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P.295 EULAR systemic sclerosis impact of disease (ScleroID): a tool to assess patient-reported disease burden and organ damage: evidence from an Italian multicenter cross-sectional and longitudinal study

jsrd · 2026-06-05 · canonical JSON source

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Introduction Systemic sclerosis (SSc) is a progressive, heterogeneous disease with major morbidity and mortality. The recently developed EULAR Systemic Sclerosis Impact of Disease (ScleroID) questionnaire was designed to capture the disease burden. 1Objectives: To assess the correlation of ScleroID with clinical features in a multicentre Italian cohort and its ability to reflect longitudinal changes in patient-reported disease status.Material and Methods The Italian ScleroID was administered to consecutive SSc patients (2013 ACR/EULAR criteria) attending five referral centres. Comparators were Health Assessment Questionnaire-Disability Index (HAQ-DI), Scleroderma Clinical Trials Consortium Damage Index (SCTC-DI), and age-modified Charlson Comorbidity Index (CCI). A subgroup was reassessed after follow-up (T1). δScleroID was calculated as T1-T0 difference. Disease progression was evaluated by predefined criteria (table 1).Results Among 714 patients ( table 2s), median baseline ScleroID was 3,5 (IQR 1,6–5,1), highest in fatigue [5 (2–7)], Raynaud’s, and hand function [4 (1–7)]. ScleroID correlated positively with HAQ-DI (rs=0,645), SCTC-DI (rs=0,355), CCI (rs=0,123), and disease duration (rs=0,107), and negatively with pFVC% (rs=-0,171) and pDLCO% (rs=-0,216) (all p<0,01; figure 1a).Higher scores were seen in patients with longer disease duration and organ involvement (figure 1b). At multivariate analysis, SCTC-DI (OR 1,1, 95% CI 1,02–1,17, p=0,018) was independently associated with ScleroID.A total of 179 patients were longitudinally evaluated after a median of 11 (8–13) months: median ScleroID at T1 was 3,7 (2,4–5,3), strongly correlated with baseline (rs=0,751). Patients self-reported stability in 118 cases (66%), improvement in 18 (10%), and worsening in 43 (24%). Worsened patients showed ScleroID increase +0,7 (0,1–1,5), while stable or improved patients had stable 0,0 (-0,8 to 0,9) or reduced -1,2 (-2,0 to -0,2) scores. Differences in δScleroID scores in worsened vs improved patients (p<0,001) and worsened vs stable patients (p=0,002) were significant. During follow-up, 14 patients (8%) met progression criteria; their ScleroID change +0,8 (-0,1 to 1,4) was not significantly different from non-progressors 0,0 (-0,8 to 0,97), p=0,315.Conclusions In this large Italian cohort, ScleroID correlated with disability, damage, and organ involvement, with SCTC-DI independently predicting higher disease burden. Longitudinal data confirmed its ability to capture self-reported improvement, stability, or worsening. However, correlation with predefined progression criteria was limited, indicating the need of further studies.Reference Becker MO, et al. Ann Rheum Dis. 2022.The Authors would like to thank the Italian association of SSc patients ‘GILS’ (Gruppo Italiano Lotta Sclerodermia) for kindly supporting the project.Abstract P.295 Table 1Predefined disease progression criteriaAbstract P.295 Figure 1Correlation of the ScleroID global score with outcome measures in SSc and comparison across different subgroups based on clinical-demographic features, assessed through univariate analysis. 1a Correlation of SclerolD with outcome measures in SSc; 1b Comparison of SclerolD global score in different subgroups by univariate analysisAbstract P.295 Table 2Clinical characteristics of Systemic Sclerosis patients at baseline (Ta) and at follow-up visits (T1) Anti-Topo-1, anti-topoisomerase-1 antibodies; CCI, Charlson Comorbidity Index; dcSSC, diffuse cutaneous disease; EF, ejection fraction; HAQ-DI, Health Assessment Questionnaire-Disability Index; HRCT, high resolution CT; ILD, interstitial lung disease; mRSS, modified RHC, right-heart catheterization; Rodnan Skin Score; PAH, Pulmonary arterial hypertension; SCTC-DI, Scleroderma Clinical Trials Consortium Damage Index; yrs, years. Continuous variables: median (IQR); categorical variables: number/number of available data (%)