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Introduction The prevalence of sicca and/or objective signs of Sjogren’s syndrome (SjS) in systemic sclerosis (SSc) patients varies from 1/3 and higher. 1 2 The differences between patients with SSc+SjS vs SSc and no SjS with respect to manifestations, functional status and quality of life remain unclear. Our study aimed to characterize the clinical phenotype of SSc patients with concomitant SjS.Material and Methods Data were obtained from the Canadian Scleroderma Research Group registry; an observational multicentre database of 1293 SSc patients (87% female; mean±SD age 55.2±12.1 yrs; disease duration 10.4±9.4 yrs) who were categorized according to the presence or absence of SjS. SjS was defined as giving 3 or more positive answers to 6 sicca symptom questions OR 2 or more positive answers AND 1 or more signs of xerostomia/xerophthalmia. Skin was assessed using modified Rodnan Skin Score (mRSS), overall disease severity - by the summed Medgser disease severity score (DSS), patient and physician global assessments, HAQ-DI, Scleroderma HAQ (SHAQ) and SF-36v2. Sub-group analyses were done in limited and diffuse cutaneous SSc. Bivariate, ANOVA, non-parametric, and chi-squared tests and multivariate regression analysis were used.Results We identified 386 SSc+SjS patients (30%) who were more likely to be female (93% vs 84%, p<0.001) with a longer disease duration (12.4±9.9 vs 9.5±9.0 years, p<0.001) compared to no SjS. SjS-SSc patients showed a more severe disease (DSS total 9.1±4.1 vs 8.3±3.8, p=0.005; patient global assessment of health 4.2±2.4 vs 3.0±2.4, p<0.001; physician-reported disease severity 2.8±2.0 vs 2.7±2.3, p=0.025), specifically lung and gastrointestinal involvement ( table 1). They also reported worse functional status (p<0.001 for all HAQ-DI, SHAQ ulcers/Raynaud’s phenomenon/breathing), but there was no difference in SF-36 summary scores and separate domains between patients with and without SjS. A higher frequency of ACA-positivity (42% vs 31%, p<0.001) and less severe skin involvement (mRSS 8.6±9.2 vs 10.0±9.6, p<0.008) were found in SSc-SjS patients. Sub-group analysis showed more severe disease and worse HAQ/SHAQ in SjS-SSc patients, regardless of SSc subtype (table 1). SjS remained a significant independent predictor for disease severity after controlling for confounding by age, sex, disease duration, mRSS, disease activity and SSc-specific autoantibodies in multivariate regression analysis (p<0.001).Conclusions Despite a prevalent ACA+ subtype with less skin involvement, SSc-SjS patients revealed a more severe SSc with predominant lung disease affecting significantly their functional status.References Couderc M, et al. Ann Rheum Dis. 2020;79:e137.Kobak S, et al. Int J Rheum Dis. 2013;16:88–92.Abstract P.346 Table 1Demographics and disease characteristics in patients with systemic sclerosis with and without concomitant Sjogren’s syndrome