Objective. To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjögren's syndrome (pSS). Methods. By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression. Results. There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of <90% in patients diagnosed at the youngest ages and >95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R 2 0.87) and the frequency of abnormal oral tests (adjusted R 2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%.

Objective To assess the prevalence of parotid gland swelling (PGS) and its association with features of SS and other causes of sialadenosis in a Latin-American cohort of primary SS. Methods We included 668 patients from Argentina, Brazil, Mexico and Paraguay. We retrospectively registered demographics, disease duration, oral/ocular symptoms, serology and scored the basal ESSDAI. We defined PGS as a recurrent or persistent increase of volume of any parotid glands during adulthood (self-reported and/or physical examination). We registered the presence of diabetes mellitus, dyslipidaemia, body mass index and alcohol consumption. We used logistic regression analysis reporting odds ratio (OR) and 95% CI. Results PGS was present in 242 patients (36.2%): 78 previous to SS diagnosis, 86 concomitantly, 73 during follow-up and five unknown. At the multivariate analysis, PGS was associated with RF (OR 2.47, 95% CI: 1.1, 6.5, P = 0.0001), basal articular ESSDAI domain (OR 1.63, 95% CI: 1.01, 2.6, P = 0.04) and alcohol consumption (OR 2.42, 95% CI: 1.41, 4-15). Patients with PGS during the follow-up had a higher prevalence of alcohol consumption (45.3%) compared with the remaining PGS cases (26.8%; OR 2.41 95% CI: 1.2, 4.7), or patients without parotid gland swelling (15.6%; OR 3.8 95% CI: 1.7, 8.2) in all the adjusted models. Conclusion PGS generally precedes or presents concomitantly with SS diagnosis, and is related to RF and articular activity. Alcohol consumption is an additional factor in PGS, especially during follow-up. The meaning of this last finding as well as its prognostic implications remains to be elucidated and deserves further evaluation in prospective studies.

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Background:Immune Mediated Inflammatory Diseases (IMIDs) are complex diseases that are believed to have a strong interaction between the genome and the environment as part of their aetiology. In studies using the candidate gene strategy, genetic variation in a gene where functionality has been associated with the pathophysiology of the disease under study is being analyzed. In the last decade, polymorphisms of the vitamin D receptor (VDR) and VDBP genes have been more emphatically studied in IMIDs in different populations, but the results reported have not yet been conclusive.Objectives:To identify an association between vitamin D receptor (VDR) and vitamin D-binding protein (VDBP) gene polymorphisms, and IMIDs in Paraguayan patients.Methods:Association study of VDR (SNPs rs731236, rs7975232, rs2228570) and VDBP (rs4588) gene polymorphisms with susceptibility to IMIDs in Paraguayan population. A total of 399 patients with IMIDs (i.e. Systemic Lupus Erythematosus (SLE), Scleroderma (ES), Rheumatoid Arthritis (RA), and Cutaneous Psoriasis (CPS) and 100 hypernormal controls (HC) from the same population were included in this study. Genotyping was performed using Taqman real-time PCR-based technology (Life Technologies, USA). Statistical analysis was performed using Rv3.0.1 statistical language software (www.R-project.org). A p value ≤ 0.05 was used for statistical significance.Results:A total of 399 individuals, 100 controls and 299 patients (99 RA, 100 SLE, 50 ES, and 50 PSO) were included. Seventy-six percent were female and 24% were male. The mean age was 43.7±14 years. Four SNPs were genotyped: rs731236, rs7975232, rs2228570, rs4588. The HWE test was not statistically significant for any of the 4 SNPs considered (P>0.05), confirming the quality of genotyping and the absence of technical bias. (Table 1).Table 1.Genotyping of SNPs of the VDR and VDBP gene in Paraguayan population with IMIDs.SNPIMIDMinor AlleleMajor AlleleMAFControlMAFCaseORIC.LIC.Hp allelicP.Geneticrs731236SLEGA0.50.40.640.420.970.0350.08rs731236RAGA0.50.410.690.461.050.0710.12rs731236SSGA0.50.420.710.421.180.180.37rs731236CPSGA0.50.380.60.361.010.0490.042rs2228570SLEAG0.360.381.140.741.740.60.45rs2228570RAAG0.360.310.830.531.280.40.56rs2228570SSAG0.360.361.020.61.7310.057rs2228570CPSAG0.360.391.160.681.960.610.83rs7975232SLECA0.360.320.820.531.260.40.072rs7975232RACA0.360.290.720.461.120.140.064rs7975232SSCA0.360.220.490.270.880.0120.0064rs7975232CPSCA0.360.411.210.722.030.450.016rs4588SLETG0.230.271.240.7720.420.48rs4588RATG0.230.220.930.561.530.810.84rs4588SSTG0.230.210.890.471.650.770.76rs4588CPSTG0.230.291.370.762.430.260.53Conclusion:There is evidence of nominal association between VDR SNPs: rs731236 (in SLE and CPS), and rs7975232 (in SS and CPS) and the presence of IMIDs disease in Paraguayan patients.Disclosure of Interests:None declared

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