POS0438 DISEASE SUBSET AND DURATION ARE ASSOCIATED WITH INCREASED PATIENT-REPORTED DISEASE BURDEN, MEASURED BY THE EULAR SYSTEMIC ScleroID IMPACT OF DISEASE (SCLEROID) QUESTIONNAIRE, IN AN ITALIAN MONOCENTRIC COHORT (2024)

Abstract

Background: Systemic sclerosis (SSc) is a chronic connective tissue disease characterized by patient-to-patient variability in clinical manifestations, internal organ involvement and outcome. The EULAR Systemic Sclerosis Impact of Disease (ScleroID) questionnaire[1] is a recently validated Patient-Reported Outcome Measure, aiming to capture the patients’ personal perception regarding their disease severity, analyzing ten health dimensions able to cover the global disease burden.

Objectives: To evaluate the correlations of ScleroID questionnaire with clinical and demographic features in SSc patients from an Italian tertiary care center.

Methods: An Italian version of ScleroID questionnaire was administered to SSc patients (2013 ACR/EULAR criteria) attending consecutively our Scleroderma Clinic (November 2022-December 2023). ScleroID was calculated as a composite score of ten health dimensions, each one assessed with Numeric Rating Scales (NRS) from 0 to 10; then NRS score of each dimension was multiplied by the specific weight for this item and the weighted scores were summed up. Health Assessment Questionnaire-Disability Index (HAQ-DI) was used as comparator. Comorbidities were measured by age-modified Charlson Comorbidity Index (CCI). The study included also a longitudinal arm who enrolled consecutive patients who completed a second visit 6-12 months after baseline (May-December 2023). Data are presented as median (lower quartile; upper quartile). SSc subgroups were compared using the Mann–Whitney U test and correlations were made by Spearman coefficient (r_s). Logistic regression model (considering age, gender, disease subset, disease duration and CCI) was used to individuate variables independently associated with the ScleroID score.

Results: After exclusion of 10 patients (for language barrier or cognitive disorders) 212 of 222 (95%) consecutive SSc patients were enrolled in this study and their clinical features are detailed in Table 1. The median overall ScleroID score was 3.7 (1.9-5.0). The health dimensions with the highest score were: fatigue [5 (2-7)], Raynaud’s phenomenon [5 (2-6)] and hand function [4 (2-7)]. A good construct validity was shown when correlating ScleroID with HAQ-DI (r_s: 0.48, p<0.0001). Evaluating correlations of the ScleroID score with other parameters, positive correlations were found with CCI (r_s: 0.19, p=0.01) and disease duration (r_s: 0.18, p=0.01), and negative correlations with FVC, % (r_s: -0.19, p=0.01) and DLCO, % (r_s: -0.15, p=0.04). Comparisons of the ScleroID global score in different SSc subgroups by univariate analysis are shown in Table 2. Higher ScleroID score were observed in patients with longer disease duration and higher cutaneous and organ involvement. In multivariate logistic regression analysis, disease duration (OR 1.03, 95% CI 1.0 to 1.1, p=0.01) and diffuse cutaneous involvement (OR 3.4, 95% CI 1.4 to 7.8, p=0.006) were independently associated with higher ScleroID score, whereas age, gender and CCI were not. Fifty-three patients were longitudinally evaluated after a median time of 8 (7-10) months (Table 1). The median overall ScleroID score at follow-up was 3.6 (2.6-5.7); and was highly correlated with baseline (r_s: 0.79, p<0.0001; ranging 0.48–0.69 for the individual items). Twenty-three patients reported their disease status as not stable: 9 (17%) improved, 14 (26%) worsened.

Conclusion: In our cohort, fatigue, Raynaud’s phenomenon and hand function were the main patient-reported drivers of disease burden, confirming previous reports[1-2]; the tool revealed a good reliability and a good correlation with HAQ-DI. Disease subset and duration, according to multivariate analysis, were independently associated with an increased patient-reported disease burden.

Acknowledgements: The Authors would like to thank the Italian association of SSc patients ‘GILS’ (Gruppo Italiano Lotta Sclerodermia) for kindly supporting the project.

Disclosure of Interests: None declared.