Explore the vast range of titles available.

BackgroundSalivary gland ultrasonography (SGUS) is commonly used in primary Sjögren Disease (pSD) as a diagnostic tool [1]. It could also be used to monitor disease activity, but severity of SGUS parenchymal abnormalities in relation to disease duration is not well characterized.ObjectivesTo assess transversally the severity of ultrasound salivary parenchymal abnormalities in relation to pSD duration.MethodsIn this prospective cross-sectional international multicentric study, patients with pSD according to 2002 or 2016 ACR/EULAR classification criteria were included. Parenchymal abnormalities assessed by ultrasound within both parotid and sub-mandibular glands were reported on a standardized form and classified according to the semi-quantitative score of the OMERACT (global score and each item evaluated separately) [2]. Reliability between experts was measured after online training. Demographic, clinical and paraclinical data were also collected and patients were separated into 4 groups according to disease duration from the first buccal dryness symptoms (group A: < 5 years, group B: between 5 and 10 years, group C: between 10 and 20 years, group D: > 20 years of evolution).The association between disease duration groups and SGUS parenchymal abnormalities was quantified in terms of odds ratio and its 95% confidence interval.Results247 patients were consecutively included between May 2019 and February 2022 in 12 international centers. They were 47, 69, 78 and 53 in groups A, B, C and D, respectively. Women represented 94.7% of patients, with a median age of 58 [range 19-89] years old. Oral and ocular dryness were reported by 99.6% and 95.1% of patients, respectively. Salivary flow was abnormal in 74.7% of patients and Schirmer’s test in 82.1%. The focus score was ≥1/4mm2 in 89% of patients. 85% of patients had positive anti-SSA and 59.6% had rheumatoid factor. The median ESSDAI score was 3 [0-48]. Considering for each patient the gland with the highest US OMERACT score, there was a global significant association between disease duration and OMERACT score (OR for 5 years duration: 1.23 [IC95% 1.04; 1.47], p=0.02). When comparing groups A+B versus C+D on the OMERACT score, the OR was 1.95 [IC95% 1.10; 3.46], p=0.02, while no significant difference was found when comparing group A versus B+C+D. Considering each item of the OMERACT score, there was not any statistical difference between the 4 groups in relation to the proportion of an/hypoechoic areas in the gland nor homogeneity or posterior border visibility. The only statistical difference between groups was found regarding the proportion of hyperechoic bands (p = 0.0009). When comparing group A versus B+C+D on this item, the OR was 2.55 [95%CI 1.30-5.01], p=0.006).ConclusionThis large international transversal study in patients with pSD found a positive association between global SGUS lesions evaluated by the OMERACT score and disease duration, with a significant difference only observed in the proportion of hyperechoic bands, when considering separately each item of the score. This may suggest a progressive fibro-adipous evolution of the gland across disease duration. The presence of diffuse hyperechoic bands (grade 3 in the OMERACT scoring system), corresponding to a higher disease duration group, could be useful in the future to stratify patients in clinical trials and to interpret SGUS modifications after treatment.References[1]Jousse-Joulin S, Gatineau F, Baldini C, Baer A, Barone F, Bootsma H, et al. Weight of salivary gland ultrasonography compared to other items of the 2016 ACR/EULAR classification criteria for Primary Sjögren’s syndrome. J Intern Med. 2020 Feb;287(2):180–8.[2]Jousse-Joulin S, D’Agostino MA, Nicolas C, Naredo E, Ohrndorf S, Backhaus M, et al. Video clip assessment of a salivary gland ultrasound scoring system in Sjögren’s syndrome using consensual definitions: an OMERACT ultrasound working group reliability exercise. Ann Rheum Dis. 2019 Jul;78(7):967–73.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

Background:In our previous Sjögren’s Disease (SjD) Genome-Wide Association Study (GWAS) in European populations, significant single nucleotide polymorphism (SNP) peaks were identified between PRDM1 and ATG5 [1]. ATG5 is an autophagy-related protein that plays a crucial role in neutrophil extracellular trap (NET) formation, degranulation, and limiting autoantigens in blood. Dysregulated autophagy has been implicated in SjD and systemic lupus erythematosus (SLE) pathology and poor disease outcomes [2, 3]. The transcriptional repressor PRDM1 plays a role in regulating lymphocyte differentiation [4].Objectives:Identify and functionally evaluate SjD and SLE risk variants in the PRDM1-ATG5 risk locus.Methods:By conducting a meta-analysis of SjD and SLE GWAS datasets, we defined a credible SNP set in the PRDM1-ATG5 locus. A GWAS involving 15,691 SjD and SLE cases and 52,521 population controls of European ancestry was performed, and SNP-trait associations were tested using logistic regression models in PLINK. Bioinformatic analyses (RegulomeDB, HaploReg v4.2, promoter capture Hi-C, eQTLs, etc.) further prioritized SNPs. A CRISPR inhibition (CRISPRi) assay was used to assess the effects of these SNPs on ATG5 expression. Luciferase assays in A235 salivary gland epithelial cell line, PLB985 malignant myelomonoblasts, and GM12878 EBV-transformed B cells tested the activation and/or repressive activity of candidate SNPs.Results:Our investigation revealed several candidate functional SNPs within the PRDM1-ATG5 region. Among them, rs533733 (p=1.15E-18), rs34582442 (p=2.79E-08), rs34599047 (p=2.82E-08), rs77846660 (p=8.39E-04), and rs56886418 (p=4.23E-03) emerged as noteworthy, suggesting their involvement in the regulatory landscape of this locus. Expanding our focus, the inclusion of rs1152966 (p=3.39E-15), rs11152964 (p=7.18E-11), rs573775 (p=6.13E-06), and rs12175062 (p=2.67E-03) in the analysis revealed a broader understanding of eQTLs and chromatin accessibility and modification patterns associated with these SNPs. The extensive influence of these SNPs was observed across various cell types, including minor salivary glands and blood cells, emphasizing their relevance in the pathogenesis of SjD and SLE. Functional assays further elucidated the allele-specific effects of selected SNPs. Notably, rs56885418 and rs3804333 demonstrated a significant decrease in enhancer activity, while rs533733 and rs62422881 exhibited an increase in enhancer activity, particularly in the A253 cell line. These findings underscore the dynamic regulatory impact of these SNPs on gene expression, providing valuable insights into the molecular mechanisms at play in the PRDM1-ATG5 locus.Conclusion:Functional characterization of SNPs in the PRDM1-ATG5 locus provides new insights into the regulatory mechanisms governing gene expression in SjD and SLE. Ongoing studies will focus on in vitro validation of predicted functional SNPs in A235 and GM12878 cells.REFERENCES:[1] Khatri B, et al. Nat Commun. 2022 Jul;13(1):4287.[2] Byun YS, et al. Sci Rep. 2017 Dec;7(1):17280.[3] Wible DJ, et al. Cell Discov. 2019; 5:42.[4] Kallies A, Nutt SL. Curr Opin Immunol. 2007 Apr;19(2):156-62.Acknowledgements:National Institutes of Health (NIH): R01AR071410, R01AR073855, R01AR065953, P50AR060804, U01DE028891; National Research Foundation of Korea (NRF-2021R1A6A1A03038899); Sjögren’s Foundation; Presbyterian Health Foundation; Jerome L. Greene Foundation.Disclosure of Interests:Marcin Radziszewski: None declared, Mandi M Wiley: None declared, Bhuwan Khatri: None declared, Astrid Rasmussen: None declared, Kandice L Tessneer: None declared, Kwangwoo Kim: None declared, Edward M. Vital: None declared, Nick Dand: None declared, Chen Gong: None declared, David Morris: None declared, Phil Tombleson: None declared, Elena Pontarini: None declared, Michele Bombardieri: None declared, Maureen Rischmueller: None declared, Marie Wahren-Herlenius: None declared, Marika Kvarnström: None declared, Torsten Witte: None declared, Hendrika Bootsma: None declared, Gwenny M. Verstappen: None declared, Frans G.M. Kroese: None declared, Arjan Vissink: None declared, Sarah Pringle: None declared, Athanasios Tzioufas: None declared, Clio Mavragani: None declared, Alan Baer Received consulting fees from Bristol Myers Squibb (BMS) and iCell Gene Therapeutics., Marta Alarcon-Riquelme: None declared, Javier Martin: None declared, Xavier Mariette: None declared, Gaetane Nocturne: None declared, Jacques-Olivier Pers: None declared, Jacques-Eric Gottenberg: None declared, Wan-Fai Ng I have consulted for Novartis, BMS, Janssen, Sanofi, Abbvie, IQVIA, Argenx, Resolve Therapeutics., Caroline Shiboski: None declared, Kimberly E Taylor: None declared, Lindsey Criswell: None declared, Blake M Warner: None declared, A Darise Farris Grant/research support from Johnson and Johnson Innovative Medicine (formerly Janssen; ended 12/31/2023)., Patrick M Gaffney: None declared, Judith A. James: None declared, R Hal Scofield Received consulting fees from Johnson and Johnson Innovative Medicine (formerly Janssen) and Merk Pharmaceuticals., Joel M Guthridge: None declared, Daniel J Wallace: None declared, Swamy Venuturupali: None declared, Michael T Brennan: None declared, Juliana Imgenberg-Kreuz: None declared, Lars Ronnblom: None declared, Eva Baecklund: None declared, Maija-leena Eloranta: None declared, Lara A Aqrawi: None declared, Øyvind Palm: None declared, Johan G Brun: None declared, Daniel Hammenfors: None declared, Malin V Jonsson: None declared, Silke Appel: None declared, Sara Magnusson Bucher: None declared, Helena Forsblad-d’Elia: None declared, Thomas Mandl Employee of UCB., Per Eriksson: None declared, Sang-Cheol Bae: None declared, Timothy J Vyse: None declared, Betty Tsao: None declared, Gunnel Nordmark: None declared, Christopher J Lessard Grant/research support from Johnson and Johnson Innovative Medicine (formerly Janssen; ended 12/31/2023).

Background:Sjögren’s disease (SjD) is a complex systemic autoimmune disease with substantial morbidity and 21 known genetic associations. The International Sjögren’s Genetics Network (SGENE) is a growing international collaboration focused on understanding how genetic variants influence SjD pathology. As sample sizes increase, we are focusing our efforts on the analyses of clinical subsets, which few studies have done.Objectives:Our genome-wide association study (GWAS) aimed to identify additional risk loci of genome-wide significance (GWS, p<5E-08; suggestive, p<5x10E-5) in European-derived subsets of SjD.Methods:This study was conducted with IRB/EC approvals. All SjD patients met the 2002 AECG criteria for SjD. A total of 5058 cases and 25943 controls were genotyped on GWAS arrays. After QC, 4855 cases and 25408 controls were included in the analyses. Logistic regression was calculated, adjusting for ancestry using the first 4 principal components to identify SjD-associated SNPs. Cases were split into Ro/SSA+ (n=2898) and Ro/SSA- (n=1313), and analyzed vs. each other, controls, and all-SjD.Results:We observed many differences in the genomic architecture of Ro/SSA- compared to Ro/SSA+ and all SjD (Figure 1a,b), most notably, a complete loss of the significance of the association with MHC on chromosome 6 in the Ro/SSA- cases(Figure 1b). The Ro/SSA+ subjects had a much stronger HLA association with OR ≈ 4 (Figure 1a), while the overall SjD showed OR ≈ 3. While none of the associations observed in the Ro/SSA- population reached GWS, 8 regions (near PLXNA2, PCDH7, IRF5-TNPO3, DLD, LOC100134229, JAK3, LOC643529, and TMTC1) show suggestive associations (Figure. 1a). Of these, only two, IRF5-TNPO3 and LOC643529, are also suggestive in the Ro/SSA+ subset. However, while the Ro/SSA+ have both the IRF5 promoter effect and the extended haplotype through TNPO3, the Ro/SSA- lack the IRF5 promoter effect. Interestingly, previous studies have shown that lupus and systemic sclerosis have both haplotypes while primary biliary cholangitis only has the haplotype extending into TNPO3, similar to Ro/SSA- SjD [1]. When comparing Ro/SSA- to the all-SjD dataset, PLXNA2 and LOC100134229 showed no association; PCDH7, DLD, and TMTC1 showed some association but did not reach suggestive levels; and LOC643529, IRF5-TNPO3, and JAK3 surpassed the suggestive threshold, the latter two nearing or surpassing GWS. Two of the novel suggestive associations in Ro/SSA- are particularly intriguing. PLXNA2 is a member of a semaphorin co-receptor family that mediates repulsive effects on axon pathfinding during nervous system development; interestingly, Ro/SSA- SjD has a higher frequency of neurological involvement. JAK3 is a member of the Janus kinase (JAK) family of tyrosine kinases involved in cytokine receptor-mediated intracellular signal transduction; it is predominantly expressed in immune cells. Mutations in this gene are associated with autosomal severe combined immunodeficiency disease. Novel drugs target the JAK-STAT pathways, making this finding markedly relevant.Conclusion:Our findings highlight the relevance of expanding genetic studies to specific subphenotypes of the disease. While we continue to increase our GWAS sample size and explore other subphenotypes, more work is needed to increase the power of these studies to determine if the suggestive regions will surpass the GWS threshold.REFERENCES:[1] Kottyan LC, et al. Hum Mol Genet. 2015 Jan 15;24(2):582-96.Acknowledgements:NIH/NIAMS R01 AR073855, P50 AR060804; NIH/NIDCR U01DE028891; Sjögren’s Foundation; Jerome L. Greene Foundation.Disclosure of Interests:Astrid Rasmussen: None declared, Marcin Radziszewski: None declared, Bhuwan Khatri: None declared, Kandice L Tessneer: None declared, Elena Pontarini: None declared, Michele Bombardieri: None declared, Maureen Rischmueller: None declared, Marie Wahren-Herlenius: None declared, Marika Kvarnström: None declared, Torsten Witte: None declared, Hendrika Bootsma: None declared, Gwenny M. Verstappen: None declared, Frans G.M. Kroese: None declared, Arjan Vissink: None declared, Sarah Pringle: None declared, Athanasios Tzioufas: None declared, Clio Mavragani: None declared, Alan Baer Received consulting fees from Bristol Myers Squibb (BMS) and iCell Gene Therapeutics., Marta Alarcon-Riquelme: None declared, Javier Martin: None declared, Xavier Mariette: None declared, Gaetane Nocturne: None declared, Jacques-Olivier Pers: None declared, Jacques-Eric Gottenberg: None declared, Wan-Fai Ng I have consulted for Novartis, BMS, Janssen, Sanofi, Abbvie, IQVIA, Argenx, Resolve Therapeutics., Caroline Shiboski: None declared, Kimberly E Taylor: None declared, Lindsey Criswell: None declared, Blake M Warner: None declared, A Darise Farris Grant/research support from Johnson and Johnson Innovative Medicine (formerly Janssen; ended 12/31/2023)., Judith A. James: None declared, R Hal Scofield Received consulting fees from Johnson and Johnson Innovative Medicine (formerly Janssen) and Merk Pharmaceuticals., Joel M Guthridge: None declared, Daniel J Wallace: None declared, Swamy Venuturupali: None declared, Michael T Brennan: None declared, Juliana Imgenberg-Kreuz: None declared, Lars Ronnblom: None declared, Eva Baecklund: None declared, Maija-leena Eloranta: None declared, Lara A Aqrawi: None declared, Øyvind Palm: None declared, Johan G Brun: None declared, Daniel Hammenfors: None declared, Malin V Jonsson: None declared, Silke Appel: None declared, Sara Magnusson Bucher: None declared, Helena Forsblad-d’Elia: None declared, Thomas Mandl Employee of UCB., Per Eriksson: None declared, Gunnel Nordmark: None declared, Christopher J Lessard Grant/research support from Johnson and Johnson Innovative Medicine (formerly Janssen; ended 12/31/2023).

ObjectivesTo take a “high-definition” picture of the main features of primary Sjögren syndrome (SjS) following a worldwide data-sharing cooperative merging of international clinical SjS databases.MethodsThe Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014 including leading clinical centres in SjS of the 5 continents that shared a harmonised data architecture and conducted cooperative online efforts to refine collected data of primary SjS patients fulfilling the 2002 classification criteria.ResultsBy January 2018, the participant centres had included 10 475 patients from 22 countries, including 7637 (73%) patients from Europe, 1420 (14%) from America, 1186 (11%) from Asia, 167 (1.4%) from Australia and 65 (0.6%) from Africa. The cohort included 9781 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years. The frequencies of fulfilment of the 2002 criteria were 92% for dry eye, 94% for dry mouth, 83% for abnormal ocular tests, 82% for positive minor salivary gland biopsy, 78% for abnormal oral diagnostic tests and 76% for positive anti-Ro/La antibodies. The frequency of positive immunological markers at diagnosis was 79% for ANA, 73% for anti-Ro, 49% for RF, 45% for anti-La, 13% for low C3 levels, 14% for low C4 levels and 7% for cryoglobulins.ConclusionsInternational data sharing-based projects merging disperse clinical registries may be essential tools to increase current knowledge and to improve patient care in specific systemic autoimmune diseases.Disclosure of InterestNone declared

Background Ultrasound (US) represents a non-invasive imaging method of the major salivary glands that may serve as a supplement to minor salivary gland biopsy. Minor salivary gland biopsy is part of the diagnostic process for primary Sjögren’s syndrome (pSS), but is not suitable for repeated follow-up. Objectives To investigate if parotid and submandibular gland ultrasonography findings may contribute to pSS diagnosis and in the assessment of glandular function and disease activity. Methods Ultrasound of the parotid and submandibular glands was performed with a GE Logiq E9 using a linear transducer with 6-15MHz. The parotid glands were evaluated in a longitudinal and cross-sectional plane and the submandibular glands in a longitudinal plane. Glandular homogeneity and presence of hypoechogenic areas was evaluated and scored (0-3) according to Hocevar et al 2005 (1). Scores 0-1 were considered “normal” and scores 2-3 “pathological”. Salivary gland functional capacity was evaluated by unstimulated and stimulated sialometry of whole saliva, in ml/15 minutes and ml/5 minutes, respectively (n=21). Ultrasound scores were compared to sialometric findings and minor salivary gland focus score where such data was available. Results Results: Ultrasound was performed in 20 patients, with scores ranging 0-1 (n=10) and 2-3 (n=10). Mean unstimulated and stimulated saliva were 1.1 ml/15 min and 3.3 ml/5 min, respectively, and levels correlated (p<0.001, r=0.726, n=21). Ultrasound scores correlated with unstimulated (p<0.001, r=0.721, n=20) and stimulated saliva (p<0.05, r=0.546, n=20). Mean unstimulated and stimulated saliva levels were higher in patients with normal ultrasound findings compared to pathological (p<0.05). 10/14 patients with unstimulated saliva ≤1.5ml/15 min had pathological ultrasound changes compared to 0/6 with normal unstimulated saliva (p<0.01). 9/12 with stimulated saliva ≤3.5ml/5 min had pathological ultrasound compared to 1/8 with normal stimulated saliva (p<0.01). Focus score was available in 17/21 patients (mean 2.1, range 0-7), and was ≥1 in 14/17 patients. Focus score correlated with ultrasound score (p<0.05, r=0.516, n=16) and mean focus score was 3.3 in patients with ultrasound pathology and 1.2 in patients with normal ultrasound (p<0.05). Conclusions In a small cohort of patients with pSS findings from non-invasive imaging method ultrasound correlate with glandular function and minor salivary gland inflammation. Ultrasound of major salivary glands may become a useful tool in both diagnostics and follow-up of patients with pSS. References Hocevar A, Ambrozic A, Rozman B, Kveder T, Tomsic M. Ultrasonographic changes of major salivary glands in primary Sjögren’s syndrome. Diagnostic value of a novel scoring system. Rheumatology (Oxford). 2005 Jun;44(6):768-72. Disclosure of Interest None Declared

ObjectivesTo analyse the influence of epidemiology and ethnicity on the clinical systemic presentation at diagnosis of primary Sjögren syndrome (SjS).MethodsThe Big Data Sjögren Database included 10 475 worldwide patients from 22 countries fulfilling the 2002 criteria. Age at diagnosis, gender and ethnicity (77% White, 14% Asian, 6% Hispanic, 1% Black/African American, 2% others) were correlated with systemic involvement at diagnosis (retrospectively scored in 9974 patients using ESSDAI/clinESSDAI)ResultsMen had higher mean ESSDAI (8.0 vs 5.9, p<0.001) and clinESSDAI (8.4 vs 6.1, p<0.001) in comparison with women; the domains more active in men included lymphadenopathy (p<0.001), glandular (p<0.001), pulmonary (p=0.001), PNS (p<0.001) and CNS (p<0.001). Highest scores were also reported in patients with young-onset disease (<35 years) with respect to older onset (>65 years) for ESSDAI (6.5 vs 5.6, p=0.002) and clinESSDAI (6.4 vs 5.8, p=0.034). Highest ESSDAI scores were reported in Black/African American, followed by White, Asian and Hispanic patients (6.7 vs 6.3 vs 5.4 vs 4.9, respectively, p<0.001). BAA patients showed the highest frequency of activity in lymphadenopathy, articular, PNS, CNS and biological domains, Whites in glandular, cutaneous and muscular, Asians in pulmonary, renal and haematological and Hispanics in the constitutional domain.ConclusionsThis study provides the first evidence for a strong influence of epidemiology and ethnicity on the systemic phenotype at diagnosis of primary SjSDisclosure of InterestNone declared

ObjectivesTo analyse the influence of geolocation on the clinical systemic presentation of primary Sjögren syndrome (SjS) at diagnosis.MethodsThe Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. Centres were classified by continent, with an additional north-south sub-classification according to latitude (>or<50’N in Europe, equator >or or<30’N in Asia). Systemic involvement at diagnosis was retrospectively scored using the ESSDAI.ResultsThe highest baseline ESSDAI scores were reported from Southern vs Northern countries in Europe (7.2 vs 4.6, p<0.001), America (5.3 vs 3.5, p<0.001) and Asia (6.3 vs 3.9, p<0.001). In Europe, the frequency of activity in each domain was higher in Southern countries (in all domains except constitutional, p<0.001). In America, Southern countries had the highest frequencies of active patients in constitutional, articular, cutaneous, pulmonary, PNS and CNS domains (p<0.001 in all) and the lowest frequencies in lymphadenopathy (p=0.018) and biological (p<0.001) domains. In Asia, patients from China had the highest frequency of activity in glandular, articular, pulmonary, muscular, haematological and biological and those from India in lymphadenopathy, cutaneous, renal and PNS.ConclusionsThis study provides the first evidence for a strong influence of geolocation on the systemic phenotype of primary SjS at diagnosis. Geographical determinants should be considered as key variables when systemic disease is scored.Disclosure of InterestNone declared

ObjectivesTo analyse the influence of ethnicity on the clinical presentation at diagnosis in an international cohort of patients with primary Sjögren syndrome (pSS).MethodsThe Big Data Sjögren Project is a multicentre registry formed by international experts from the EULAR-SS Task Force. By January 2016, the database included 8315 consecutive patients fulfilling the 2002 AE criteria (20 countries, 5 continents). Ethnicity was classified according to FDA (White=W, Black/African American=B, Asian=A, Hispanic=H, Others=O).ResultsEthnicity data was available in 7887 (95%) patients: 6177 W (78%), 1066 A (13%), 393 H (5%), 104 B (1%) and 147 O (2%). European patients were overwhelmingly white compared with Americans (96% vs 52%, p<0.001). The greatest differences between ethnicities (p<0.001) were observed for mean age at diagnosis (47yrs in B vs 54yrs in W), and the frequency of males (12% in B vs 4% in A), dry eyes (76% in A vs 98% in O), dry mouth (83% in A vs 99% in O), abnormal ocular tests (79% in O vs 90% in H), abnormal oral tests (65% in O vs 91% in H), positive minor salivary biopsy (75% in O vs 95% in H), positive anti-Ro (54% in O vs 84% in A) and positive anti-La (28% in O vs 50% in A).ConclusionsWhite patients had the oldest age at diagnosis, Black/African Americans the highest frequency of men and the lowest mean age, Asians the highest frequency of women, the lowest frequency of sicca symptoms and the highest frequency of Ro/La and Hispanics the highest frequency of abnormal diagnostic tests. Ethnicity plays a capital role in the phenotypic expression at diagnosis of primary SS.Disclosure of InterestNone declared

ObjectivesTo analyse the diagnostic approach used in the largest international cohort of patients with primary Sjögren syndrome (pSS)MethodsThe Big Data Sjögren Project is a multicentre registry formed in 2014 by experts participating in the EULAR-SS Task Force. By January 2016, the database included 8315 consecutive patients fulfilling the 2002 criteria. The main features at diagnosis/recruitment (time of criteria fulfilment) were collected and analysedResultsThe cohort included 7753 (93%) women (mean age at diagnosis 53 years). Sicca symptoms were reported in more than 90% of patients at diagnosis (92% for dry eye and 92.5% for dry mouth). The diagnostic tests used included the determination of anti-Ro/La antibodies in 8250 (99%) patients, Schirmer test in 6205 (75%), salivary gland biopsy in 5988 (72%), salivary flows in 4941 (59%), corneal stainings in 3304 (40%), scintigraphy in 2578 (31%) and sialography in 873 (10%) patients. The mean number of diagnostic tests included in the 2002 AE criteria was 4.85 ± 1.44 (of a maximum of 8 different tests), and was higher in patients with negative ANA (5.18 vs 4.80 in ANA+, p<0.001) or negative RF (5.06 vs 4.81 in RF+, p<0.001), and was also higher in American patients vs European or Asian patients (5.28 vs 4.83 and 4.63, respectively). A correlation was found between the number of tests carried out and the number of 2002 criteria finally fulfilled (R=0.48)ConclusionsWe found a heterogeneous diagnostic approach of pSS with respect to the number of 2002AE diagnostic tests carried out; the approach varied significantly according to geographic origin and baseline immunological profileDisclosure of InterestNone declared

ObjectivesTo characterize and quantify systemic involvement at diagnosisinalarge international cohort of patients diagnosed with primary Sjogren syndrome (SS).MethodsThe Big Data Sjögren Project is an international, multicenter registry formed in 2014 with the aim of taking a “high-definition” picture of the main features of primary SS at diagnosis by merging international SS databases. By January 2015, the database included 5027 consecutive patients fulfilling the 2002 classification criteria for primary SS from 13 countries (9 European, 4 American). Systemic involvement was defined according to the ESSDAI and retrospectively calculated.ResultsBaseline ESSDAI data was collected in3314 patients (94% female, mean age at diagnosis 54.25 years). The main features of systemic involvement at diagnosis included biological activity in 43%of patients, articular involvement in 35%, hematological activity in 25%and glandular involvementin 19%. The mean ESSDAI score at diagnosis of the entire cohort was 5.63 (range, 0-62). Low DAS was reported in 1267 (38%) patients, moderate DASin 943 (28%) and high DAS in 378 (11%) patients; in the remaining 726 patients (22%), the ESSDAI score at diagnosis was 0. The mean baseline ESSDAI was higher in males (7.33 vs 5.52, p<0.001), patients diagnosed ≤35 years (6.71 vs 5.63, p=0.001), those with positive ocular tests (5.75 vs 4.98, p=0.023) and those with positive immunological markers including ANA (6.71 vs 4.63, p<0.001), RF (7.46 vs 5.49, p<0.001), anti-Ro/SSA (6.77 vs 5.48, p<0.001) and anti-La/SSB (6.91 vs 6.0, p<0.001). According to the number of criteria fulfilled at diagnosis, a higher mean baseline ESSDAI was found in patients fulfilling the six criteria (7.67 vs 5.65 in those fulfilling 5 criteria and 5.23 in those fulfilling 3-4 criteria, p<0.001).ConclusionsEpidemiological features (male sex, younger age at diagnosis) and positive autoantibodies at diagnosis were closely-related to greater systemic activity. Using the ESSDAI in real-life situations may help identify epidemiological and immunological subsets with high systemic activity at diagnosis and, therefore, at high risk of suffering a complicated clinical course.Disclosure of InterestNone declared