AB1174 VALIDITY OF THE ASSESSMENT OF SYSTEMIC SCLEROSIS – ASSOCIATED RAYNAUD PHENOMENON (ASRAP) QUESTIONNAIRE
Authors: K. A. Madathanapalli, K. Shelton, A. Makhijani, A. Memon, N. Page, B. Gunes, W. Odell, S. E. Kujawski, S. Perez, J. Pauling, R. Domsic, L. Yu, F. Wilson, M. Hinchcliff
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> There is a need to establish valid methods to quantify the burden of systemic sclerosis- associated Raynaud phenomenon (SSc-RP) from the patient perspective. The newly developed Assessment of Systemic Sclerosis – Associated Raynaud Phenomenon (ASRAP) Questionnaire was developed with SSc patient input to capture the impact of SSc-RP across various physical and emotional domains. <h3>Objectives:</h3> We tested the construct validity of the ASRAP by investigating associations between ASRAP scores and clinical characteristics including the degree of microvascular damage assessed by nailfold capillaroscopy (NFC). <h3>Methods:</h3> Healthy controls (HC) (n=15) and patients who fulfilled ACR/EULAR 2013 SSc Classification Criteria with RP symptoms (n=75) were prospectively recruited from December 2021 to November 2023. Following a clinic visit, patients completed the ASRAP Questionnaire (range 20-80, with higher scores indicating worse symptoms) and underwent NFC of the bilateral fingers, excluding the thumbs, performed using the Dinolite™ (AF4515-N2UT) microscope. Two blinded assessors classified NFC as having or lacking the "scleroderma pattern" as previously described. Dinolite Software was used to analyze the distal NFC row to calculate capillary density (per linear mm) and a semi-quantitative score (0 = no changes, 1 = ≤ 33%, 2 = 34–66%, 3 = ≥ 67%) based on the proportion of capillary alterations/ reductions consistent with scleroderma pattern (Figure 1). Demographic and SSc disease-specific data were collected. Inter-rater reliability of NFC scoring was evaluated using Kappa (Stata, v.17.0, College Station, TX). Descriptive statistics, univariable and multivariable linear regression, and Spearman's Rho were used to investigate associations between NFC semi-quantitative score, ASRAP score, and clinical characteristics. <h3>Results:</h3> Patients vs. HC were predominantly white (n=58, 77% vs. n=11, 73%) women (n=65, 87% vs. n=14, 93%) with a median (IQR) age (years) of [60 (50-67) vs. 53 (46-58)], respectively. Among SSc participants, the majority had limited cutaneous SSc (n=58, 78%) and anticentromere antibody was the most prevalent SSc specific antibody (n=24, 33%). Mean (SD) time in years since onset of RP was 18.1±13.4 and 12 patients (16%) reported digital ulcers in the past. The scleroderma NFC pattern was more prevalent in SSc patients vs. HC (83% vs. 7%, p&lt;0.001) with moderate inter-rater reliability (Kappa=0.51). Mean (SD) capillary density was lower in SSc vs. HC (5.2±1.4 vs. 8.5±1.4). In SSc, the mean (SD) ASRAP score was 44.55 ±10.5 and correlated significantly with mean (SD) semi-quantitative score (1.46 ± 1.19) (rho 0.24, p=0.03). Limiting analysis to patients studied in winter, the correlation was stronger (rho 0.35, p=0.03) (Figure 2a &amp; b). In univariable linear regression analyses of patient factors and ASRAP score: female sex (β= 11.75, p=0.001), digital ulcer history (β= 7.34, p=0.03), and "other" vs. "white" race (β= 11.4, p=0.005) were significantly associated with higher ASRAP scores. A multivariable linear regression model including patient factors (age, sex, race, SSc subtype, time since RP onset, digital ulcer history, NFC semi quantitative score, current RP treatment) was generated to predict ASRAP scores (R<sup>2</sup> =0.53) (Figure 2c). This model was also applied solely to patients recruited in winter months (R<sup>2</sup> =0.79). Controlling other variables: i) women had higher ASRAP scores than men (+14.16 overall, +17.86 in winter), ii) patients who identified as "other" race had higher ASRAP scores compared to those who identified as "white" (+9.32 overall, no significant difference in winter), iii) association between semi-quantitative score and ASRAP was no longer significant (both overall, and in winter). <h3>Conclusion:</h3> NFC abnormalities as assessed by Dinolite™ are significantly associated with ASRAP scores, particularly in winter. Sex and race appear to influence ASRAP scores. Future work includes a longitudinal study to assess sensitivity to change of ASRAP and NFC.REFERENCES: NIL. <h3>Acknowledgements:</h3> Research was funded by Resident Research Preceptorship awarded to Dr. Madathanapalli by the Rheumatology Research Foundation <h3>Disclosure of Interests:</h3> Krishnasai Abhishek Madathanapalli: None declared, Kyra Shelton: None declared, Amrita Makhijani: None declared, Areeka Memon: None declared, Nicolas Page: None declared, Baran Gunes: None declared, William Odell: None declared, Sophia Esme Kujawski: None declared, Stephanie Perez: None declared, John Pauling Janssen, Janssen, Astra Zeneca, Sojournix Pharma, Isomab, Permeatus Inc, Boehringer Ingelheim, Robyn Domsic Aisa Pharmaceuticals Inc and Astra- Zeneca, Lan Yu: None declared, Francis Wilson Amgen, Vifor Pharma, and Astrazeneca, Monqiue Hinchcliff Boehringer Ingelheim, Boehringer Ingelheim and Kadmon.