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Introduction Notre objectif était de décrire la prévalence de la séroconversion au SARS-CoV-2 par un dépistage sérologique systématique des patients atteints de rhumatisme inflammatoire chronique (RIC) et d’en étudier les facteurs associés. Matériels et méthodes COVID-RIC1 est une étude transversale multicentrique nationale menée dans 17 centres tertiaires en France. Critères d’inclusion : – ≥ 18 ans ; – diagnostic de polyarthrite rhumatoïde (PR), spondylarthrite axiale (axSpA) ou rhumatisme psoriasique (RPso) ; – traitement ≥ 1 mois par AINS, corticoïdes ou DMARD ; – accepter un test sérologique SARS-CoV-2. Données collectées par formulaire électronique centralisé et anonyme : démographiques, RIC, traitements symptomatiques et de fond, symptômes évocateurs de COVID-19. Les tests sérologiques pour le SARS-CoV-2 pouvaient être réalisés soit au centre tertiaire, soit dans le laboratoire habituel du patient. En cas de tests sérologiques antérieurs, les résultats ont également été recueillis. La séroprévalence était estimée comme le rapport du nombre de tests positifs sur le nombre de tests sérologiques SARS-CoV-2. Une régression logistique a été réalisée pour étudier l’impact des différentes covariables sur la séroconversion. Toutes les variables avec p

Introduction L’arthrite juvenile idiopathique (AJI) est un groupe heterogene de maladies inflammatoires. Les traitements constituent un enjeu majeur afin de prevenir le handicap. La presence de synovites, parfois difficiles a detecter chez l’enfant, est associee au risque de destruction articulaire et l’echographie articulaire permet de detecter des synovites infra-cliniques. Notre objectif principal etait de determiner si l’echographie articulaire etait associee a une modification therapeutique chez les patients atteints d’AJI. Les objectifs secondaires etaient d’identifier les autres facteurs associes au changement therapeutique. Patients et methodes Nous avons mene une etude observationnelle basee sur la cohorte multicentrique JIRECHO, issue de la JIR Cohorte, incluant des patients atteints d’AJI. Les patients avaient un suivi clinique et echographique semestriel standardise. L’echographie articulaire etait realisee par des echographistes prealablement entraines et reproductibles selon les recommandations OMERACT [1] . La synovite echographique etait definie par la presence d’un epanchement et/ou d’une hypertrophie synoviale en mode B (≥ grade 1) associee ou non a une vascularisation doppler (≥ grade 1). Les donnees cliniques, d’activite globale (EVA, JADAS), biologiques (CRP, VS) et therapeutiques etaient recueillies. Resultats Nous avons inclus 112 patients. Le nombre total de visites medicales etait de 185. Trois groupes ont ete definis selon le traitement : augmente (22 %), diminue (14 %) et stable (64 %). Dans un premier temps, nous avons compare les patients chez qui le traitement a ete augmente avec les autres patients. La presence d’au moins une synovite echographique en mode B n’etait pas statistiquement plus eleve dans le groupe « augmentation de traitement » quoique numeriquement plus elevee (80 % vs 65 %, p = 0,06). Il n’y avait pas de difference significative pour la presence d’au moins une synovite en mode doppler (30 % vs 23 %, p = 0,4). Les EVA patients et medecins etaient en revanche significativement plus elevees lorsque le traitement etait augmente [3,3 vs 1,7, p Fig. 1 ). Conclusion Notre etude a montre que la presence d’au moins une synovite echographique en mode B et/ou D n’etait pas statistiquement associee au changement de traitement dans l’AJI, meme si la balance Se/Sp est proche de celle de l’avis du medecin et du score d’activite clinique.

Introduction Le nombre croissant de publications dans le domaine de l’immunologie souligne la necessite d’un filtrage automatique des publications afin d’extraire rapidement les informations integrees dans ce corpus de litterature scientifique. Patients et methodes Pour effectuer une revue systematique de la litterature, nous avons utilise BIBOT, un logiciel de traitement du langage naturel developpe autour d’approches d’apprentissage automatique [1] , [2] , pour analyser automatiquement les etudes sur les aspects epigenetiques des maladies auto-immunes. Nous avons fourni les mots-cles suivants a BIBOT : « Mecanisme epigenetique » et « Maladies auto-immunes ». Resultats En utilisant ces mots-cles comme demande d’utilisateur, 307 articles ont ete identifies comme candidats potentiels a partir de la base de donnees PubMed par le programme. Parmi eux, 162 se referaient a des maladies auto-immunes et 240 a differents mecanismes epigenetiques. L’intersection de ces deux ensembles etait composee de 122 articles. Une analyse approfondie des articles selectionnes a revele 211 cibles bien etablies de mecanismes epigenetiques dans les maladies auto-immunes. Nous avons extrait ces cibles, leur maladie associee et leurs mecanismes associes. Parmi notre selection d’articles, nous avons observe une forte proportion d’etudes concernant le lupus, la methylation et les mecanismes d’ARN non codants ( Tableau 1 ). Nous avons ensuite detaille les mecanismes identifies (methylation, miRNA, etc.), par cible (CD40L, CD70, IFN, etc.) et par pathologie (lupus, polyarthrite rhumatoide, etc.). Conclusion Notre traitement du langage naturel a permis de decrire les tendances generales dans les publications et a fourni un fichier de donnees contenant des meta-informations sur chacune des publications selectionnees sur l’epigenetique.

Introduction La pandémie du SARS-CoV-2 a entraîné une crise sanitaire exceptionnelle, pouvant potentiellement impacter l’observance des traitements immunomodulateurs chez les patients présentant une arthrite juvénile idiopathique (AJI). En France, après le premier confinement et malgré des données rassurantes concernant les enfants et la COVID-19, de nombreux patients et parents semblaient appréhender le retour à l’école. Nous avons évalué l’impact de la pandémie sur l’observance des traitements et sur le retour à l’école. Patients et méthodes Les patients atteints d’une AJI de moins de 18 ans, ayant un traitement de fond antirhumatismal (DMARD), ont été prospectivement inclus à leur consultation et ont complété un questionnaire standardisé. Le critère de jugement principal était la modification du traitement par DMARD du fait de la pandémie. Le retour à l’école a été évalué secondairement. Résultats Un total de173 patients au sein de 8 centres experts en France ont été inclus entre mai et août 2020. Quatre centres étaient situés dans des zones de haute circulation virale et 4 autres centres dans des zones de faible circulation virale. Leur âge médian était de 11,6 ans, 31,2 % avait une AJI polyarticulaire facteur rhumatoïde négative. 50 % étaient traités par méthotrexate, 72,5 % par biologiques DMARD. Une modification de leur traitement par DMARD en lien avec la pandémie a été observée dans 4,0 %. Il n’y avait pas de différence sur la modification des traitements de fond lors de la comparaison entre les zones de haute et de faible circulation virale. L’appréhension au retour à l’école a été retrouvée chez 57,1 % des patients/parents et ainsi les patients ne retournèrent pas à l’école dans 49,1 % des cas dont 69,9 % du fait d’une décision personnelle ou parentale. Deux patients furent diagnostiqués positifs au SARS-CoV-2. Discussion Nos résultats reflètent une bonne observance des patients/parents vis-à-vis des traitements immunomodulateurs avec seulement 4 % d’entre eux ayant diminué ou arrêté leur traitement pendant l’étude. Cependant, une appréhension au retour à l’école a été rapportée chez 57,6 % des patients, et ainsi près de 50 % des enfants ne sont pas retournés à l’école dans les suites du premier confinement. Conclusion Les patients atteints d’AJI traités par DMARD sont restés observants durant la pandémie. Cependant, la réticence des parents a constitué un obstacle majeur au retour à l’école. Des stratégies plus solides de réouverture des écoles doivent donc être développées.

BackgroundRheumatoid arthritis (RA) is one of the most common chronic inflammatory diseases and regularly affects women of childbearing age1. However, there is limited knowledge about the impact of the disease and its treatment on fertility.ObjectivesThe aim of the study was to determine factors associated with time-to-conception in women with RA.MethodsAll RA patients (diagnosis according to the Rheumatologist) included in the national multicenter GR2 cohort from 2015 to June 2021 were included in the analysis. Patients could be included either with a pregnancy wish (i.e., preconceptional period) or because of a clinical pregnancy (12 months or non-achievement of pregnancy), as well as the number exposed to csDMARDs and biologics in the preconception period. We performed survival analyses, using a Cox model including a random effect for the center to account for heterogeneity of practices among participating centers. We used a multiple imputation to address missing data among the explanatory variables. Results are presented as a hazard ratio (HR) with confidence interval (CI) to assess associations between the factors studied and time-to-conception.ResultsAmong the 167 patients with RA included in the GR2 cohort, 78 were selected for the main analysis of time-to-conception. Of these, 40 (51.3%) had a clinical pregnancy during follow-up. Subfertility was observed in 33 (42.3%) women and median time-to-conception was 19.1 months; mean preconception DAS28-CRP score was 2.3 (+/- 1.2).Patients were treated during the preconceptional period with NSAIDs, corticosteroids, csDMARDs and biotherapy in 10 (12.8%), 35 (44.9%), 24 (30.8%), and 32 (41.0%) cases, respectively. The multivariate model adjusted for age, BMI, DAS28-CRP, disease duration, ACPA positivity, and exposure to corticosteroids and biologics in the preconception period found an association between increased preconception delay and age (HR (per year) 1. 12 95% CI [1.04-1.16] p = 0.01) as well as disease duration (HR (per year) 1.06 95% CI [1.02-1.15] p = 0.03).ConclusionThis study provides original results on fertility in women with RA. It found a median time-to-conception of 19.1 months, with a subfertility rate of 42.3%, which is significantly higher than the general population2. In this context, it seems essential to discuss this topic from the beginning of the disease in women of childbearing age.References[1]Van den Brandt S. Arthritis Res Ther. 2017;19(1):64.[2]Junul S. Hum Reprod. 1999;14(5):1250-4.Table 1.Survival analyses (Cox model): factors associated with time-to-conception in women with RA.Univariate analysesMultivariate analysesCrude HR 95% CIpAdjusted HR 95% CIpAge1.11 [1.04-1.18]0.0021.12 [1.04-1.16]0.015BMI1.06 [0.99-1.16]0.1031.08 [0.99-1.16]0.062ACPA positivity1.75 [0.90-3.39]0.1071.44 [0.65-2.86]0.310Disease duration1.03 [0.98-1.08]0.2671.06 [1.02-1.15]0.032DAS28-CRP score1.08 [0.81-1.45]0.5921.08 [0.92-1.32]0.170Corticosteroids0.91 [0.51-1.65]0.7690.86 [0.42-1.68]0.620Biologics1.52 [0.82-2.81]0.1891.30 [0.62-2.78]0.630Figure 1.Cumulative incidence curves for pregnancies in women with RA.AcknowledgementsThe GR2 Cohort is supported by the French Society of Rheumatology, the French Internal Medicine Society, and unrestricted grants from UCB.Disclosure of InterestsSABRINA HAMROUN: None declared, Marion Couderc: None declared, Laure Gossec: None declared, Rene-Marc Flipo: None declared, Hubert MAROTTE: None declared, Christophe Richez Speakers bureau: CR has received consulting/speaker’s fees from Abbvie, Amgen, Astra Zeneca, Biogen, BMS, Celltrion, Eli Lilly, Galapagos, GSK, MSD, Novartis, and Pfizer, all unrelated to this manuscript., Aline Frazier-Mironer: None declared, Jérémie SELLAM: None declared, Elisabeth Gervais: None declared, Valerie Devauchelle-Pensec: None declared, Alban Deroux: None declared, Rakiba Belkhir: None declared, AZEDDINE DELLAL: None declared, Laëtitia Dunogeant: None declared, Cédric Lukas: None declared, Emmanuel Chatelus: None declared, Nathalie Costedoat-Chalumeau: None declared, Anna Moltó: None declared

BackgroundPrimary Sjögren’s syndrome (pSS) is a rare systemic autoimmune disease with no specific treatment at present. To better assess patient symptoms, we have developed a web application (WebApp) to collect patient symptom intensity on a regular basis.ObjectivesTo measure the daily variability of symptoms using the WebApp. We also evaluated its ease of use.Methods45 consecutive patients with pSS were included in 3 referral centers. Symptoms were assessed during the baseline and 3 month visits. We collected the VAS relating to fatigue, dryness and pain as well as the ESSPRI score. Patients used the WebApp daily for 3 months. The variability of symptoms over time was assessed by the predicted median error. This value was determined using a linear regression model, in order to predict the value at the 3rd month, then this value was compared to the actual value collected at the 3rd month during the clinical visit. The ease of use of the WebApp was assessed using a satisfaction score (SUS score).ResultsOf the 45 patients included, 91.1% were women with an average age of 57 years, and low systemic disease activity (84.4% had an ESSDAI score below 5). The intensity of the symptoms collected during the clinical visits was similar at baseline and at 3 months. The values ​​of the median error for each measurement are between 0.5 and 0.8. The 3-month predicted median error values ​​ranged from 2 to -3. The patients all used the web application for 3 months with good attendance (80% of data completion) and were satisfied with this tool (median SUS score = 90).ConclusionSymptoms of pSS fluctuate from day to day in the majority of patients, making a point measurement imprecise. The developed WebApp is easy to use, and could allow more sensitive detection of the effect of a therapeutic intervention. This tool will soon be evaluated during prospective interventional clinical trials.AcknowledgementsI would like to thanks all people who have helped and were directly or indirectly involved in this study.Disclosure of InterestsNone declared

BackgroundTreatment of children with juvenile idiopathic arthritis (JIA) is a major challenge in paediatric rheumatology. The presence of synovitis, which is difficult to detect in children, is associated with structural damage. Musculoskeletal ultrasonography (MSUS) can be used in JIA patients to reveal subclinical synovitis.ObjectivesOur aim was to determine if the use of MSUS is associated with therapeutic modifications in JIA. Secondary outcomes were to identify other factors associated with therapeutic modifications.MethodsWe conducted an observational study based on the JIRECHO multicentre cohort which was developed to provide a systematic MSUS follow-up for JIA patients. Follow-up occurred every six months and included clinical and US examinations. We included children who underwent MSUS of the elbows, wrists, second metacarpophalangeal joints, knees and ankles. Synovitis in US was defined by the presence of joint effusion and/or synovial hypertrophy in B-mode (≥ grade 1) associated or not with Doppler signals (≥ grade 1). US was performed by expert sonographers with good experience in the field of JIA who previously participated in the study of the reliability of the OMERACT paediatric US synovitis definitions and scoring system in JIA (1). Clinical and biological data, disease activity score and information on therapeutics were collected.ResultsWe included 112 patients with 185 visits in total. Three groups of patients were defined according to their therapeutic status: increased(22%), decreased(14%) or stable(64%) treatment. First, we compared patients with treatment escalation with the other patients. Patients with “increased treatment” had more synovitis in B-mode US than the other patients (80% vs. 65%, p=0.06). There was no difference for the presence of synovitis in Power Doppler (PD) US (30% vs 23%, p=0.4). Patient’s and physician’s visual analogue scale (VAS) scores were significantly higher in patients with therapeutic escalation [3.3 vs 1.7, pWe performed ROC curves analysis that showed that the sensitivity and specificity of the US in B-mode was similar to the physician’s VAS, disease score activity or inflammatory biological markers (Figure 1).Figure 1.ROC curves for clinical and biological items and US in B-mode in patients with « therapeutic escalation »ConclusionIn our study, MSUS of ten joints was not statistically associated with treatment escalation or de-escalation in B-mode and PD in patients with JIA.References[1]Rossi-Semerano, L. et al. Application of the OMERACT synovitis ultrasound scoring system in juvenile idiopathic arthritis: a multicenter reliability exercise. Rheumatol. Oxf. Engl. (2020) doi:10.1093/rheumatology/keaa804.Disclosure of InterestsNone declared

BackgroundSpondyloarthritis (SpA) is one of the most common chronic inflammatory diseases and regularly affects women of childbearing age1. However, there is limited knowledge about the impact of the disease and its treatment on pregnancy.ObjectivesThe aim of the study was to determine the factors associated with adverse pregnancy outcome in women with SpA.MethodsAll SpA patients (diagnosis according to the Rheumatologist) included in the national multicenter GR2 cohort from 2015 to June 2021 were included in the analysis. Patients could be included either with a pregnancy wish (i.e., preconceptional period) or because of a clinical pregnancy (ResultsAmong the 207 pregnancies in women with SpA included in the GR2 cohort, 126 were retained for analysis of obstetrical outcome. Of these, 29 (23.0%), 14 (11.1%), 69 (54.8%) were exposed to corticosteroid, NSAID and biologics at least once during pregnancy, respectively. An active disease at least once during pregnancy was found in 47 (37.3%) pregnancies. A live birth was found in 116 (92.1%) women, including 110 (87.3%) full-term births. Early miscarriages and stillbirths were observed in 7 (0.06%) and 3 (0.02%) women, respectively. A caesarean section was performed in 20 (17.2%) cases.A favorable pregnancy outcome was found in 80 (63.5%) of the women. Unfavorable pregnancy outcome was most frequently due to small for gestational age, observed in 22 (19%) pregnancies. The multivariate model adjusted for age, BMI, nulliparity, active disease during pregnancy, smoking, and exposure to NSAIDs and corticosteroids during pregnancy found an association between unfavorable pregnancy outcome with nulliparity (OR 2.63 95% CI [1.01-6.81] p = 0.05).ConclusionThis study provides original results on pregnancy in women with SpA. It found a favorable pregnancy outcome in 63.5% of women. Unfavorable pregnancy outcome was most frequently due to small for gestational age, which should lead to a coordinated management with obstetricians for the follow-up of pregnancy in women with SpA.References[1]Van den Brandt S. Arthritis Res Ther. 2017;19(1):64.Table 1.Multilevel logistic regression model: factors associated with unfavorable pregnancy outcome in women with SpA.Univariate analysesMultivariate analysesCrude OR 95% CIpAdjusted OR 95% CIpAge1.01 [0.92-1.10]0.8591.05 [0.95-1.17]0.297BMI0.99 [0.91-1.07]0.7960.99 [0.90-1.08]0.747Nulliparity2.16 [0.94-4.94]0.0712.63 [1.01-6.81]0.049Smoking0.84 [0.23-3.03]0.8050.84 [0.22-3.21]0.805Disease activity*0.98 [0.40-2.43]0.9641.15 [0.43-3.07]0.778Corticosteroids**1.09 [0.45-2.65]0.8761.15 [0.51-2.71]0.902NSAIDs**0.65 [0.18-2.33]0.1960.67 [0.18-2.56]0.565* BASDAI score ≥ 4 at least once during pregnancy.** Use at least once during pregnancyAcknowledgementsThe GR2 Cohort is supported by the French Society of Rheumatology, the French Internal Medicine Society, and unrestricted grants from UCB.Disclosure of InterestsSABRINA HAMROUN: None declared, Marion Couderc: None declared, Rene-Marc Flipo: None declared, Jérémie SELLAM: None declared, Christophe Richez Speakers bureau: CR has received consulting/speaker’s fees from Abbvie, Amgen, Astra Zeneca, Biogen, BMS, Celltrion, Eli Lilly, Galapagos, GSK, MSD, Novartis, and Pfizer, all unrelated to this manuscript., Rakiba Belkhir: None declared, Laure Gossec: None declared, Hubert MAROTTE: None declared, Emmanuelle Dernis: None declared, Aline Frazier-Mironer: None declared, Elisabeth Gervais: None declared, Cédric Lukas: None declared, Valerie Devauchelle-Pensec: None declared, Laëtitia Dunogeant: None declared, Alban Deroux: None declared, Nathalie Costedoat-Chalumeau: None declared, Anna Moltó: None declared

BackgroundThe prevalence of salivary glands ultrasound (SGUS) abnormalities in Sjögren’s syndrom (SS) is well described(2). However, the prevalence is still unknown in rheumatic inflammatory conditions such as rheumatoid arthritis (RA).ObjectivesThe main objective of this study was to describe the prevalence of SGUS parenchymal structural abnormalites in patients with RA. Secondary objectives were: i) to study correlation between disease duration and the SGUS OMERACT score and ii) to study correlation between duration of sicca syndrome and the SGUS OMERACT score.Methods561 patients with RA satisfying ACR/EULAR 2010 classification criterias were included in 10 french centers in the prospective cohort BCD, comparing joint ultrasonography to clinical follow-up. Cross sectionnal SGUS examination (parotid and submandibular) was performed in a substudy of this cohort. The new OMERACT-SGUS scoring system(1) was used and clinical, biological, immunological and radiological data were collected.Results100 patients agreed to be included in this substudy of BCD cohort, and a total of 98 SGUS patients data were evaluated (lack of SGUS data for 2 patients). Most patients were women (81%), mean age 59 years, with time from RA diagnosis of 11 years on average. The mean CRP-DAS-28 at baseline was at 3.2 with a third of patients in remission at inclusion. Anti-CCP antibodies or RF was positive in 92 patients (92%). 27 patients (27%) complained of eye dryness and 20 (20%) of mouth dryness. 12 (12%) suffered from both. The levels of self-reported fatigue was higher than in the general group of RA included in the study. Two thirds of patients benefited from csDMARD, with a third treated with bDMARDS. 33 (33%) also benefited from a corticosteroid treatment. Among 98 patients, 22 (22.5%) had at least one salivary gland scored grade 1 or more, this number was reduced to 18 patients (18.4%) when considering only the parotid glands. 7 patients (7.1%) had at least one salivary gland scored grade 2 or more, with a number reduced to 4 patients (4.1%) when considering only the parotids. Only one patient (1%) had a parotid gland scored 3. In the 7 patients presenting significant abnormalities in SGUS (grade 2 or more), 5 patients had either dry eye or dry mouth symptoms (71,4%).ConclusionOur findings suggest that 7% of RA patients present significant SGUS abnormalities according to OMERACT scoring system, associated with clinical sicca syndrome in 71% of cases. There was no significant association between the duration of rheumatoid arthritis and the OMERACT score (Spearman coefficient for correlation -0,028, p = 0,99). There was also no significant association found between the duration of sicca symptoms and the OMERACT score (Spearman coefficient for correlation 0,025, p = 0,89). This study highlights the importance of SGUS assessment in RA sicca patients to improve monitoring and follow-up in routine clinical practice.References[1]Jousse-Joulin S, D’Agostino MA, Nicolas C, Naredo E, Ohrndorf S, Backhaus M, Tamborrini G, Chary-Valckenaere I, Terslev L, Iagnocco A, Collado P, Hernández-Díaz C, Gandjbakhch F, Schmidt WA, Filippou G, Dejaco C, Stradner MH, Mortada MA, Hočevar A, Chrysidis S, El Mardenly G, de Agustín JJ, Thiele R, MacCarter DK, Finzel S, Hanova P, Zabotti A, Glaser C, Alavi Z, Hammenfors DS, Gatineau F, Bruyn GA. 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):967-973.[2]Zhang X, Feng R, Zhao J, Wang Y, He J, Liu L, Cheng Y, Yao H, Tang S, Chen J, Zhang S, Zhang Z, Wang Q, He J, Li Z. Salivary gland ultrasonography in primary Sjögren’s syndrome from diagnosis to clinical stratification: a multicentre study. Arthritis Res Ther. 2021 Dec 20;23(1):305.Disclosure of InterestsNone declared

BackgroundThe low prevalence of systemic autoimmune diseases and the diversity of their clinical manifestations make complex to conduct randomised clinical trials to assess the potential efficacy of targeted treatments.ObjectivesTo assess the tolerance and efficacy of targeted therapies prescribed off-label in refractory autoimmune diseases.MethodsThe TATA registry (TArgeted Therapy in Autoimmune Diseases) is a prospective, observational, national and independent cohort follow-up. The inclusion criteria in the registry are as follows: age > 18 years; rare systemic autoimmune disease (systemic lupus erythematosus, Sjögren’s syndrome, systemic scleroderma, inflammatory myopathy, vasculitis) or other refractory rheumatism treated with off-label drugs started after 1st January 2019.ResultsHundred (100) patients (79 females) were enrolled. The median age was 52.5 years [49;56], the median disease duration before enrolment was 5 years [3;7]. The targeted therapies at enrolment were as follows: JAK/STAT inhibitors (44%), anti-IL6R (22%), anti-IL12/23, anti-IL23 and anti-IL17 (9%), anti-BAFF (5%), abatacept (5%), other targeted treatments (9%), and combination of targeted treatments (6%). 73% of patients were receiving corticosteroid therapy at enrolment (median dose 10 mg/day). The current median follow-up time is 9 months [8;10].Safety: 11 serious infections (incidence rate of 14.8 /100 patient-years) and 1 cancer (1.3 cancers/100 patient-years) were observed. Two patients died from severe COVID-19 (2.7 deaths/100 patient-years).Efficacy: The targeted treatment was considered effective by the clinician in 56% of patients and allowed in responders a median reduction of oral corticosteroids of 15 [9-21] mg/day.ConclusionThese initial results of the TATA registry confirm the diversity of targeted treatments prescribed off-label in refractory autoimmune diseases and their corticosteroid-sparing effect when effective. Tolerance was acceptable in these refractory patients with a long history of treatment with immunosuppressive drugs.References[1]B. Terrier et al., Safety and efficacy of rituximab in systemic lupus erythematosus: results from 136 patients from the French AutoImmunity and Rituximab registry. Arthritis Rheum 62, 2458-2466 (2010).[2]J. E. Gottenberg et al., Efficacy of rituximab in systemic manifestations of primary Sjogren’s syndrome: results in 78 patients of the AutoImmune and Rituximab registry. Ann Rheum Dis 72, 1026-1031 (2013).[3]J. E. Gottenberg et al., Risk factors for severe infections in patients with rheumatoid arthritis treated with rituximab in the autoimmunity and rituximab registry. Arthritis Rheum 62, 2625-2632 (2010).[4]F. R. S. S. S. C. I. consortium, contributors, Severity of COVID-19 and survival in patients with rheumatic and inflammatory diseases: data from the French RMD COVID-19 cohort of 694 patients. Ann Rheum Dis, (2020).[5]R. Felten et al., B-cell targeted therapy is associated with severe COVID-19 among patients with inflammatory arthritides: a 1-year multicentre study in 1116 successive patients receiving intravenous biologics. Ann Rheum Dis 81, 143-145 (2022).[6]D. J. Wallace et al., Baricitinib for systemic lupus erythematosus: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet 392, 222-231 (2018).[7]J. J. Paik et al., Study of Tofacitinib in Refractory Dermatomyositis: An Open-Label Pilot Study of Ten Patients. Arthritis Rheumatol 73, 858-865 (2021).[8]S. Cole et al., Integrative analysis reveals CD38 as a therapeutic target for plasma cell-rich pre-disease and established rheumatoid arthritis and systemic lupus erythematosus. Arthritis Res Ther 20, 85 (2018).[9]S. J. Bowman et al., Safety and efficacy of subcutaneous ianalumab (VAY736) in patients with primary Sjogren’s syndrome: a randomised, double-blind, placebo-controlled, phase 2b dose-finding trial. Lancet 399, 161-171 (2022).AcknowledgementsFrench networks (FAI2R, CRI, IMIDIATE, SFR, SNFMI) focused on rare systemic autoimmune diseases contributed this work by the contribution of network-affiliated physicians.Disclosure of InterestsJacques-Eric Gottenberg Consultant of: Abbvie, BMS, Gilead, Galapagos, Novartis, Lilly Roche Chugai, Sanofi, Janssen, Pfizer, Grant/research support from: BMS.Lilly and Pfizer for this register (with no access to data)., Aurore Chaudier: None declared, Yves Allenbach: None declared, Arsene Mekinian: None declared, Zahir Amoura: None declared, Patrice cacoub: None declared, Divi Cornec: None declared, Eric Hachulla: None declared, Pierre Quartier: None declared, isabelle melki: None declared, Christophe Richez: None declared, Raphaèle Seror: None declared, Benjamin Terrier: None declared, Valerie Devauchelle-Pensec: None declared, Julien Henry: None declared, MARC GATFOSSE: None declared, LAURENCE BOUILLET: None declared, Emeline GAIGNEUX: None declared, Vincent ANDRE: None declared, Gildas BAULIER: None declared, Aurélie SAUNIER: None declared, Marie Desmurs: None declared, Antoine POULET: None declared, Mathieu ETE: None declared, Marie-Elise Truchetet: None declared, Martin Michaud: None declared, Claire Larroche: None declared, AZEDDINE DELLAL: None declared, Amelie LEURS: None declared, Sebastien Ottaviani: None declared, Hubert NIELLY: None declared, Guillaume VIAL: None declared, Roland JAUSSAUD: None declared, Benedicte ROUVIERE: None declared, Pierre-Yves JEANDEL: None declared, Aurelien GUFFROY: None declared, Anne-Sophie Korganow: None declared, Mathieu JOUVRAY: None declared, alain meyer: None declared, Emmanuel Chatelus: None declared, Christelle Sordet: None declared, Renaud FELTEN: None declared, Jean Sibilia: None declared, Samira AHMED YAHIA: None declared, Jean François Kleinmann: None declared, Xavier Mariette Consultant of: BMS, Galapagos, GSK, Janssen, Novartis, Pfizer, Sanofi, UCB

BackgroundRheumatoid arthritis (RA) is one of the most common chronic inflammatory diseases and regularly affects women of childbearing age1. However, there is limited knowledge about the impact of the disease and its treatment on pregnancy.ObjectivesThe aim of the study was to determine the factors associated with adverse pregnancy outcome in women with RA.MethodsAll RA patients (diagnosis according to the Rheumatologist) included in the national multicenter GR2 cohort from 2015 to June 2021 were included in the analysis. Patients could be included either with a pregnancy wish (i.e., preconceptional period) or because of a clinical pregnancy ( 3.2 at least once during pregnancy. We performed a multilevel logistic regression model, in which we considered patient and center random effects (patient random effect for some women included in the cohort two times). We used a multiple imputation procedure to address missing data among the explanatory variables. Results are presented as an odds ratio (OR) with confidence interval (CI).ResultsAmong the 167 pregnancies in women with RA included in the GR2 cohort, 92 were retained for analysis of obstetrical outcome. Of these, 43 (46.2%), 8 (7.9%), 40 (43.5%) were exposed to corticosteroid, NSAID and biologics at least once during pregnancy, respectively. A moderate or severe disease activity at least once during pregnancy was found in 20 (21.8%) pregnancies. A live birth was found in 83 (90.2%) women, including 69 (83.1%) full-term births. Early miscarriages were observed in 9 (0.1%) women. A caesarean section was performed in 22 (23.9%) cases.A favorable pregnancy outcome was found in 52 (56.5%) of the women. Unfavorable pregnancy outcome was mainly due to prematurity and small for gestational age, observed in 14 (16.9%) and 17 (20.5%), respectively. The multivariate model adjusted for age, BMI, nulliparity, active disease during pregnancy, smoking, and exposure to biologics and corticosteroids during pregnancy found an association between an unfavorable pregnancy outcome and nulliparity (OR 6.2 95% CI [2.1-17.8] p = 0.002), age (OR (per year) 1.1 95% CI [1.0-1.3] p = 0.02) and exposition to corticosteroids during pregnancy (OR 3.2 95% CI [1.1-9.6] p = 0.04).ConclusionThis study provides original results on pregnancy in women with RA. It found a favorable pregnancy outcome in 56.5% of women. Unfavorable pregnancy outcome was associated with age, nulliparity and corticosteroids use during pregnancy, which argues for their careful use during pregnancy.References[1]Van den Brandt S. Arthritis Res Ther. 2017;19(1):64.Table 1.Multilevel logistic regression model: factors associated with unfavorable pregnancy outcome in women with RA.Univariate analysesMultivariate analysesCrude OR 95% CIpAdjusted OR 95% CIpAge1.09 [1.01-1.19]0.0361.14 [1.02-1.28]0.019BMI0.93 [0.83-1.04]0.1960.91 [0.77-1.08]0.204Nulliparity4.18 [1.66-10.53]0.0036.16 [2.13-17.76]0.002Smoking1.08 [0.29-3.36]0.9961.65 [0.37-7.22]0.141Disease activity*1.06 [0.40-2.81]0.9110.98 [0.21-2.28]0.753Corticosteroids**2.45 [1.05-5.68]0.0393.22 [1.09-9.57]0.038Biologics**1.05 [0.11-3.54]0.5892.02 [0.70-4.12]0.194* Moderate or severe disease activity at least once during pregnancy.** Use at least once during pregnancyAcknowledgementsThe GR2 Cohort is supported by the French Society of Rheumatology, the French Internal Medicine Society, and unrestricted grants from UCB.Disclosure of InterestsSABRINA HAMROUN: None declared, Marion Couderc: None declared, Rene-Marc Flipo: None declared, Laure Gossec: None declared, Christophe Richez Speakers bureau: CR has received consulting/speaker’s fees from Abbvie, Amgen, Astra Zeneca, Biogen, BMS, Celltrion, Eli Lilly, Galapagos, GSK, MSD, Novartis, and Pfizer, all unrelated to this manuscript., Rakiba Belkhir: None declared, Aline Frazier-Mironer: None declared, Valerie Devauchelle-Pensec: None declared, Hubert MAROTTE: None declared, Jérémie SELLAM: None declared, Elisabeth Gervais: None declared, Alban Deroux: None declared, Cédric Lukas: None declared, Emmanuelle Dernis: None declared, Emmanuel Chatelus: None declared, Nathalie Costedoat-Chalumeau: None declared, Anna Moltó: None declared

BackgroundToday, there are still no DMARDs licensed for primary Sjögren Syndrome (pSS) patients. Among the explanations, are the limitations of current outcome measures used as primary endpoints: e.g; high placebo response rate, evaluation of either symptoms or systemic activity, and important features not being assessed. The NECESSITY consortium (https://www.necessity-h2020.eu/), including pSS experts from academia, pharmaceutical industry and patient groups formed to develop a new composite responder index, the Sjögren’s Tool for Assessing Response (STAR) that solve the issues of current outcome measures in pSS and is intended for use in clinical trials as an efficacy endpoint.ObjectivesTo develop a composite responder index in primary Sjögren’s syndrome (pSS): the STAR.MethodsTo develop the STAR, the NECESSITY consortium used data-driven methods, based on 9 randomized controlled trials (RCTs), and consensus techniques, involving 78 experts and 20 patients. Based on reanalysis of rituximab trials (TRACTISS and TEARS) and literature review, the Delphi panel identified a core set of domains to include in the STAR, with their respective outcome measures. STAR options combining these domains were designed and proposed to the panel to select and improve them. For each STAR option, sensitivity to change was estimated by the C-index (derived from Effect size) in all 9 RCTs. Delphi rounds were run for selecting STAR among these options. The Delphi panel also voted to classify trials as positive, negative or “in between” in regards to primary but also key secondary endpoints. For the options remaining before the final vote, meta-analyses of the RCTs were performed separately for positive and “in between” trials together, and for negative trials.ResultsThe Delphi panel identified 5 core domains (systemic activity, patient symptoms, lachrymal gland function, salivary gland function and biological parameters), and 227 STAR options, combining these domains, were selected to be tested for sensitivity to change. After two Delphi rounds, meta-analyses of the 20 remaining options were performed. The candidate STAR was selected by a final vote based on metrological properties and clinical relevance. In positive/in between trials, candidate STAR detected a difference between arms (OR 3.29, 95%-CI [2.07;5.22], whereas it did not in negative trials (OR 1.53, 95%-CI [0.81;2.91]).ConclusionThe candidate STAR is a composite responder index, including in a single tool all main disease features, and is designed for use as a primary endpoint in pSS RCTs. Its rigorous and consensual development process ensures its face and content validity. The candidate STAR showed good sensitivity and specificity to change. The candidate STAR will be prospectively validated in a dedicated three arms RCT of the NECESSITY consortium that will evaluate combination of synthetic DMARDs (hydroxychloroquine + lefunomide or hydroxychloroquine + mycophenolate vs placebo). We encourage the use of STAR in any ongoing and future trials.Table 1.Candidate STARDomainPointDefinition of responseSystemic activity3Decrease of clinESSDAI ≥ 3Patient reported outcome3Decrease of ESSPRI ≥ 1 point or ≥ 15%Lachrymal gland function1Schirmer:If abnormal score at baseline: increase ≥ 5 mm from baselineIf normal score at baseline: no change to abnormalOrOcular Staining Score:If abnormal score at baseline: decrease ≥ 2 points from baselineIf normal score at baseline: no change to abnormalSalivary gland function1Unstimulated Whole Salivary Flow:If score > 0 at baseline: increase ≥ 25% from baselineIf score is 0 at baseline: any increase from baselineorUltrasound:Decrease ≥ 25% in total Hocevar score from baselineBiological1Serum IgG levels: decrease ≥ 10%orRheumatoid Factor levels: decrease ≥ 25%Candidate STAR responder≥ 5 pointsESSDAI: EULAR Sjögren syndrome disease activity index; ESSPRI: EULAR Sjögren syndrome patient reported index; IgG: Immunoglobulin G;AcknowledgementsNECESSITY WP5 STAR development participants: Suzanne Arends (University Medical Center Groningen, Department of Rheumatology and Clinical Immunology, Groningen 9700 RB, Netherlands), Francesca Barone (Centre for Translational Inflammation Research, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK), Albin Björk (Division of Rheumatology, Department of Medicine, Karolinska Institutet, Stockholm, Sweden), Coralie Bouillot (Association Française du Gougerot Sjögren et des Syndromes Secs, France), Guillermo Carvajal Alegria (University of Brest, Inserm, CHU de Brest, LBAI, UMR1227, Brest, France; Service de Rhumatologie, Centre de Référence Maladies Autoimmunes Rares CERAINO, CHU Cavale Blanche, Brest, France), Wen-Hung Chen (GlaxoSmithKline, Research Triangle Park, North Carolina, USA), Kenneth Clark (GlaxoSmithKline Medicines Research Centre, Gunnels Wood Road, Stevenage, Hertfordshire SG1 2NY, United Kingdom), Konstantina Delli (Department of Oral and Maxillofacial Surgery, University Medical Center Groningen (UMCG), University of Groningen, The Netherlands), Salvatore de Vita (Rheumatology Clinic, University Hospital of Udine, Italy), Liseth de Wolff (University Medical Center Groningen, Department of Rheumatology and Clinical Immunology, Groningen 9700 RB, Netherlands), Jennifer Evans (Novartis Pharmaceuticals corporation USA), Stéphanie Galtier (Institut de Recherches Internationales Servier (IRIS), Suresnes Cedex, France), Saviana Gandolfo (Rheumatology Clinic, Department of Medical area, University of Udine, ASUFC, 33100 Udine, Italy), Mickael Guedj (Institut de Recherches Internationales Servier (IRIS), Suresnes Cedex, France), Dewi Guellec (CHU de Brest, Service de Rhumatologie, Inserm, CIC 1412, Brest, France), Safae Hamkour (Center of Translational Immunology, Department of Immunology, University Medical Center Utrecht, Utrecht 3584 GA, Netherlands), Dominik Hartl (Novartis Institutes for BioMedical Research, Basel, Switzerland), Malin Jonsson (Section for Oral and Maxillofacial Radiology, Department of Clinical Dentistry, Faculty of Medicine and Dentistry, University of Bergen, Norway), Roland Jonsson (Broegelmann Research Laboratory, Department of Clinical Science, University of Bergen, Department of Rheumatology, Haukeland University Hospital, Bergen, Norway), Frans Kroese (University Medical Center Groningen, Department of Rheumatology and Clinical Immunology, Groningen 9700 RB, Netherlands), Aike Albert Kruize (University Medical Center Utrecht, Department Rheumatology and Clinical Immunology, Utrecht, Netherlands), Laurence Laigle (Institut de Recherches Internationales Servier (IRIS), Suresnes Cedex, France), Véronique Le Guern (AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne, Paris, France), Wen-Lin Luo (Department of Biometrics and Statistical Science, Novartis Pharmaceuticals, East Hanover, New Jersey), Esther Mossel (University Medical Center Groningen, Department of Rheumatology and Clinical Immunology, Groningen 9700 RB, Netherlands), Wan-Fai Ng (Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne NE2 4HH, UK), Gaëtane Nocturne (Department of Rheumatology, Université Paris-Saclay, INSERM U1184: Centre for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Le Kremlin Bicêtre, Paris, France), Marleen Nys (Global Biometric Sciences, Bristol Myers Squibb, Braine L’Alleud, Belgium), Roald Omdal (Clinical Immunology Unit, Department of Internal Medicine, Stavanger University Hospital, PO Box 8100, 4068, Stavanger, Norway), Jacques-Olivier Pers (LBAI, UMR1227, University of Brest, Inserm, Brest, France and CHU de Brest, Brest, France), Maggy Pincemin (Association Française du Gougerot Sjögren et des Syndromes Secs, France), Manel Ramos-Casals (Department of Autoimmune Diseases, Hospital Clinic de Barcelona Institut Clinic de Medicinai Dermatologia, Barcelona, Catalunya, Spain), Philippe Ravaud (Centre d’Epidémiologie Clinique, Hôpital Hôtel-Dieu, Assistance Publique-Hôpitaux de Paris, Paris, France), Neelanjana Ray (Global Drug Development - Immunology, Bristol Myers Squibb Company, Princeton, New Jersey, USA), Alain Saraux (HU de Brest, Service de Rhumatologie, Univ Brest, Inserm, UMR1227, Lymphocytes B et Autoimmunité, Univ Brest, Inserm, LabEx IGO, Brest, France), Athanasios Tzioufas (Rheumatology Clinic, Department of Medical area, University of Udine, ASUFC, 33100 Udine, Italy), Gwenny Verstappen (University Medical Center Groningen, Department of Rheumatology and Clinical Immunology, Groningen 9700 RB, Netherlands), Arjan Vissink, Marie Wahren-Herlenius (Division of Rheumatology, Department of Medicine, Karolinska Institutet, Stockholm, Sweden). We thank the following experts: Esen Karamursel Akpek, Alan Baer, Chiara Baldini, Elena Bartoloni, Marí-Alfonso Begona, Johan Brun, Vatinee Bunya, Laurent Chiche, Troy Daniels, Paul Emery, Robert Fox, Roberto Giacomelli, John Gonzales, John Greenspan, Robert Moots, Susumu Nishiyama, Elizabeth Price, Christophe Richez, Caroline Shiboski, Roser Solans Laque, Muthiah Srinivasan, Peter Olsson, Tsutomu Takeuchi, Frederick Vivino, Paraskevi Voulgari, Daniel Wallace, Ava Wu, Wen Zhang. We thank the anonymous patients from the NECESSITY Patient Advisory Group and the Sjögren Foundation for their valuable contribution to the Delphi process. We thank EW StClair and AN Baer who generated the baminercept data and made them publicly available.Disclosure of InterestsRaphaèle Seror Consultant of: GlaxoSmithKline, Boehringer, Janssen and Novartis, Grant/research support from: GlaxoSmithKline and Amgen, Gabriel Baron: None declared, Marine Camus: None declared, Divi Cornec Consultant of: GlaxoSmithKline, Bristol Myers Squibb, Janssen, Amgen, Pfizer and Roche, Elodie Perrodeau: None declared, Simon J. Bowman Consultant of: Abbvie, Astra Zeneca, Galapagos and Novartis Pharmaceuticals, Michele Bombardieri Consultant of: UCB, Amgen/Medimmune, Janssen, and GlaxoSmithKline, Grant/research support from: Amgen/Medimmune, Janssen, and GlaxoSmithKline, Hendrika Bootsma: None declared, Jacques-Eric Gottenberg Consultant of: AbbVie, Bristol Myers Squibb, Eli Lilly, Galapagos, Gilead, Pfizer, Roche, Sanofi, Novartis, MSD, CSL-Behring and Genzyme, Grant/research support from: Bristol Myers Squibb, Benjamin Fisher Speakers bureau: Bristol Myers Squibb and Novartis, Consultant of: Novartis, Bristol Myers Squibb, Janssen and Servier, Grant/research support from: Servier, Galapagos and Janssen, Wolfgang Hueber Shareholder of: Novartis Pharma, Employee of: Novartis Pharma, Joel van Roon: None declared, Valerie Devauchelle-Pensec: None declared, Peter Gergely Shareholder of: Novartis Pharma, Employee of: Novartis Pharma, Xavier Mariette Consultant of: Bristol Myers Squibb, Galapagos, GlaxoSmithKline, Janssen, Novartis, Pfizer and UCB, Grant/research support from: Ose Pharmaceuticals, Raphaël Porcher: None declared

BackgroundThe COVID-19 pandemic has raised concerns about its psychological effects. Sleep disturbances, anxiety and/or depressive symptoms, post-traumatic stress symptoms have been reported in general population. Patients with chronic rheumatism, systemic autoimmune disease or auto-inflammatory disease, due to immunosuppression, are at risk of severe forms of infection. Currently, there is little information on psychological impact of the pandemic on the mental health of these more vulnerable patients.ObjectivesTo compare psychological assessment between patients with chronic rheumatic, autoimmune and/or autoinflammatory diseases who presented with COVID-19 infection between March and September 2020, first wave of French pandemic, and patients with same diseases who did not presented with infection to date.MethodsThe MentCOVRMD study was a multicenter descriptive study. Cases were patients with chronic rheumatic, autoimmune and/or autoinflammatory diseases from the French RMD cohort who presented COVID-19 infection between March and September 2020. Controls were patients with same diseases who did not develop infection. The study is registered in Clinical Trials under number 2020-A02058-31.For participants, following criteria were collected: demographics (age, gender, smoking status); psychological assessment questionnaires: Insomnia Severity Index (ISI); Post-traumatic stress disorder (PTSD) checklist; Patient Health Questionnaire (PHQ9) Depression; Generalized Anxiety Disorder (GAD7) Anxiety; Patient Health Questionnaire-15 (PHQ-15) and Somatic Symptom Disorder (SSD)-12.ResultsBetween February and December 2021, 60 cases (46 (76.7%) women), median age 52.0 (39.0; 63.0) were included, of which 15 (25%) had been hospitalized during infection, and 169 controls (148 (87.6%) women), median age of 52.0 (38.0; 63.0). There were more smokers in the group of cases 12 (20%) than controls 14 (9.1%) (p=0.028) as well as more cases on ARA2 treatment (8 (13.3%)) than controls (7 (4.5%)) (p=0.035) with no statistically significant difference in others comorbidities or treatments.There was no statistically difference concerning the ISI scores between cases (11.83 ± 7.31) of which 60% had sleep disorders and controls (11.64 ± 6.82) of which 70.4% had sleep disorders. There was no statistically significant difference in PTSD scores of 15.5 (5.0 to 28.0) for cases and 18.0 (8.0 to 35.0) for controls, of which respectively 12 (20%) had values indicating possible PTSD for cases and 50 (29.6%) for controls. There was no statistically significant difference in PHQ-9 scores (5.5 (1.5 to 11.0)) of which 50% had depressive symptoms and controls (6.0 (2.0 to 11.0)) of which 54.5% had symptoms. There was no statistically significant difference in GAD-7 scores (3.5 (0.0 to 8.0)) of which 40% had anxiety symptoms and controls (4.0 (0.0 to 8.0)) of which 43.2% had symptoms. There was no statistically significant difference in PHQ-15 scores (11.4 ± 6.7), 85% of whom reported presence of symptoms, and controls (10.9 ± 6.2), 82.3% of whom reported symptoms. There was no statistically significant difference in SSD scores between cases (17.7 ± 10.9) and controls (18.4 ± 10.9).There was a statistically significant difference in reported VAS scores of pain related to inflammatory rheumatism in cases with a median of 4.5 (3.0 to 6. 0) compared to controls with a median of 4.0 (1.0 to 6.0) (p=0.011).There was no statistically significant difference in any of the psychological assessment scores between the inpatient and outpatient COVID cases.ConclusionThere was no statistically significant difference between COVID cases and controls in the evaluation of these psychological parameters. Prevalence of all these variables were high in the whole study population, testifying to the need to manage these psychological aspects for patients with chronic rheumatisms, autoimmune and/or autoinflammatory diseases.Disclosure of InterestsNone declared

BackgroundInterleukin-6 (IL-6) is elevated in patients with active polymyalgia rheumatica (PMR) and is associated with disease activity, relapse and severity. Clinical trials with IL-6 receptor (IL-6R) inhibitors in PMR showed higher remission rates and reduced glucocorticoid (GC) use vs GC alone.1-4ObjectivesThe SAPHYR study (NCT03600818) assessed the efficacy and safety of sarilumab (SAR), a fully human anti IL-6Rα monoclonal antibody, with a 14 week (wk) GC taper in patients with steroid resistant active PMR who flared on ≥7.5 mg/day prednisone or equivalent.MethodsPatients were randomized (1:1) to 52 wks of treatment with SAR 200 mg every 2 wks (Q2W) + 14 wk GC tapered regimen (SAR arm) OR placebo Q2W + 52 wk GC tapered regimen (comparator arm). The primary endpoint was the proportion of patients achieving sustained remission at wk 52, defined as disease remission by wk 12, absence of disease flare, CRP normalization from wks 12 to 52 and adherence to the per protocol GC taper from wks 12 to 52.ResultsThe study was terminated early due to protracted recruitment timelines during the COVID-19 pandemic, resulting in 118 of the intended 280 patients recruited between Oct 2018 and Jul 2020, and 117 were treated (SAR n=59, comparator n=58). The demographics were balanced; patients were primarily female, Caucasian, and a median age of ~70 years (Table 1). Overall, 78 patients completed the treatment (SAR n=42; comparator n=36). Primary reasons for treatment discontinuation were adverse events (AEs; SAR n=7, comparator n=4) and lack of efficacy (SAR n=4, comparator n=9). Sustained remission rate was significantly higher in the SAR arm vs the comparator arm (28.3% vs 10.3%; P=0.0193). Results of a sensitivity analysis excluding CRP from the sustained remission definition was consistent with the primary analysis (31.7% vs 13.8%; P=0.0280). All sustained remission components favored SAR (Figure 1). Patients in the SAR arm were 44% less likely to have a flare after achieving clinical remission vs the comparator arm (16.7% vs 29.3%; HR 0.56; 95% CI 0.35–0.90; P=0.0158). The comparator arm required more additional GCs vs the SAR arm, mainly due to PMR flare (median difference in actual and expected cumulative dose 199.5 mg vs 0.0 mg; P=0.0189). The cumulative GC toxicity index scores numerically favored SAR but the difference was not statistically significant. PMR activity scores improved in the SAR arm vs the comparator arm (LS mean -15.57 vs -10.27, nominal P=0.0002). Patient reported outcomes (eg, physical and mental health component scores, disability index, etc) favored SAR (Figure 1). Incidence of treatment-emergent AEs (TEAEs) was numerically higher in the SAR arm vs the comparator arm (94.9% vs 84.5%) and included neutropenia (15.3%) and arthralgia (15.3%) in the SAR arm, and insomnia (15.5%) in the comparator arm. Conversely, the frequency of serious AEs was higher in the comparator arm vs the SAR arm (20.7% vs 13.6%). No deaths were reported.Table 1.Demographics and baseline characteristicsParameterSAR + 14 wk GC taperPlacebo + 52 wk GC taper(n=60)(n=58)Age, median years (range)69 (51–88)70 (52–88)Sex (female), n (%)45 (75.0)37 (63.8)Race, n (%) Caucasian50 (83.3)48 (82.8) Asian1 (1.7)2 (3.4) Not reported9 (15.0)8 (13.8)PMR duration (diagnosis date to baseline),* median days (range)292 (78–3992)310 (66–2784)Any prior disease modifying anti rheumatic drugs, n (%) Methotrexate5 (8.3)10 (17.2) Leflunomide2 (3.3)1 (1.7) Azathioprine01 (1.7) Hydroxychloroquine1 (1.7)1 (1.7) Adalimumab1 (1.7)0 Tocilizumab01 (1.7)CRP (mg/L), median (range)6.8 (0.5–38.2)5.7 (0.1–62.3)Erythrocyte sedimentation rate (mm/h), median (range)25.0 (2.0–115.0)22.0 (5.0–85.0)*SAR n = 54; comparator n= 50.ConclusionSAR + 14 wk GC taper demonstrated significant efficacy vs the comparator arm in steroid refractory PMR patients, including clinically meaningful improvement in quality of life. Safety was consistent with the known safety profile of SAR.References[1]Mori 2016;[2]Akiyama 2020;[3]Lally 2016,[4]Devauchelle Pensec 2015AcknowledgementsMedical writing support was provided by Vijay Kadasi of Sanofi and funded by Sanofi.Disclosure of InterestsBhaskar Dasgupta Consultant of: Sanofi, Roche Chugai, Speakers bureau: Roche Chugai, Cipla, Grant/research support from: Sanofi, Roche, Abbvie, Sebastian Unizony Consultant of: Sanofi, Kiniksa, Janssen, Grant/research support from: Genentech, Kenneth J Warrington Paid instructor for: Chemocentryx, Grant/research support from: Eli Lilly, Kiniksa, GSK, Jennifer Sloane Lazar Employee of: Sanofi, Angeliki Giannelou Shareholder of: Regeneron, Employee of: Regeneron, Chad Nivens Shareholder of: Regeneron, Employee of: Regeneron, Bolanle Akinlade Shareholder of: Regeneron, Employee of: Regeneron, Wanling Wong Employee of: Sanofi, Yong Lin Employee of: Sanofi, Frank Buttgereit Consultant of: Sanofi, Horizon Pharma, Roche, Galapagos, Abbvie, Novartis, Grant/research support from: Sanofi, Horizon Pharma, Roche, Galapagos, Abbvie, Novartis, Valerie Devauchelle-Pensec: None declared, Andrea Rubbert-Roth Consultant of: Sanofi, Speakers bureau: Sanofi, Roche, Robert Spiera Consultant of: Sanofi, GSK, Novartis, Chemocentryx, Roche-Genetech, Abbvie, Vera, Grant/research support from: GSK, Chemocentryx, Corbus, Inflarx, Boehringer Ingelheim

Introduction La physiopathologie de la pseudo-polyarthrite rhizomelique (PPR) reste mal comprise et il n’y a pas de mecanisme auto-immun clairement identifie a l’heure actuelle. D’un autre cote, l’augmentation de cytokines pro-inflammatoires a ete bien decrite. Malgre cela, la classification de la PPR en tant que maladie auto-immune ou maladie inflammatoire n’est toujours pas claire. Notre objectif etait d’etudier les marqueurs inflammatoires et auto-immuns dans une population bien caracterisee de PPR debutantes. Patients et methodes Dix-huit patients avec une PPR precoce, non traites et 18 temoins sains (TS) apparies pour le sexe et l’âge ont ete inclus. Les patients faisaient partie du protocole TENOR evaluant l’efficacite du tocilizumab en ouvert. Ils ont recu 3 perfusion de tocilizumab entre l’inclusion et la semaine 12 puis des corticoides de la semaine 12 a la semaine 24. Les leucocytes, neutrophiles, plaquettes, hemoglobine, γ-globulines, IgG, IgA et IgM ont ete compares entre les patients PPR et les TS avant et apres le traitement par tocilizumab dans le groupe PPR. Un large panel d’auto-anticorps depistables en routine a ete realise chez les patients PPR et chez les TS. Resultats L’âge moyen etait de 68 ± 7 et 66 ± 11 ans, et le taux serique moyen de proteine C-reactive etait de 82 ± 16 et 5 ± 2 mg/l pour les patients PPR et les TS, respectivement. A l’inclusion, les leucocytes (p Discussion Les donnees de notre etude soulignent les consequences de l’inflammation marquee dans la PPR. Le seul element orientant vers une possible auto-immunite est l’augmentation des gammaglobulines. Toutefois, cette augmentation peut egalement se voir dans les syndromes inflammatoires marques. De plus, il n’y a pas d’autoimmunite plus marquee chez les patients PPR, en comparaison aux HC, sur un large panel d’auto-anticorps. Il est toutefois possible qu’un mecanisme auto-immun impliquant des antigenes non identifies soit a l’oeuvre dans la PPR. Le lien fort existant entre la PPR et l’arterite a cellules geantes peut faire suspecter un mecanisme auto-immune, mais il est aussi possible que la PPR soit un etat tres inflammatoire qui puisse evoluer vers une vascularite si une composante auto-immune vient s’ajouter chez certains patients. Conclusion Cette etude suggere que la PPR est plus une maladie inflammatoire qu’une maladie auto-immune. Le tocilizumab ameliore tous les marqueurs de l’inflammation. Les patients PPR presentant des γ-globulines elevees repondent plus rapidement au tocilizumab.

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Introduction L’essai francais ToLEDO (Towards the Lowest Efficacious Dose) cherchait a comparer une strategie de decroissance des bDMARDs (tocilizumab (TCZ) ou abatacept (ABA)) a une strategie de maintien a pleine dose chez des patients ayant une polyarthrite rhumatoide (PR) en remission prolongee. La non inferiorite n’a pas ete demontree pour le critere principal (evolution du DAS44). Un des objectifs secondaires de cet essai etait d’evaluer l’impact de cette strategie de decroissance en termes de progression radiographique a 2 ans. Patients et methodes ToLEDo est un essai randomise controle ouvert multicentrique de non-inferiorite (NI). Les patients devaient presenter une PR selon les criteres ACR-EULAR 2010, etre traites depuis au moins 1 an par ABA ou TCZ (seul ou en association) sans ou avec une corticotherapie a une dose ≤ 5 mg/j, et en remission depuis au moins 6 mois (DAS28 ans , et la progression structurale etait evaluee a l’aide du score de Sharp modifie par van der Heijde (vSHS) par 2 lecteurs entraines et en insu du groupe de randomisation. Les criteres de jugement pour cette analyse secondaire etaient : l’evolution vSHS sur 2 ans (compares entre les deux groupes au moyen d’un modele lineaire mixte), et le taux de progresseurs a 2 ans avec ΔvSHS > 0 et avec ΔvSHS > plus petit changement detectable (SDC = 6,9). Les analyses ont ete realisees en per protocol (PP). Resultats 80 sujets avaient un vSHS renseigne a l’inclusion et a au moins un des deux temps de lecture (44 bras M et 36 bras S) et ont ete consideres pour l’analyse de l’evolution du vSHS. La difference d’evolution du vSHS entre le bras M et le bras S sur 2 ans etait non significative, de 2,47 [IC95 % : -0,34 ;5,28]. Parmi les 80 sujets, 62 (34 bras M et 28 bras S) ont ete consideres pour l’analyse du taux de progresseurs (vSHS renseigne a l’inclusion et a 2 ans). Le taux de progresseurs a 2 ans avec ΔvSHS > 0 n’etait pas significativement plus eleve dans le bras S compare au bras M : 75 % contre 68 %, soit une difference de 7 % [IC95 % de precision : -15 % ; 30 %]. De meme, le taux de progresseurs a 2 ans avec ΔvSHS > SDC n’etait pas significativement plus eleve dans le bras S compare au bras M : 29 % contre 15 %, soit une difference de 14 % [IC95 % de precision : -07 % ; 34 %]. Discussion Cet essai a pu etre penalise par un manque de puissance, un nombre consequent de deviations au protocole ainsi que par l’anciennete et la severite de la PR chez les patients etudies. Conclusion Les resultats sur ces criteres de jugement secondaires radiographique sont coherents avec la conclusion sur le critere de jugement principal : bien que non statistiquement significative, la difference entre les deux groupes, en defaveur du groupe espacement, conduisent a ne pas recommander l’espacement des injections de TCZ et d’ABA chez des patients avec une PR tres erosive ou a risque de progression structurale.

Introduction Les lymphocytes B (LB) sont au centre des mecanismes des maladies auto-immunes. Ces maladies sont definies par une rupture de la tolerance du soi du systeme immunitaire chez les patients et la production d’auto-anticorps contre des composants de son propre organisme. Des auto-anticorps specifiques sont detectables chez tous les sujets sains. Neanmoins, ceci n’engendre pas le developpement des maladies auto-immunes, on parle d’auto-immunite naturelle. Les auto-antigenes et auto-anticorps ont ete bien decrits dans les maladies auto-immunes, cependant tres peu d’etudes existent sur les caracteristiques des cellules productrices de ces auto-anticorps : les lymphocytes B (LB) auto-reactifs. L’objectif de ce travail est d’etudier ces LB auto-reactifs dans deux maladies auto-immunes differentes : la vascularite associee aux ANCA (VAA) et le Syndrome de Sjogren (SS) comparativement a des sujets sains afin de mieux comprendre leur role dans l’auto-immunite naturelle et l’auto-immunite pathologique. Materiels et methodes Nous avons mis au point une methode de reconnaissance de ces LB auto-reactifs circulants par la specificite de leur recepteur a l’antigene (BCR) envers la proteinase 3 (PR3) et la SSA, les deux auto-antigenes cles des deux MAI. L’etude des marqueurs de surface par cytometrie en flux a permis de definir le stade de maturation des LBs. Nous avons egalement mesure leur capacite a secreter des immunoglobulines de differents isotypes par ELISPOT, et leur reponse a differents stimuli. Une analyse du repertoire des immunoglobulines ainsi que le profil d’expression genique a l’echelle unicellulaire sur des LB auto-reactifs, comparativement chez des sujets sains et des malades, a ete effectue pour mieux comprendre la nature de la diversite residente au sein des LB auto reactifs. Resultats Nos resultats montrent que ces LB auto reactifs representent 1 a 4 % des LB circulants chez les patients et les sujets sains. Chez les patients atteint de Syndrome de Sjogren, nous avons une augmentation de la frequence des LB auto-reactifs pathogenes specifiques de la SSA par rapport a la frequence des LB auto reactifs naturel. Nos resultats montrent que ces cellules se repartissent differemment parmi les sous-populations de LB ainsi ils ont un stade de maturation plus avance par rapport aux sujets sains (5 a 9 % sont des LB memoires producteurs d’IgG). Sur un plan fonctionnel, les LB auto-reactifs naturels sont quant a eux capables de secreter des auto-anticorps d’isotype IgM mais pas IgG. Une analyse transcriptomique chez un sujet sain est en cours, montrant que les LB auto reactifs ont un profil transcriptomique different des lymphocyte B totaux. Discussion Cette etude montre qu’il existe bien des LB auto-reactifs circulants chez les patients et chez les sujets sains. Il semble que chez les patients atteints de MAI, ces cellules auto-reactives sont plus enrichis dans les compartiments des cellules matures par rapport aux sujets sains. Ceci suggere que ces LB specifiques d’auto-antigene echappent aux mecanismes de tolerance peripherique, et sont capable d’entrer dans les centres germinatifs afin de finir leur maturation et se transformer en cellules secretrices d’IgG de forte affinite contrairement au LB auto-reactifs chez les sujets sains ou elles restent bloquer a certain stade de differentiation. Conclusion Une meilleure comprehension de la diversite unicellulaire existante au sein des LB auto reactifs chez les sujets sains et les patients nous permettra de mieux comprendre la physiopathologie des MAI.

Introduction Une homogeneisation des pratiques internationales echographiques des glandes salivaires dans le syndrome de Sjogren comportant un grand nombre de centres et d’echographistes est necessaire pour la realisation des essais internationaux. Patients et methodes Exercice international de reproductibilite echographique des glandes salivaires (SGUS) dans le syndrome de Sjogren (SS). Quatorze echographistes de 7 pays ont evalue 60 images echographiques statiques en mode B (30 parotides et 30 glandes sous-maxillaires). Une formation prealable par pays en visioconference a decrit les anomalies pathologiques des glandes salivaires dans le SS(1) et le nouveau systeme de cotation OMERACT (2). Nous avons evalue l’homogeneite (oui/non), la localisation de zones heterogenes (0 a 3), la presence de bandes fibreuses (0-3), le systeme de cotation OMERACT (0-3), l’OMERACT en items binaires (0-1 contre 2-3) et l’appreciation diagnostique du clinicien (oui/non). La reproductibilite intra-lecteur et inter-lecteur a ete estimee en calculant les coefficients κ de Cohen en utilisant SPSS 25,0 (SPSS Inc., Chicago, IL). L’echographiste le plus experimente etait considere comme le gold standard. Resultats La reproductibilite intra-lecteur du plus experimente etait substantielle pour le systeme de cotation OMERACT (kappa a 0,73). La reproductibilite intra-lecteur des autres echographistes etait moyenne a presque parfaite pour l’homogeneite et le diagnostic, la reproductibilite etait moyenne a substantielle pour les autres items (localisation des zones heterogenes, presence des bandes fibreuses). Pour la cotation OMERACT 5/14 (35 %) echographistes obtenaient une reproductibilite substantielle > a 0,6, 6/14 (42 %) avaient une reproductibilite moderee et 3/14 (21 %) avaient une reproductibilite passable. La reproductibilite inter-lecteurs comparee a l’echographiste le plus experimente, montrait une fiabilite moderee a presque parfaite pour l’homogeneite et le diagnostic, mais seulement passable a moderee pour la cotation OMERACT. En changeant le systeme de cotation OMERACT en items binaires, la fiabilite de l’echographiste le plus experimente etait bonne a 0,65, mais nettement inferieur a celui de l’homogeneite. Discussion Les resultats montrent que le systeme de cotation en mode binaire oui/non concernant l’homogeneite et l’appreciation diagnostique du clinicien basee essentiellement sur le caractere homogene ou non de la glande donnent les meilleurs resultats en terme de reproductibilite. En revanche, pour ce qui est de la nouvelle cotation en semi quantitatif (0-3) de l’OMERACT, les resultats montrent qu’une formation en visioconference permet d’homogeneiser les pratiques mais necessiterait un approfondissement. Conclusion L’homogeneite est l’item le plus fiable, et est tres proche de l’appreciation du diagnostic. Le nouveau score semi quantitatif OMERACT presente en visioconference et defini lui aussi sur l’homogeneite glandulaire a donne des valeurs kappa plus faibles mais reste tres utile pour le diagnostic.

Introduction Depuis 2017, le TOCILIZUMAB (TCZ) beneficie d’une AMM dans le traitement de l’arterite a cellules geantes (ACG), ce qui n’est pas le cas dans la pseudo polyarthrite rhizomelique (PPR). Sur la base des donnees d’efficacite du TCZ dans l’ACG, certains centres utilisent le TCZ dans les PPR corticodependantes ou en cas de necessite de sevrage rapide en corticoides. Nous presentons ici une etude observationnelle multicentrique de PPR traitees par TCZ, afin d’en etudier les modalites d’utilisation. Materiels et methodes Plusieurs centres ont ete sollicites incluant les CHU de Lille, Paris (Cochin), Brest, Reims, Amiens, Clermont Ferrand, Nantes, ainsi que les CH d’Arras, Lomme, et Valenciennes. Les donnees ont ete collectees de facon retrospective entre 2015 et 2020. La duree minimale de traitement etait de 3 mois. Les donnees ont ete collectees a M0 (date de decision d’initiation du TCZ), M3, M6, puis tous les ans. Resultats 24 patients ont ete inclus issus de 5 centres. Le sex ratio etait de 2/1 pour les femmes. L’âge moyen au diagnostic etait de 67,5 ans (± 8,2). Le delai moyen d’initiation du TCZ par rapport au debut de la maladie etait de 30,7 mois (± 27,2). 11 patients (45,8 %) presentaient une ou plusieurs complications de la corticotherapie (CTC). La CRP moyenne a M0 etait de 30,7 mg/l (1-56,6). La dose de CTC moyenne a M0 etait de 10,35 mg/j (± 6,3). 17 patients (70,8 %) beneficiaient ou avaient beneficie d’un csDMARD, dans 94,1 % des cas, il s’agissait du METHOTREXATE. Chez 20 patients, le TCZ a ete initie par voie IV a 8 mg/kg toutes les 4 semaines, chez 4 patients par voie SC a 162 mg/semaine. Les indications du TCZ etaient chez 18 patients (75 %) une corticodependance, chez 4(16,6 %) une necessite d’epargne cortisonique, chez 2(8,3 %) une cause mixte. La duree de suivi a varie entre 3 et 66 mois. La duree moyenne de sevrage en CTC etait de 9,8mois (0-32), dont 4 arrets a M0. 2 patients sont toujours sous CTC. 10 patients ont beneficie d’une tentative d’espacement du TCZ. Toutes ces tentatives etaient motivees par une remission prolongee, alors que les diminutions de posologie (n = 6) etaient motivees par un probleme de tolerance. 5 patients ont interrompu le TCZ : (1infarctus du myocarde a M7, 1 lymphome cutane a M9, 2 remissions prolongees a M24 et M60, 1 echec primaire). Cote tolerance, 15 patients ont presente un effet indesirable, 66 % des cas (10/15) etait une perturbation du bilan biologique. Conclusion Actuellement, aucune etude en vraie vie ne renseigne sur les modalites d’utilisation et de suivi des patients traites par TCZ pour une PPR. En l’absence de recommandation, ce travail a permis de mettre en lumiere ces differents aspects. Malgre la faiblesse de l’effectif, le TCZ semble etre efficace sur le sevrage de la corticotherapie, en attente d’une etude controlee randomisee.

Background Giant cell arteritis (GCA) is an immune-mediated, primary systemic vasculitis that affects large and medium-sized arteries. GCA may cause vision loss in up to 20% and requires long term glucocorticoids (GCs). There are currently few data available in France on the epidemiology, patients’ (pts) characteristics, diagnosis and management of GCA in a real-world setting. Objectives The objectives of this study were to address these questions using Health insurance claims data. Methods This retrospective cohort study used the EGB (Echantillon Generaliste des Beneficiaires) database which is a 1% random and representative sample of the French national Health insurance system. EGB contains anonymous demographic and comprehensive medical data on conditions with long-term disease (LTD) status, hospitalizations and reimbursement claims for medications dispensed in the community. The study used the data collected between January 1, 2007 and December 31, 2015. Inclusion criteria were: 1) age ≥50 years; 2) hospitalisation for GCA or LTD status for GCA (ICD-10 codes: M31.5/6); and 3) at least 4 drug dispensing of oral GCs within 6 months around the index date. The index date was defined as the date of 1 st occurrence of GCA code and cases were considered as incident if the GCA code first occurred after ≥2 years of follow-up. Demographics, co-morbidities, diagnostic tests and therapies were analysed. A treatment sequence was defined as the start of a new drug or the resumption of the same drug after a stop ≥3 months. Annual incidence was calculated by using the people recorded in the EGB as denominator. Results Among the 7 52 717 pts recorded in the EGB, 241 pts fulfilled our criteria. Around 24 pts were newly diagnosed/year with an annual incidence of 7 to 10/100,000 people≥50 years-old. 72% of the 241 pts were females, mean age was 77.5 (±8.9) years, mean follow-up 3.7 (±2.6) years. In the 12 months before index date, 74.3% of the pts had at least 1 proxy for hypertension, 39.4% for depression/insomnia and 33.6% for osteoporosis. After index date, temporal artery biopsy (TAB) was performed in 43.2%, high-resolution Doppler ultrasound in 35.3% and positron emission tomography (PET) in 11.6%. Among the 235 pts (97.5%) who had at least 1 drug dispensing of oral GCs, 198 pts (84.3%) used only GCs while 37 (16.7%) also received 1 to 3 adjunctive agents. Mean 1 st GCs sequence duration was 17.2 months (±16.5) in 96.6%. 95 pts (40.4%) had a 2nd sequence, i.e. resume GCs and or start a new drug for a duration of 6.7 months (±8.1) for GCS alone or 12.2 months (±8.8) for GCs+adjunctive drug. The most prescribed GCs-sparing agent was methotrexate (12.0%). Others were marginal: hydroxychloroquine 7 pts, azathioprine 4, cyclophosphamide 1, infliximab 1, adalimumab 2 and etanercept 1 pt. Conclusions These real-world data indicate an incidence of GCA in France of 7 to 10 cases/100,000 people≥50 years-old and underline that most patients with GCA are treated with GCs alone whereas adjunctive agents, mainly methotrexate, are given to 17% of patients. The utilisation of TAB in only half of the patients might reflect a shift towards increasing use of imaging techniques to diagnosed GCA. Disclosure of Interest V. Devauchelle Pensec: None declared, E. Hachulla: None declared, M. Paccalin: None declared, S. Gandon Employee of: Roche SAS, I. Idier Employee of: Chugai Pharma France, M. Nolin: None declared, M. Belhasen: None declared, A. Mahr: None declared

Objectives To define disease activity levels, minimal clinically important improvement (MCII) and patientacceptable symptom state (PASS) with the primary Sjögren's syndrome (SS) disease activity indexes: European League Against Rheumatism (EULAR) SS disease activity index (ESSDAI) and EULAR SS patientreported index (ESSPRI). Methods For 790 patients from two large prospective cohorts, ESSDAI, physician evaluation of disease activity, ESSPRI and patients' satisfaction with their current health status were recorded. Receiver operating characteristic curve analyses and anchoring methods were used to estimate disease activity levels of ESSDAI and the PASS of ESSPRI. At follow-up visit, patients and physicians assessed, respectively, whether symptoms and disease activity have improved or not. An anchoring method based on this evaluation was used to estimate MCII of ESSDAI and ESSPRI. Results Low-activity (ESSDAI

Primary Sjogren's syndrome (pSS) is an autoimmune disease affecting predominantly the salivary glands. Most of the classification criteria have considered involvement of salivary glands as a major sign of the disease. This involvement can be assessed by imaging procedures such as sialography or scialoscintigraphy (considered in the classification criteria currently used), which were the most commonly used procedures until 1990. More recently the development of magnetic resonance imaging (MRI) and ultrasonography (US) offers the opportunity to visualize specific abnormalities of major salivary glands using noninvasive and nonionizing procedures. Because of its easy accessibility and ability to save time in comparison with MRI, US appears to be the major imaging procedure used to classify Sjogren's syndrome that has demonstrated its capacity to improve the diagnostic value of American-European Consensus Group (AECG) and American College of Rheumatology (ACR) criteria. Fluorodeoxyglucose positron emission tomography/computed tomography could have importance in case of suspected lymphoma.

El sindrome de Sjogren primario es una enfermedad autoinmunitaria sistemica que se caracteriza, principalmente, por una lesion inflamatoria de las glandulas salivales y lagrimales, lo que produce un sindrome seco ocular y bucal, aunque tambien puede afectar a otros organos. A veces se asocia a otra enfermedad sistemica, en cuyo caso se lo denomina sindrome de Sjogren secundario. Una de las complicaciones mas graves es el desarrollo de un linfoma, en general tras varios anos de evolucion. La prevalencia del sindrome de Sjogren primario se calcula en el 0,01-3%, con un marcado predominio femenino y un pico de frecuencia en torno a los 50 anos. La fisiopatologia es compleja, con participacion de factores geneticos, ambientales e inmunologicos y, desde hace algunos anos, de los linfocitos B en un papel central. El diagnostico se basa en los criterios de clasificacion publicados en 2002, aunque es probable que pronto sean revisados y se incluyan nuevas exploraciones complementarias, sobre todo la ecografia de las glandulas salivales. Hace poco se han desarrollado escalas especificas para la evaluacion de la repercusion funcional y de la actividad que deberian permitir demostrar mas facilmente el efecto de los tratamientos. Hasta ahora, el tratamiento era basicamente sintomatico, con sustitutos de lagrimas y de saliva o con medicamentos que estimulen su produccion. El tratamiento de las complicaciones sistemicas se basaba en la corticoterapia y los inmunosupresores. Con la bioterapia se abre una via de progreso terapeutico para los proximos anos. Aunque los antifactor de necrosis tumoral alfa (anti-TNF-α) no serian eficaces, los tratamientos dirigidos a los linfocitos B, sobre todo el rituximab y los antifactores activadores de celulas B que pertenecen a la familia de TNF (anti-BAFF), han producido resultados alentadores en estudios abiertos o con muestras pequenas. Tambien se estan desarrollando ensayos terapeuticos cientificos de alta potencia.