms, physical function, and QoL.

To evaluate the relationship between fecal calprotectin (FC) and interstitial lung disease (ILD) in systemic sclerosis (SSc). The study enrolled 129 outpatients with SSc. Data about disease characteristics, in particular lung involvement, were collected and FC was measured. Patients with ILD (35, 27.1%) had higher values of FC (p < 0.001). In multivariate analysis, these variables were associated with increased risk of ILD: diffuse disease subset, higher modified Rodnan skin score, longer disease duration, higher severity scores, steroid treatment, and higher FC levels, while diverticulosis was protective. ILD is independently associated with increased FC levels in SSc.

We undertook this hypothesis-generating study to identify skin transcripts correlating with severity of interstitial lung disease (ILD) in systemic sclerosis (SSc). Skin biopsy samples from 59 patients enrolled in the Genetics versus Environment in Scleroderma Outcome Study (GENISOS) cohort or an open-label imatinib study (baseline visit) were examined by global gene expression analysis using Illumina HT-12 arrays. Skin transcripts correlating with concomitantly obtained forced vital capacity (FVC) values and the modified Rodnan skin thickness score (MRSS) were identified by quantitative trait analysis. Also, immunofluorescence staining for selected transcripts was performed in affected skin and lung tissue. Plasma levels of CCL2, soluble SELP, and soluble P-selectin glycoprotein ligand 1 (sPSGL-1) were examined in all patients enrolled in the GENISOS cohort (n = 266). Eighty-two skin transcripts correlated significantly with FVC. This gene list distinguished patients with more severe ILD (FVC <70% predicted) in unsupervised hierarchical clustering analysis (P < 0.001). These genes included SELP, CCL2, and matrix metalloproteinase 3, which are involved in extravasation and adhesion of inflammatory cells. Among the FVC correlates, 8 genes (CCL2, HAPLN3, GPR4, ADCYAP1, WARS, CDC25B, PLP1, and STXBP6) also correlated with the MRSS. Immunofluorescence staining revealed that SELP and CCL2 were also overexpressed in affected skin and lung tissue from SSc patients compared to those from controls. Plasma levels of CCL2 and sPSGL-1 correlated with concomitantly obtained FVC values (r = -0.22, P = 0.001 and r = 0.17, P = 0.015, respectively). This relationship was independent of potential confounders (age, sex, ethnicity, smoking status, anti-topoisomerase I positivity, treatment with immunosuppressive agents, MRSS, disease type, and disease duration). A limited number of skin transcripts including genes involved in extravasation and adhesion of inflammatory cells correlate with severity of ILD.

Interstitial lung disease (ILD) is common in systemic sclerosis (SSc) and a major cause of death. While clinical risk factors for ILD onset at 1-year and long-term follow-up have been identified (1,2), risk stratification remains suboptimal. Pulmonary vascular volume (PVV) on HRCT is linked to both presence and severity of SSc-ILD (3). Here, we investigate the potential of radiomics in identifying patients at risk of developing ILD. We included patients from a single referral centre, with no ILD on baseline HRCT and at least one clinical and HRCT follow-up. Images 1) were assessed for presence/absence of ILD at all timepoints, 2) underwent lung texture analysis (LTA,Imbio), quantifying PVV, normal lung, lung pathologies (hyperlucency, ground-glass, reticular, honeycombing), as percentage of the lung volume, for whole lungs and upper, middle, lower zones. Univariable and multivariable prediction models were developed to identify risk factors for ILD onset, using Cox regression and generalizes estimating equation (GEE) analysis for ILD onset ever and at 1-year, the latter allowing for multiple observations of the same patient. Both models were adjusted for the established clinical risk factors (1,2). Among 248 SSc patients with no ILD at baseline HRCT, new onset of ILD occurred in 54 (22%) cases over a median follow-up of 39(24-72)months. Patients developing ILD were more frequently males, with diffuse skin involvement, anti-topoisomerase I antibodies and shorter disease duration (Table I). Additionally, patients with new onset ILD over the study period presented higher values of PVV across all lung zones at baseline HRCT (Table II). In multivariable Cox regression analysis, new ILD onset was independently predicted by PVV% from the whole lung [HR 1.054 (1.030-1.078)], as well as from each individual zone. When integrating the PVV% of each single zone with the clinic risk factors in the multivariable analysis, we observed the significant, independent impact of the lower zone PVV% [HR 1.217 (1.113–1.332)], in addition to the known risk associated with clinical features [HR 3.281 (2.145–5.021)] (Figure I-A). Focusing on 1-year ILD onset, we analysed 279 follow-up visits from 193 patients and observed 22 (11.4%) new onset ILD (Table I). On univariable GEE, we confirmed the association of PVV from the whole lung on ILD onset, with particular focus on the middle and upper zones, alongside the clinical risk factors. In the multivariable model adjusted for clinical risk factors, PVV% of the upper zone remained significantly associated with ILD onset [OR 4.127 (1.016–16.761)] (Figure I-B). PVV% from HRCT scans of SSc patients without ILD can predict new onset of ILD at both 1-year and long-term follow-up, supporting the use of radiomics in risk phenotyping. References. Petelytska L, Bonomi F, Cannistrà C, Fiorentini E, Peretti S, Torracchi S, et al. Different definitions of disease severity, progression and activity in systemic sclerosis: data from the EUSTAR database. Arthritis Rheumatol. 2023;75(Suppl 9):Abstract 1234. Hoa S, et al. Arthritis Rheumatol. 2025. Occhipinti M, Bosello S, Sisti LG, Cicchetti G, de Waure C, et al. Quantitative and semi-quantitative computed tomography analysis of interstitial lung disease in systemic sclerosis. PLoS One. 2019;14(11):e0224982. doi:10.1371/journal.pone.0224982.

BackgroundIn systemic sclerosis (SSc) it is well-documented that severe interstitial lung disease (ILD), defined as an extent of lung fibrosis >20% on high resolution computed tomography (HRCT) is strongly associated...

To develop a clinical decision rule to predict the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc; scleroderma) and to estimate the prevalence of SSc-ILD. Patient data were extracted from the Canadian Scleroderma Research Group registry. Three algorithms for the clinical decision rule were considered based on lung auscultation, chest radiography (CXR), and % predicted forced vital capacity (FVC). High-resolution computed tomography (HRCT) scans were used as the gold standard to determine the diagnostic properties of the 3 algorithms. Multiple imputation was used to impute HRCT data when missing, thereby avoiding bias due to differential referral for HRCT. This study included 1,168 patients. Of the patients with HRCT scans, 65% had evidence of ILD, compared to 26% by physical examination and 22% by CXR. The FVC of those who did not have HRCT was 8.8% greater than those who did (95% confidence interval [95% CI] 6.0-11.6%). Algorithm A, which identified the presence of ILD based on crackles on lung auscultation and/or findings on CXR, had a likelihood ratio of 3.9, compared to 3.2 for Algorithm B (which included patients with FVC <70%) and 2.2 for Algorithm C (which included patients with FVC <80%). The prevalence of ILD in the cohort was estimated to be 52% (95% CI 46-59%). We developed a simple clinical decision rule to predict SSc-ILD with good test characteristics. The prevalence of ILD in a large, unselected SSc cohort was estimated to be 52%.

To provide an overview of the role of lung ultrasound (LUS) in the assessment of interstitial lung disease (ILD) in systemic sclerosis (SSc) and to discuss the state of validation supporting its clinical relevance and application in daily clinical practice. Original articles published between January 1997 and October 2017 were included. To identify all available studies, a detailed search pertaining to the topic of review was conducted according to guidelines of the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA). A systematic search was performed in PubMed and EMBASE. The quality assessment of retrieved articles was performed according to the Oxford Center for Evidence-based Medicine. The methodological quality of the studies was assessed using the Cochrane Handbook for Systematic Reviews and the Quality Assessment of Diagnostic Accuracy Studies-2 tool. From 300 papers identified, 12 were included for the analysis. LUS passed the filter of face, content validity, and feasibility. However, there is insufficient evidence to support criterion validity, reliability, and sensitivity to change. Despite a great deal of work supporting the potential role of LUS for the assessment of ILD-SSc, much remains to be done before validating its use as an outcome measure in ILD-SSc.

PurposeThe purpose of this study was to evaluate the efficacy of radiomics derived from slice-reduced CT (srCT) scans versus full-chest CT (fcCT) for diagnosing and staging of interstitial lung disease (ILD) in systemic sclerosis (SSc), considering the potential to reduce radiation exposure. Material and methodsThe fcCT corresponded to a standard high-resolution full-chest CT whereas the srCT consisted of nine axial slices. 1451 radiomic features in two dimensions from srCT and 1375 features in three dimensions from fcCT scans were extracted from 166 SSc patients. The study included first- and second-order features from original and wavelet-transformed images. We assessed the predictive performance of quantitative CT (qCT)-based logistic regression (LR) models relying on preselected features and machine learning workflows involving LR and extra-trees classifiers with data-driven feature selection. The area under the receiver operating characteristic curve (AUC) was used to estimate model performance. ResultsThe best models for diagnosis and staging ILD achieved AUC=0.85±0.08 and AUC=0.82±0.08 with srCT, and AUC=0.83±0.06 and AUC=0.76±0.08 with fcCT, respectively. srCT-based models showed slightly superior performance over fcCT-based models, particularly in 2D-radiomic analyses when interpolation resolution closely matched the original in-plane resolution. For diagnosis, the LR outperformed qCT-models, whereas for staging, the best results were obtained with a qCT-based model. ConclusionsRadiomics from srCT is an effective and preferable alternative to fcCT for diagnosing and staging SSc-ILD. This approach not only enhances predictive accuracy but also minimizes radiation exposure risks, offering a promising avenue for improved treatment decision support in SSc-ILD management.

ObjectivesEarly diagnosis of interstitial lung disease (ILD), currently the main cause of death in systemic sclerosis (SSc), is needed. The gold standard is high-resolution CT (HRCT) of the chest, but...