POS0823 MULTI-CENTRE VALIDATION OF THE PASTUL QUESTIONNAIRE FOR SELF-ASSESSMENT OF SKIN SEVERITY IN SYSTEMIC SCLEROSIS
Authors: J. Spierings, V. H. Ong, P. M. J. Welsing, M. Hughes, J. D. Pauling, F. Del Galdo, A. L. Herrick, C. P. Denton
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> The PASTUL (Patient self-Assessment of Skin Thickness in Upper Limb) questionnaire was developed during the COVID pandemic to allow self-assessment of skin remotely in systemic sclerosis (SSc) [1]. <h3>Objectives:</h3> The aim of this study was to validate PASTUL, evaluate responsiveness and assess impact of skin on health related quality of life (HRQoL). <h3>Methods:</h3> SSc patients were included in four UK centres. The PASTUL questionnaire specifies a grading of skin (normal, mild, moderate, severe thickness) at eight sites corresponding to the modified Rodnan Skin Score (mRSS) with maximum score assigned to each site. Validity was assessed by comparing PASTUL scores with mRSS assessed by trained rheumatologists. HRQoL (EQ5D5L and Leeds SSc HRQoL) and Scleroderma Skin Patient reported Outcome (SSPRO) were collected for construct validity, using Pearson's correlation coefficient (0-0.19 = negligible, 0.2-0.39 = weak, 0.4-0.59 = moderate, 0.6-0.79 = strong, 0.8-1.0 = very strong). Test-retest reliability was estimated 2 weeks after baseline and using intraclass correlation coefficient (ICC). Patients were followed for 12 months to assess responsiveness of PASTUL to change in mRSS. <h3>Results:</h3> 200 patients were included, mean age 56.0 years (SD 14.0), 79% female (n=142), 90 (53%) had limited cutaneous SSc (lcSSc) and 79 (47%) diffuse cutaneous SSc (dcSSc). At baseline mean disease duration was 12.0 years (SD 9.7), mRSS was 8.2 (SD 8.0) and PASTUL score was 9.3 (SD 6.1). At baseline, 6 and 12 months follow-up, PASTUL and mRSS were strongly correlated (r=0.63, r=0.76, r=0.72, p&lt;0.001, respectively). Test-retest reliability, assessed in 84 patients, was very good (ICC of 0.82 (95% CI 0.74-0.88), p&lt;0.001). Correlation between PASTUL and mRSS was moderate in lcSSc and strong in dcSSc (r=0.51 p&lt;0.001, and 0.66 p&lt;0.001, respectively). In patients with disease duration &lt; 4 years, correlation between PASTUL and mRSS was strong (r=0.76, p&lt;0.001) and PASTUL was strongly correlated with HRQoL. SSPRO scores were not correlated with skin thickness (Table 1). Presence of digital ulcers did not influence the correlation between mRSS and PASTUL. PASTUL was found to be comprehensible (mean score 4.3 out of 5 (SD 1.0)), and achievable 4.0 (SD 0.9). The mean time to complete the self-assessment was 5.0 minutes (SD 3.7). At 6 months 41% of patients had a change in mRSS &gt; 2, at 12 months this was 36%. Responsiveness of PASTUL at 6 and 12 months was r=0.17 and r=0.19. <h3>Conclusion:</h3> PASTUL is a valid and feasible outcome tool that adds a self-reported measure of skin severity to impact assessments like SSPRO. Skin thickening is associated with HRQoL in patients with early disease. PASTUL might be promising for clinical outcome assessment of skin thickening over time but needs further assessment in a larger cohort <h3>REFERENCES:</h3> [1] <i>Spierings J, Ong, VH &amp; Denton, CP. (2021). PASTUL questionnaire: a tool for self-assessment of scleroderma skin during the COVID-19 pandemic. Ann Rheum Diseases, 80, 819 - 820.</i> <h3>Acknowledgements:</h3> This work was supported by a grant from Scleroderma and Raynaud's United Kingdom (SRUK). <h3>Disclosure of Interests:</h3> Julia Spierings has received research support from Boehringer Ingelheim (outside the submitted work), Voon H Ong: None declared, Paco M.J. Welsing: None declared, Michael Hughes: None declared, John D Pauling has received consultancy fees from Astra Zeneca, Boehringer Ingelheim, Janssen, Permeatus Inc, Sojournix Pharma and IsoMab; all outside the submitted work , Francesco Del Galdo has received research funding and/or consulting fees from GlaxoSmithKline, AstraZeneca, Boehringer Ingelheim, Actelion, Capella Bioscience, Chemomab, Kymab, Actelion, iqvia, and Mitsubishi Tanabe; all outside the submitted work, Ariane L. Herrick has received speaker fees from Janssen, consultancy fees from Arena, Boehringer Ingelheim, Camurus, CSL Behring Galderma and Gesynta Pharma., financial research grants from Gesynta Pharma; all outside the submitted work, Christopher P Denton has received speaker fees from Janssen and Boehringer Ingelheim, consultancy fees from Janssen, GlaxoSmithKline, Boehringer Ingelheim, Roche, CSL Behring, Corbus, Acceleron, Horizon, Arxx Therapeutics, Lilly, Novartis, Certa, research grants from GSK, Abbvie, Horizon, Arxx, Servier; all outside the submitted work.