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ImportanceFew treatments are available for patients with glucocorticoid-dependent polymyalgia rheumatica. IL-6 antagonists may reduce disease activity in patients with active glucocorticoid-dependent polymyalgia rheumatica.ObjectiveTo compare the efficacy of tocilizumab vs placebo in patients with glucocorticoid-dependent polymyalgia rheumatica.Design, Setting, and ParticipantsThis double-blind, parallel-group, placebo-controlled randomized clinical trial enrolled 101 patients with polymyalgia rheumatica at 17 hospitals in France from February 2017 to October 2019. Final follow-up occurred in November 2020. Inclusion criteria were persistent disease activity (polymyalgia rheumatica activity score computed using the C-reactive protein level [CRP PMR-AS] >10) and prednisone dose greater than or equal to 10 mg per day.InterventionsPatients were randomly assigned to receive intravenous tocilizumab (8 mg/kg; n = 51) or placebo (n = 50) every 4 weeks for 24 weeks, combined with predefined standardized tapering of oral prednisone.Main Outcomes and MeasuresThe primary efficacy end point was CRP PMR-AS less than 10 (range, 0-100; higher values indicate greater activity; no minimal clinically important difference defined) combined with either prednisone dose less than or equal to 5 mg per day or a decrease in prednisone dose greater than or equal to 10 mg from baseline at week 24. There were 11 secondary outcomes assessed at week 24 included in this report, including disease activity (measured by CRP PMR-AS) and the proportion of patients no longer taking prednisone.ResultsOf the 101 randomized patients (mean age, 67.2 years; 68 [67.3%] women), 100 (99%) received at least 1 infusion and 100 completed the trial. The primary end point was achieved in 67.3% of patients in the tocilizumab group and 31.4% of patients in the placebo group (adjusted difference, 36.0% [95% CI, 19.4%-52.6%]; adjusted relative risk, 2.3 [95% CI, 1.5-3.6]; P P P Conclusions and RelevanceAmong patients with active polymyalgia rheumatica despite prednisone therapy, tocilizumab, compared with placebo, resulted in a significantly greater percentage of patients with a CRP PMR-AS less than 10 with reduced prednisone requirements at week 24. Further research is needed to confirm efficacy and to determine the balance of potential benefits and harms.Trial RegistrationClinicalTrials.gov Identifier: NCT02908217

Objectives To determine whether acetabular dysplasia is associated with hip pain at physical examination among adults with recent-onset inflammatory back pain (IBP) suggesting axial spondyloarthritis (axSpA). Methods This cross-sectional ancillary study was conducted on the prospective DESIR cohort, which enrolled patients aged 18–50 years who had recent-onset IBP. Two readers used antero-posterior pelvic radiographs to assess the Tönnis angle, acetabular angle (AA), lateral centre-edge angle (LCEA), and femoral head extrusion index (FHEI). Abnormality of one or more of these four variables defined acetabular dysplasia. Hip pain upon physical examination was assessed based on Ritchie’s articular index. Results The overall prevalence of acetabular dysplasia was 22% (139/636). The proportion of females was higher in the group with acetabular dysplasia. Hip pain was found in 21% (29/139) of patients with versus 12% (59/497) without acetabular dysplasia (OR, 1.96; 95% CI, 1.20 to 3.20); the association was significant in males (OR, 3.14; 95% CI, 1.44 to 6.86) but not females (OR, 1.39; 95% CI, 0.74 to 2.62). Results were similar when acetabular dysplasia was defined on the basis of LCEA alone (OR, 2.15; 95% CI, 1.18 to 2.62). Conclusion Among patients with recent-onset IBP suggesting axSpA, acetabular dysplasia was significantly associated with hip pain in males. Hip pain related to acetabular dysplasia might result in overdiagnosis of hip involvement by axSpA.

To assess, in patients with recent-onset arthritis, whether a self-reported familial occurrence of rheumatoid arthritis (RA) is associated with a clinical presentation of the disease, final diagnosis, long-term outcome and treatment decisions.The study was conducted from data of patients included between 2002 and 2005 in the early arthritis ESPOIR cohort. Patients were recruited on the basis of having at least two swollen joints for6 weeks and6 months, no other diagnosis than RA and no previous exposure to glucocorticoids or disease-modifying antirheumatic drugs (DMARDs). Patients were stratified into two groups according to the presence of a self-reported familial occurrence of RA at baseline. Data concerning final diagnosis (2-year visit), long-term outcome (5-year visit) and therapeutic decisions were compared between the 2 groups of patients, using logistic and Cox regression models.At baseline, 115 patients (14.1%) reported a familial occurrence of RA and showed, as compared with the remaining participants, higher prevalence of extra articular manifestations (EAMs) (51.8% vs. 39.6%, p=0.01) and severe EAMs (7.9% vs. 3.1%, p 0.01). Both unadjusted (hazard ratio, 1.57; 95% CI, 1.1-2.21; p = 0.01) and adjusted analysis (hazard ratio, 1.51; 95% CI, 1.06-2.15; p=0.02) identified a higher probability for the initiation of a targeted DMARD over time among patients with a self-reported familial occurrence of RA.In the specific context of early arthritis, a self-reported familial occurrence of RA is associated with the future decision to initiate a targeted DMARD.

To determine whether repeated minor salivary gland biopsy (MSGB) has a clinical diagnostic utility in patients with suspicion of Sjögren's syndrome (SS).Clinical, biological, pathological data and physician's diagnosis after each MSGB from patients with suspected primary or secondary SS who had benefited from 2 MSGB at Brest University Hospital between January 1st, 1990 and January 14th, 2015, were retrospectively collected. We compared the characteristics of patients with and without first positive MSGB, concordance between the MSGB, and analyzed the modifications of diagnosis after the second MSGB.Ninety-three patients were included, first MSGB was positive for 23 and negative for 70. Patients with first positive MSGB had more often renal involvement (P0.05) and hypergammaglobulinemia (P=0.01), anti-SSA antibodies (P0.05) and positive second biopsy with focus score ≥ 1 or Chisholm2 (P0.01). The mean time between the 2 MSGB was 5.7±4.3 years. The concordance between the results of the 2 biopsies was low (κ = 0.34). MSGB influenced diagnostic's change in 10 cases where the second MSGB was always guided by new specific clinical manifestations.We observed a low concordance between 2 MSGB in patients with suspected pSS in our study. Despite this variability, performing a second MSGB changed the initial diagnosis in only a minority of the patients and was particularly useful when clinical manifestations had deeply evolved.

RESUME Le conflit ischio-femoral (CIF) est une cause inhabituelle de douleur de hanche. Certains sports contraignants peuvent causer ce conflit, comme la danse.Nous rapportons le cas d'une danseuse de ballet de 11 ans presentant un CIF bilateral secondaire a un surentrainement et a une hypertrophie du muscle carre femoral.Ce syndrome est frequemment evoque dans le domaine de l'orthopedie suite a une prothese de hanche. Le CIF bilateral est rare et doit etre recherche dans le cadre d'un sport intense comme la danse lorsque toute autre cause a ete eliminee.

Ischiofemoral impingement syndrome (IFIS) is an unusual cause of hip pain. Certain strenuous sports can cause this impingement, such as dancing. We report the case of an 11-year-old ballet dancer with bilateral IFIS secondary to overtraining and hypertrophy of the quadratus femoris. This syndrome is frequently evoked in the field of orthopedics following hip replacement. Bilateral IFIS is rare and should be sought in the context of an intense sport such as dance when any other cause has been eliminated.

Resume Un inhibiteur de kinase est un traitement qui inhibe une ou plusieurs kinases intervenant dans une voie d’activation cellulaire utilisee preferentiellement par les cellules impliquees dans la maladie a traiter (cancer, maladies auto-immunes ou inflammatoires). En ce qui concerne les affections dysimmunitaires, ce sont actuellement les anti-JAK kinases (tofacitinib et baricitinib) qui font l’objet de travaux cliniques, essentiellement dans la polyarthrite rhumatoide. Les etudes qui ont ete realisees pour le developpement des anti-JAK dans la PR sont extremement completes, puisque des phases 3 ont etudie leur efficacite en premiere ligne, en association au methotrexate lorsque celui-ci etait insuffisant seul, en association au methotrexate a la place des anti-TNF lorsque ceux-ci etaient insuffisants, et meme la possibilite de leur decroissance en cas de reponse satisfaisante. Leur toxicite (risque infectieux, risque de neoplasie) parait similaire a celle des traitements biologiques, mais une meilleure connaissance de ces risques apres une exposition prolongee est necessaire. Parmi leurs avantages indiscutables, on retient la prise orale, leur demi-vie courte, la simplicite de prise (envisageable une fois par jour), leur efficacite, le cout de production a priori plus faible que celui des biotherapies et leur caractere non immunogene. Le positionnement de ces traitements peut se discuter a toutes les phases du traitement de la PR, mais il est evident que le prix des molecules va serieusement impacter les choix. Pour l’EULAR, sa place actuelle est reservee a l’echec d’un traitement de fond (DMARD) ou d’un biologique.

Resume Medicament antifolate, le methotrexate a ete prescrit dans la polyarthrite rhumatoide et le rhumatisme psoriasique a partir des annees 1980 et est devenu la pierre angulaire du traitement de la polyarthrite au cours des annees 1990. Les recommandations actuelles de l’EULAR et de la SFR considerent qu’un traitement par methotrexate (un autre medicament synthetique etant propose seulement en cas de contre-indication ou d’intolerance a ce dernier) doit etre debute des le diagnostic de polyarthrite rhumatoide pose. Sa dose optimale (au maximum 25 mg une fois par semaine), doit etre atteinte au maximum en 4 a 8 semaines. En cas d’inefficacite ou de mauvaise tolerance de la voie orale, un passage a la voie sous cutanee est conseille sur la base de donnees pharmacocinetiques et cliniques. Les examens complementaires et les vaccinations a realiser avant l’initiation du traitement sont l’objet de recommandations. Le methotrexate est contre-indique en cas de grossesse ou d’allaitement. Les effets secondaires graves sont hematologiques (cytopenies severes) et plus exceptionnellement pulmonaires (pneumopathie interstitielle), justifiant l’arret du traitement. Ses principaux effets secondaires mineurs sont gastro-intestinaux, buccaux (stomatites), et biologiques (cytopenie legere, cytolyse) et sont en partie prevenus par la prise d’acide folique (dose hebdomadaire maximale de 5 ou 10 mg) et reversibles a la baisse ou l’arret du traitement.

Introduction La prise en charge de la spondyloarthrite (SpA) axiale est dans certains cas difficile, tant du point de vue de sa confirmation diagnostique que de la decision therapeutique. Chez certains patients, des pathologies autres que la SpA sont susceptibles d’influencer son evaluation clinique. La dysplasie acetabulaire (DA) constitue une anomalie frequente de l’architecture de la hanche, pour laquelle plusieurs etudes ont montre qu’elle peut etre responsable de symptomes douloureux. L’objectif de cette etude ancillaire de la cohorte DESIR etait de determiner si l’identification d’un phenotype de DA est associee a la presence d’une douleur de hanche a l’examen clinique chez les patients presentant une lombalgie inflammatoire recente evocatrice de SpA axiale, pouvant alors constituer une source de confusion lors de l’evaluation des patients. Patients et methodes Cette etude a ete conduite a partir des donnees de la cohorte prospective DESIR, dans laquelle ont ete inclus des patients âges de 18 a 50 ans, presentant une lombalgie inflammatoire recente evocatrice de SpA axiale. L’evaluation radiographique de la DA a ete realisee par l’intermediaire d’une double lecture (DG et GP), a partir des radiographies de bassin de face obtenues lors de la visite d’inclusion. Pour chaque parametre d’evaluation de la DA, la moyenne des mesures faites par chaque lecteur a ete prise pour reference. Les parametres qui ont ete mesures et les valeurs limites correspondantes retenues pour definir la DA etaient : l’angle de Tonnis (> 12°) ; l’angle acetabulaire (> 45°) ; l’angle VCE–Vertical Center Edge - ( Resultats La prevalence globale de la DA etait de 21,9 % (139/636). Elle etait de 26,4 % chez les femmes (90/341) et de 16,6 % (49/295) chez les hommes. A l’exception d’une proportion plus elevee de femmes, il n’a pas ete mis en evidence de difference significative entre les patients avec DA et les autres participants pour ce qui concerne leurs caracteristiques generales et celles relatives a la SpA axiale (activite de la maladie, impact fonctionnel, caracteristiques radiologiques). La presence d’une douleur de hanche etait rapportee chez 20,9 % (29/139) des patients avec DA versus 11,9 % (59/497) chez les autres participants (p = 0,007). En limitant la definition de la dysplasie a la presence d’un angle VCE anormal, la presence d’une douleur de hanche etait rapportee chez 23,6 % (17/72) des patients avec DA versus 12,6 % (71/564) des autres participants (p = 0,01). Conclusion Parmi les patients presentant une lombalgie inflammatoire recente compatible avec une SpA axiale, la presence d’un phenotype de DA (selon plusieurs definitions) est associee a la constatation clinique d’une douleur de hanche. L’existence d’une DA sous-jacente semble pouvoir constituer un facteur de confusion lors de l’evaluation de l’activite de la maladie et/ou d’une eventuelle atteinte inflammatoire de la hanche dans le contexte de la SpA axiale.

Introduction Le mode de vie influence la survenue et l’evolution de nombreuses pathologies. Dans le domaine de la medecine du sport, l’inactivite physique et certaines habitudes alimentaires qui ont un role en tant que determinant dans l’evolution de maladies chroniques sont particulierement discutees lors des consultations et des seances d’education therapeutique. Le volume des etudes dediees a ces domaines est tres important, ce qui complique l’analyse de la litterature. Il est pourtant indispensable d’en prendre connaissance pour conseiller les patients sur le type de modification de mode de vie a privilegier. Le but de cette etude pilote est de comparer le nombre, la proportion, le type d’essais randomises et leurs resultats dans la gonarthrose et la polyarthrite rhumatoide, en se limitant aux modifications de mode de vie dans les domaines de la nutrition et de l’activite physique. Patients et methodes Cette revue systematique de la litterature selon les recommandations Preferred Reporting Items For Systematic Reviews and Meta-Analyses (PRISMA) s’appuie sur un programme d’intelligence artificielle (logiciel BIBOT) [1] complete d’une lecture manuelle, puis l’analyse les donnees identifiees en termes descriptif (nombre, proportion) et comparatif (proportions d’etudes positives selon la maladie en utilisant le Chi2). Resultats L’analyse a ete decomposee en 4 requetes (nutrition et activite physique dans la polyarthrite rhumatoide et la gonarthrose). Au total, 7931 articles publies entre 1990 et 2020 ont ete analyses. Concernant la nutrition, 20 articles ont ete conserves : parmi tous les articles selectionnes, 75 % atteignaient leur objectif. L’utilisation de probiotiques representait 25 % des interventions iteratives des etudes randomisees. Concernant l’activite physique, un total de 267 articles a ete conserve : parmi tous les articles selectionnes, 80,9 % atteignaient leur objectif. Les exercices etaient en majorite supervises (57,4 % dans la gonarthrose et 81 % dans la PR) combines (45,5 % pour la gonarthrose et 62,1 % pour la PR). Concernant l’activite physique, un total de 267 articles a ete conserve : parmi tous les articles selectionnes, 80,9 % atteignaient leur objectif. Il est a noter que parmi les criteres de jugement primaires concernant la gonarthrose, l’amelioration du WOMAC, critere principal dans les etudes ou il est utilise, est statistiquement associee au type d’activite physique utilise avec p = 0,033. Il n’y a en revanche pas d’association significative entre le fait d’avoir atteint l’objectif d’amelioration du WOMAC et la supervision ou non de l’activite physique (p = 0,138). Le choix de l’objectif principal apparait important et les etudes qui utilisent un autre outil que le WOMAC sont beaucoup plus souvent associees au fait d’atteindre l’objectif principal. Les exercices etaient en majorite supervises (57,4 % dans la gonarthrose et 81 % dans la PR) combines (45,5 % pour la gonarthrose et 62,1 % pour la PR). Mais le type d’intervention etudie n’est statistiquement pas le meme dans la PR et dans l’arthrose du genou (p = 0,001). Conclusion La prise de complements alimentaires specifiques tels que les probiotiques a demontre son efficacite dans la gonarthrose et de la polyarthrite rhumatoide. L’activite physique a surtout ete evaluee sous forme d’exercices supervises sans qu’un programme specifique adapte donnant un meilleur resultat puisse etre degage du fait de l’heterogeneite des etudes. Nous proposons d’utiliser le terme de mega-analyse pour ce type de revue de litterature explorant un tres grand nombre d’etudes grâce a l’intelligence artificielle. Des etudes portant sur les autres aspects du mode de vie et d’autres facteurs de risques (tabac, polluants environnementaux, etc.) pourront etre envisagees sur le meme modele.

Background Myositis is recognized as a manifestation of primary Sjogren’s syndrome (pSS). Grading muscle involvement using the EULAR Sjogren Syndrome Disease Activity index (ESSDAI) does not require biopsy-proven active myositis or extensive immunological investigations. However, several studies have reported that a high proportion of pSS-associated myositis is either inclusion body myositis (IBM) or an overlap with idiopathic inflammatory myopathies. Objectives The objective of our study was to investigate the nature of inflammatory muscle involvement in a large sample of patients with pSS and concurrent myositis. Methods We conducted a French nation-wide survey among reference centers known for their experience in the field of systemic auto-immune diseases. Patients with physician-based diagnosis of pSS and inflammatory muscle involvement were included. They were categorized and subsequently described and compared according to the identification of an associated condition likely to explain inflammatory muscle involvement. Results 60 cases of myositis in patients with pSS were identified. Patients were mostly female (52/60) and pSS classification criteria were met in 43/60. Muscle biopsy was performed in 50/60 patients and showed inflammatory lesions in 80% of cases. An associated condition likely to explain myositis was identified in 31 patients: typical dermatomyositis rash (n=8), MCTD-specific antibodies (n=11), myositis specific antibodies (n=10), and systemic lupus erythematosus, scleroderma or ANCA associated vasculitis specific antibodies in favor of an overlap syndrome (n=7). Among the 29 patients without associated condition likely to explain myositis, 17 had a biopsy proven inflammatory muscle disease, showing histopathologic features consistent with IBM in 5 cases. 4 of the 5 patients with IBM histological features received corticosteroids and/or other immunosuppressive agents to treat muscle disease. Worsening of muscular involvement despite treatment was reported in these 4 patients. Comparative analysis showed a lower ESSDAI (p = 0.001) among patients without identified associated condition likely to explain myositis. Conclusion In most cases of concurrent pSS and myositis, inflammatory muscle involvement is explained by a context of overlap syndrome or has a histological pattern consistent with IBM. Extensive immunological investigations and muscle biopsy appear to be necessary in pSS patients with suspected inflammatory muscle involvement, in order to avoid misclassification and/or overtreatment. Disclosure of Interests Camille Houssais: None declared, Jean-baptiste Noury: None declared, Yves Allenbach: None declared, Laure Gallay: None declared, Florence Assan: None declared, Olivier Benveniste: None declared, Jonathan Broner: None declared, Pierre Duffau: None declared, Alexandra Espitia-Thibault: None declared, Anne Grasland: None declared, Sebastien SANGES: None declared, Gilles Hayem: None declared, Veronique LE GUERN: None declared, Nihal Martis: None declared, Kuberaka Marianpillai: None declared, Denis Mulleman Speakers bureau: Pfizer, Novartis, Grifols, Gaetane Nocturne: None declared, alain meyer: None declared, Valerie Devauchelle-Pensec Grant/research support from: Roche-Chugai, Speakers bureau: MSD, BMS, UCB, Roche, Divi Cornec: None declared, Dewi Guellec: None declared

Objective.To assess the prevalence of myofascial inflammatory lesions visible by magnetic resonance imaging (MRI) and their changes after tocilizumab (TCZ) therapy in active polymyalgia rheumatica (PMR).Methods.We conducted a posthoc analysis of data from the TENOR study of TCZ monotherapy in PMR. The 18 patients each received TCZ injections at weeks 0, 4, and 8. The shoulder and pelvic girdles were assessed at baseline then at weeks 2 and 12 using T1- and T2- short-tau inversion recovery–weighted MRI. Radiologists blinded to patient data assessed each muscle group for localized myofascial inflammation on baseline, Week 2, and Week 12 MRI. Reproducibility was estimated by having 2 radiologists assess the Week 2 MRI of 13 patients, then computing the κ coefficient.Results.For myofascial lesion detection, intraobserver reproducibility was almost perfect (κ = 0.890) and interobserver reproducibility was substantial (κ = 0.758). At baseline, all patients had at least 1 inflammatory myofascial lesion; sites involved were the shoulder in 10 (71.4%) patients, hip in 13 (86.7%), ischial tuberosity in 9 (60.0%), and pubic symphysis in 12 (80.0%). Sites involved at Week 12 were the shoulder in 8 patients (53.3%), hip in 5 (33.3%), ischial tuberosity in 1, and pubic symphysis in 3 (20.0%). At Week 12, of 103 muscle groups studied in all, 43 (41.7%) had no inflammatory lesions, compared to 33 at baseline (p = 0.002); improvements were noted in 66 (64.1%) muscle groups, worsening in 2 (1.9%), no change in 35 (34.0%; p = 0.034).Conclusion.Localized myofascial inflammatory lesions are common in recent-onset PMR and improve during TCZ therapy. Clinicaltrials.gov (NCT01713842).

Background:The SARS-CoV-2 pandemic has induced an exceptional sanitary crisis, potentially having an impact on treatment continuation, for juvenile idiopathic arthritis (JIA) patients receiving immunosuppressive therapies. In France, after the first lockdown from March to May 2020, many parents and children were then also concerned about whether reopening plans for school could ensure the safety of students, despite data concerning children with COVID-19 seem reassuring, and very few of them develop severe forms of the disease (1, 2, 3).Objectives:Our objectives were to evaluate the impact of the COVID-19 pandemic on the therapeutic management of JIA, the frequency of returning to school after the first lockdown period and the prevalence of SARS-CoV-2 infection at the time of the survey.Methods:JIA patients under 18 years of age, usually treated with disease-modifying anti-rheumatic drugs (DMARDs) were prospectively included during their outpatient visit and completed a standardized questionnaire. Data regarding the general characteristics of the participants, medical history, SARS-CoV-2 infection, characteristics of JIA subtypes and treatment modifications were collected.Results:A total of 173 patients from 8 different expert centers were included between May and August 2020. Their mean age was 11.6 years (± 4.1 years), and most of them 31.2% (54/173) had a rheumatoid factor-negative polyarticular JIA. Fifty percent (86/172) were treated with methotrexate, and 72.5% (124/171) were treated with bDMARDs. DMARD treatment modification in relation to the pandemic was observed in 4.0% (7/173) of participants, our results reflect good adherence of the patient/parents to their immunosuppressive treatments. 49.1% (81/165) of the patients did not return to school due to a personal/parental decision in 69.9% (55/81) of cases, due to anxiety of the patient/parents regarding COVID-19. Two patients were diagnosed positive for SARS-CoV-2 infection.Conclusion:This study suggests that JIA patients treated with DMARDs continued their treatment during the pandemic. In contrast, parents’ reluctance was a major obstacle for returning to school. Therefore, more solidified school reopening strategies should be developed.References:[1]Filocamo G, et al. “Absence of severe complications from SARS-CoV-2 infection in children with rheumatic diseases treated with biologic drugs.” J Rheumatol. 25 avr 2020;[2]Tagarro A, et al. “Screening and Severity of Coronavirus Disease 2019 (COVID-19) in Children in Madrid, Spain.” JAMA Pediatr. 8 avr 2020;[3]Lu X, et al. « SARS-CoV-2 Infection in Children”. N Engl J Med. 23 avr 2020;382(17):1663-5.Acknowledgements:We would like to thank all the participants involved in this clinical research and six medical students who helped completing the forms: Margaux Blondel, Alice Bonnod, Marie Desval, Béatrice Dordain, Gabrielle Fagnet, and Madouc De Saint Martin Pernot.Disclosure of Interests:None declared

Introduction L’existence d’un antecedent familial de polyarthrite rhumatoide (PR) constitue l’un des principaux facteurs de risque de developper la maladie. Plusieurs etudes conduites chez des patients presentant une PR etablie ont montre une association entre la presence d’un antecedent familial de PR et certaines caracteristiques de la maladie telles que le statut serologique, l’âge de debut et possiblement le profil de reponse a certains des traitements de fond de la maladie. A ce jour, l’impact d’un antecedent familial de PR dans le contexte particulier de la polyarthrite debutante n’a pas ete etudie. L’objectif de cette etude ancillaire de la cohorte ESPOIR etait de determiner, chez des patients presentant une polyarthrite debutante, l’impact d’un antecedent familial de PR sur la presentation initiale du rhumatisme inflammatoire, le diagnostic final et le devenir a long terme de la pathologie. Patients et methodes Cette etude a ete conduite chez l’ensemble des patients inclus dans la cohorte ESPOIR entre 2002 et 2005. Dans le cadre de cette cohorte prospective, des patients âges de 18 a 70 ans ont ete recrutes sur la base de la presence d’au moins 2 articulations gonflees depuis plus de 6 semaines et moins de 6 mois, l’absence d’utilisation prolongee de la corticotherapie et l’absence de recours prealable a un traitement de fond antirhumatismal. De plus, le diagnostic de PR devait etre considere comme certain ou probable par le rhumatologue referent. Dans le cadre de cette etude ancillaire, les patients ont ete stratifies en deux groupes selon l’identification d’un antecedent familial de PR lors de l’evaluation systematique a la visite d’inclusion. Les donnees concernant la presentation initiale du rhumatisme inflammatoire, le diagnostic final (avec un recul de 2 ans) et l’evolution a long terme (a 5 ans) ont ete comparees entre les deux groupes de patients. Les analyses statistiques ont ete realisees a l’aide du logiciel SPSS, ajustees sur les facteurs de confusion potentiels pour ce qui concerne les variables dichotomiques relatives au devenir des patients. Resultats Cent quinze patients (14,1 %) ont rapporte un antecedent familial de PR. En ce qui concerne la presentation initiale du rhumatisme inflammatoire les patients ayant rapporte un antecedent familial de PR presentaient de facon plus frequente des manifestations extra-articulaires (51,8 % versus 39,6 %, p = 0,01). En ce qui concerne le diagnostic final, les patients ayant rapporte un antecedent familial avaient de facon moins frequente un diagnostic alternatif plausible a la PR d’apres le rhumatologue referent (28,3 % versus 42,1 %, p-ajuste = 0,02). En ce qui concerne le devenir a long terme, le recours a un traitement de fond biologique etait plus frequent chez les patients ayant rapporte un antecedent familial (24,7 % versus 14,8 %, p-ajuste = 0,02), donnees etayees par des analyses supplementaires de survie. Conclusion Cette etude demontre que dans le contexte de la polyarthrite debutante, l’identification d’un antecedent familial de PR constitue une donnee cliniquement pertinente, contenant des informations en rapport avec la certitude diagnostique et l’indication future a un traitement de fond biologique.

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.

Background Myositis prevalence in primary Sjogren’s syndrome (pSS) and whether it is associated with peculiar extra-muscular involvement and/or a biological profile is unknown. Objectives To refine prevalence and characteristics of patients with pSS and myositis. Methods In the national multicenter prospective cohort ASSESS (395 patients with pSS with a 5 year-prospective follow-up), patients with suspected myositis were identified. Their charts were reviewed and the patients were compared with the rest of the cohort. Results Myositis was suspected in 38 patients (2 men, 36 women, 5829–78 yr old) because of myalgia and/or exercise intolerance (n=38) along with high blood CK level (n=28). As compared with the rest of the cohort, they had higher patient-reported signs including pain VAS, dryness VAS and patient reported index ESSPRI (6.3 vs 5.3, p=0.007). In contrast, proportion of systemic involvements (76.3% vs 70.9%, p=0.57) and systemic disease activity measured by the ESSDAI, without taking the muscular domain into account (2 vs 3, p=0.93), were not different from patients without suspected myositis. Demographic characteristics and disease duration were also similar. Electromyographic recording and/or muscle histology performed in these 38 patients objective muscular involvement in only 4 patients (1% of the cohort). - Three patients were diagnosed with inclusion body myositis (sIBM). Accordingly, age at first sign was 49 years,49–60 muscle disease had progressive onset as suggested by the 11 years1–28 delay between first muscle sign and diagnosis. CK level was - One pSS patient with myositis was a 39 years old woman with polymyositis. She developed proximal muscle weakness, CK was 750UI/L and muscle biopsy found endomysial infiltrate. Muscle force and CK level normalised with immunomodulatory drugs. Compared to the rest of the cohort, patient with myositis had longer disease duration at inclusion (15 vs 5 year, p=0.01) and tended to be younger at pSS diagnosis (41.5 vs 53 year, p=0.07). They had more frequent history of systemic manifestations (100% vs 65.0%, p=0.011) but ESSDAI at baseline (2 vs 3, p=0.56) and the biological characteristics were not different from patients without myositis. In contrast with the patients with suspected but not confirmed myositis, patient-reported signs (i.e. fatigue, pain, dryness and ESSPRI score) were similar to patients without myositis. Conclusions Myositis is frequently suspected in patients with pSS, especially when patients-reported signs are particularly disabling. Myositis occurs very infrequently (1% of the cohort), in patients with longer disease duration. sIBM was the most predominant subset of myositis in the present study. Disclosure of Interest None declared

Background In everyday practice, myofascial lesions are not usually evaluated on MRIs obtained for patients with PMR. Objectives To assess the prevalence of myofascial inflammatory lesions visible by magnetic resonance imaging (MRI) and their changes after tocilizumab therapy in active polymyalgia rheumatica (PMR). Methods We conducted a post hoc analysis of data from the TENOR study of tocilizumab monotherapy in PMR.1 The 18 patients each received tocilizumab injections at weeks 0, 4, and 8. The shoulder and pelvic girdles were assessed at baseline then at weeks 2 and 12 using T1- and T2-STIR-weighted MRI. Radiologists blinded to patient data assessed each muscle group for myositis and fasciitis on baseline, week-2, and week-12 MRIs. Reproducibility was estimated by having two radiologists assess the week-2 MRIs of 13 patients then computing the kappa coefficient. Results For myofascial lesion detection, intraobserver reproducibility was almost perfect (κ=0.890) and interobserver reproducibility was substantial (κ=0.758). At baseline, all patients had at least one inflammatory myofascial lesion (example on right shoulder fig 1); sites involved were the shoulder in 10 (71.4%) patients, hip in 13 (86.7%), ischial tuberosity in 9 (60.0%), and pubic symphysis in 12 (80.0%). Sites involved at week 12 were the shoulder in 8 (53.3%) patients, hip in 5 (33.3%), ischial tuberosity in 1, and pubic symphysis in 3 (20.0%). At week 12, of 103 muscle groups studied in all, 43 (41.7%) had no inflammatory lesions, compared to 33 at baseline (Mac Nemar; p Conclusions Myositis and fasciitis are common in recent-onset PMR and improve during tocilizumab therapy. They could be used for the diagnosis in routine practice and as criteria of outcome evaluation. References [1] Devauchelle-Pensec V, Berthelot JM, Cornec D, Renaudineau Y, Marhadour T, Jousse-Joulin S, et al. Efficacy of first-line tocilizumab therapy in early polymyalgia rheumatica: a prospective longitudinal study. Ann Rheum Dis2016;75:1506–1510. Disclosure of Interest J. P. Laporte: None declared, F. Garrigues: None declared, A. Huwart: None declared, S. Jousse-Joulin: None declared, T. Marhadour: None declared, D. Guellec: None declared, D. Cornec: None declared, V. Devauchelle-Pensec: None declared, A. Saraux Grant/research support from: Chugai, Speakers bureau: Chugai

Background Technological advances have improved phenotypical characterisation of blood cells, and flow cytometry is currently used in haematology, infectious disease, systemic auto-immune diseases. Abnormalities of blood B cell subset profile might provide a useful diagnostic tool in systemic auto-immune diseases, especially for primary Sjogren’s Syndrome1 in which the activated B cells to memory B cells ratio is increased. Nevertheless, we observed that some patients suffering from chronic infection had lymphocytes disturbances similar to those observed in primary Sjogren’s Syndrome. Objectives Whipple’disease (WD) is a rare, systemic, disease caused by intracellular gram positive bacterium, Tropheryma Whipplei (TW). No previous study evaluated the role of B cells in WD. The aim of this study was to analyse whether the circulating blood B cell subset disturbances is characteristic of WD. Methods We collected characteristics of all patients coming for inflammatory rheumatism in our rheumatology department between April 2010 and December 2016. All of them had systematically routine examination, immunological tests, lymphocyte subsets in peripheral blood by flow cytometric analysis. We selected among this population those patients who also had PCR for TW for suspicion of WD, and compared the distribution of lymphocyte subsets of those with and without WD. Then, we evaluated their diagnostic value for WD using a ROC curve. Results Among 3494 patients with inflammatory rheumatism, 121 patients (212 visits) had a suspicion of WD and the diagnosis of WD was retained by an expert rheumatologist for 9 (7.4%) (22 visits). T cells and NK cells were not different whereas percentage of circulating memory B cells (IgD-CD38low) was lower (18.0%±9.7% vs 26.0±14.2%, p=0.041) and the ratio of activated B cells to memory B cells higher (4.4±2.0 vs 2.9±2.2, p=0.023), in patients compared with controls. More precisely, the analysis of the frequency of peripheral blood B cells showed that IgD +CD27 – naive B cells were higher (66.2%±18.2% vs 54.6±18.4%, p=0.047) and IgD-CD27 +switched memory B cells lower (13.3%±5.7% vs 21.4±11.9%, p=0.023), in patients compared with the controls. The best diagnostic value was obtained for the IgD +CD27- naive B cells (cut off 70.5, sensitivity 73%, specificity 80%). Conclusions Our study provides data on blood B cells disturbances and a first step towards understandings of immunological abnormalities in WD. These disturbances provide guidance for diagnosis and allow physiopathological hypothesis. Reference [1] Binard A, Le Pottier L, Devauchelle-Pensec V, Saraux A, Youinou P, Pers J-O. Is the blood B-cell subset profile diagnostic for Sjogren syndrome?Ann Rheum Dis2009;68(9):1447–52. Disclosure of Interest None declared

Background This study assessed inflammatory changes using ultrasound (US) and magnetic resonance imaging (MRI) in patients taking tocilizumab for polymyalgia rheumatica (PMR). Methods Eighteen patients were included in the prospective open-label TENOR study and received three tocilizumab infusions, without corticosteroids. B-mode and power Doppler US and MRI (T1 and T2-short time inversion recuperation weighted sequences) of the hips and shoulders were performed at weeks 0, 2, and 12. Subacromial, trochanteric, and iliopsoas bursitis and intraarticular glenohumeral and coxofemoral effusions/synovitis were scored from 0 to 3. Changes over time and US–MRI correlations were evaluated. Results At baseline, the proportions of shoulders and hips with bursitis were 93 and 100% by MRI and 61 and 13% by US; and the corresponding proportions for intraarticular effusions/synovitis were 100 and 100% by MRI and 57 and 53% by US. Imaging findings did not improve during the first two treatment weeks. From baseline to week 12, bursitis improved significantly at all four joints by MRI (P = 0.005) and US (P = 0.029) and intraarticular effusions/synovitis by US only (P = 0.001). The proportion of abnormalities that improved by week 12 was 42% by MRI and 37% by US. MRI detected bursitis in a larger proportion of hips (73% versus 13%) and US in a larger proportion of shoulders (57% versus 28%), whereas no difference was found for intraarticular effusions/synovitis. At baseline, agreement between US and MRI findings was poor. Conclusions US and MRI showed significant improvements in inflammatory lesions during tocilizumab treatment of PMR.

Neurological manifestations of primary Sjögren's syndrome are multiple and appear frequently. Depending on data analysis, patient recruitment, and diagnosis criteria used to defined primary Sjögren's syndrome or neurological manifestations, the estimated prevalence is between 0 and 70%. Peripheral neurological complications seem the most common, particularly sensory-motors axonal neuropathies. Neuronopathy seems to be the most specific neurological complication of primary Sjögren's syndrome. Central manifestations of primary Sjögren's syndrome are not uncommon, but the neurological complication's spectrum is not well defined. Neuromyelitis optica is regularly found among central complications. To conclude, although central and peripheral complications of primary Sjögren's syndrome are difficult to assess, partly because of the wide spectrum of possible manifestations, it is around 20%. Neuronopathy is still the most specific complication.

Resume Les atteintes neurologiques du syndrome de Sjogren primitif sont multiples et semblent frequentes. Elles atteignent 0 a 70 % des patients selon le mode d’analyse des donnees, le mode de recrutement des patients et les criteres diagnostic utilises pour definir le syndrome de Sjogren primitif et l’atteinte neurologique. L’atteinte du systeme nerveux peripherique semble la plus frequente, particulierement la polyneuropathie axonale sensitivo-motrice. La neuronopathie semble la plus specifique du syndrome de Sjogren primitif. Les atteintes du systeme nerveux central ne sont pas rares au cours de la maladie mais le spectre des atteintes neurologiques du syndrome de Sjogren n’est pas bien defini. Le syndrome de Devic est regulierement retrouve parmi les atteintes neurologiques centrales. Pour conclure, bien que la prevalence des atteintes neurologiques centrales et peripheriques du syndrome de Sjogren primitif soit difficile a evaluer du fait du large spectre des atteintes possibles, elle avoisine 20 %. La neuronopathie reste l’atteinte la plus specifique du syndrome de Sjogren primitif.

Objective The PMR activity score (PMR-AS) includes the CRP value, which may be lacking or invalid owing to anti-IL-6 therapy. Our objective was to develop alternatives to PMR-AS that do not require CRP. Methods We used the Club Rhumatisme et Inflammation (CRI; 89 patients with PMR) and the Tolerance and Efficacy of tocilizumab iN pOlymyalgia Rheumatica (TENOR; 20 patients with recent-onset PMR naive to glucocorticoid who received three tocilizumab infusions, at weeks 0, 4 and 8, followed by prednisone from weeks 12 to 24) cohorts. In the CRI cohort, we evaluated correlations between PMR-AS items to select the best item for imputing CRP. Then we calculated the PMR-AS with (PMR-AS) and without (clin-PMR-AS) CRP and we used the linear regression between PMR-AS and clin-PMR-AS to obtain CRP-imputed (CRP-imp) PMR-AS. Finally, we evaluated agreement between clin-PMR-AS, CRP-imp PMR-AS, PMR-AS and ESR-PMR-AS in the TENOR cohort during tocilizumab therapy. Results In the CRI cohort, agreement between PMR-AS and clin-PMR-AS was excellent (κ = 0.90). Linear regression between PMR-AS and clin-PMR-AS [CRP-imp PMR-AS = 1.12(clin-PMR-AS)+0.26] allowed us to build the CRP-imp PMR-AS. Mean (s.d.) values were as follows: 8.40 (9.76) for PMR-AS, 7.24 (8.58) for clin-PMR-AS and 7.84 (9.61) for CRP-imp PMR-AS. CRP-imp PMR-AS agreed more closely with PMR-AS than did clin-PMR-AS. The results in the TENOR cohort confirmed that CRP-imp PMR-AS or ESR-PMR-AS could be used. Conclusion Alternatives to the PMR-AS obtained without CRP can be used to monitor PMR activity in everyday practice in patients without available CRP values and in those receiving IL-6 antagonist therapy.

Background New consensual classification criteria for primary Sjogren9s syndrome (pSS) have been recently developed and endorsed by ACR and EULAR. They differ substantially from previously used AECG criteria in that they consider systemic involvement (defined as ESSDAI score ≥1) as well as sicca symptoms as entry criteria before applying a weighted score. Evaluation of the concordance and differences between the two sets of criteria in independent patient populations is mandatory to establish how future clinical studies using the new criteria will be comparable to previously published studies. Major salivary gland ultrasonography (SGUS) has demonstrated promising diagnostic performance in previous studies, but was not included in these new classification criteria. Methods This cross-sectional study was conducted in the monocentric Brittany cohort (DIApSS cohort) of patients with suspected pSS (sicca symptoms, parotidomegaly or extraglandular manifestations suggestive of pSS). All patients had standardized clinical examination, basic biology, immunological tests and minor labial salivary gland biopsy. SGUS in mode B was performed by the same experienced operator, who was blinded to the diagnosis. Agreement between the two sets of criteria was assessed using Cohen9s κ coefficient and the characteristics of discordant patients were detailed. Results 290 patients were prospectively included between 2006 and 2016. Mean age was 55.6±13.2 years, 92.1% were female and mean duration of the symptoms 6.0±6.6 years. More patients fulfilled ACR/EULAR criteria (n=125, 43.1%) than AECG criteria (n=114, 39.3%). 114 patients (39.3%) fulfilled both criteria, 11 (3.8%) fulfilled ACR/EULAR only, 0 AECG only and 165 (56.9%) none of the criteria. Concordance between both criteria was good (kappa=0.9). Compared to patients fulfilling both criteria, patients fulfilling ACR/EULAR but not AECG criteria (n=11) had similar age, similar symptom duration, but less frequent sicca symptoms (eye dryness 18.2% versus 96.5%; mouth dryness 54.5% versus 97.4%, p Conclusions Agreement between AECG criteria and new ACR/EULAR criteria is good suggesting that they select quite similar patients. ACR/EULAR criteria display a slightly higher sensitivity and are able to detect more patients with systemic involvement, but some of these patients did not have pSS according to the physician diagnosis. As previously demonstrated for AECG criteria, SGUS inclusion into ACR/EULAR criteria may further enhance their sensitivity. Disclosure of Interest None declared

Background The ultrasonography of salivary glands (USSG) has proved its utility in diagnosing and following primary Sjogren patient9s (pSS) (1,2). The evaluation of disease activity is still of interest and can be studied by assessing the inflammatory status of SG using US Doppler. Objectives To evaluate the vascularization of salivary glands, and particularly the parotid gland (PG) using Pulsed Doppler color ultrasonography (USSGPD) in patients complaining of xerostomia before and after treatment by pilocarpine. Methods We prospectively included patients with objective dry mouth syndrome (using salivary flow rate) at Brest University Hospital (DiapSS cohort). The vascularization was assessed by the resistive index (RI) at the left parotid. Only patients with pathological RI ( Results Among the 19 patients included, 11 received pilocarpine treatment for the whole 3 months period, 6 of the 8 remaining patients stopped the pilocarpine due to side effects. Among the 11 patients with a follow-up evaluation at 3 months, 5 had pSS according to AECG criteria. The differences of RI before and after lemon stimulation were on average of -0.04 at baseline and -0.04 at M3. The sum of ultrasound9s grades average of the four glands was 3.47 at M0 and 4.18 at M3. The non-simulated salivary flow was on average of 1.96 mL/mn at M0 and 5.23 mL/mn at M3, whereas the average of stimulated salivary flow was 2.84 mL/mn at M0 and 8.51 mL/mn at M3. None of these observed differences were statistically significant before and after 3 months of treatment by Pilocarpine: RI before and after lemon stimulation (p=0.953), the sum of the four glands9 grades (p=0.858), the non-stimulated (p=0.26) and stimulated salivary flow (p=0.139). Concerning the 3 patients with Sjogren9s syndrome, there was no differences using RI before and after treatment but the RI was lower in this subgroup compared to the xerostomia patients.The study was marked by a large number of pilocarpine9s discontinuation (31%) due to adverse effects. Conclusions Preliminary results showed no significant differences between the 4 gland9s grade, ultrasound9s RI and salivary non and stimulated flow before and after three months of pilocarpine9s treatment. The vascularisation of salivary glands could be an opportunity to follow our treated patients with Sjogren9s syndrome or with xerostomia but more studies are needed to prove the interest of this procedure. References Cornec D, et al. Contribution of salivary gland ultrasonography to the diagnosis of Sjogren9s syndrome: toward new diagnostic criteria? Arthritis Rheum. 2013 Jan;65(1):216–25. Jousse-Joulin S et al. Brief Report: Ultrasonographic Assessment of Salivary Gland Response to Rituximab in Primary Sjogren9s Syndrome. Arthritis Rheumatol. 2015 Jun;67(6):1623–8. Acknowledgements We thank the Professor luc Bressollette and Muriel Korpet for their contribution in the study. Disclosure of Interest None declared

Resume L’actualisation 2010 des recommandations ASAS/EULAR pour le traitement de la spondylarthrite ankylosante (SA) indique qu’un traitement continu par anti-inflammatoires non steroidiens (AINS) devrait etre utilise en priorite chez les patients ayant une maladie symptomatique avec une activite persistante. L’objectif de notre mise au point a ete d’evaluer si un traitement continu par AINS ameliore le controle de la maladie et a un impact sur la progression radiographique en comparaison avec une utilisation a la demande. Nous avons egalement evalue la tolerance des deux modalites d’utilisation des AINS. Methodes Nous avons realise une revue systematique en recherchant dans les bases de donnees PubMed et Embase, en utilisant 2 combinaisons de termes MeSH, afin de comparer les traitements AINS en continu et a la demande, en ce qui concerne le controle de la maladie, la progression radiographique et la tolerance. Resultats La seule etude evaluant l’impact d’une utilisation en continu des AINS sur le controle de la maladie n’a pas montre de difference significative par rapport a une utilisation a la demande. Dans quatre etudes, le traitement continu a ete retrouve associe a une moindre progression radiographique, evaluee par le score m-SASSS (modified Stoke Ankylosing Spondylitis Spinal Score) dans 3 etudes. Concernant la tolerance d’un traitement continu et d’un traitement a la demande par le celecoxib, trois etudes, deux realisees dans l’arthrose et une dans la SA, n’ont pas retrouve de difference significative pour les effets indesirables habituels des coxibs. Conclusion Plusieurs etudes mettent en evidence une moindre progression structurale grâce a l’utilisation en continu des AINS dans la SA. Aucune etude n’a demontre une superiorite du traitement continu pour le controle des symptomes de la maladie. Le traitement AINS en continu (au moins pour les coxibs) ne modifie pas la tolerance par rapport a un traitement a la demande.

Objective The 2010 update of ASAS/EULAR recommendations for managing ankylosing spondylitis (AS) specify that continuous non-steroidal anti-inflammatory drug (NSAID) treatment should be preferred in patients with persistently active, symptomatic disease. Here, our objective was to assess whether continuous NSAID therapy improves disease control and influences radiographic progression compared to on-demand therapy. We also assessed the safety profiles of both regimens. Methods We performed a review by searching the PubMed and Embase databases using two MeSH term combinations to compare continuous and on-demand NSAID therapy in terms of disease control, radiographic progression, and safety. Results The only study evaluating the impact of continuous NSAID therapy on disease control showed no significant difference with on-demand therapy. In four studies, continuous treatment was associated with slower radiographic progression, as assessed in three studies using the modified Stoke Ankylosing Spondylitis Spinal Score (m-SASSS). Three studies compared the safety of continuous and on-demand celecoxib, two in osteoarthritis and one in AS, and found no significant differences regarding the usual side effects of Cox-2 inhibitors. Conclusions Several studies showed slower radiographic progression with continuous NSAID therapy in AS. No studies demonstrated superiority of continuous NSAID therapy regarding symptom control. Continuous NSAID therapy (at least with Cox-2 inhibitors) does not modify safety compared to on-demand therapy.

Primary Sjogren’s syndrome (pSS) is an autoimmune disease. Eleven classification or diagnostic criteria have been published for pSS since 1965. The American–European Consensus Group criteria have a better specificity than its predecessor; however, they have been criticized. New classification criteria for pSS were proposed by the ACR in 2012. The fact that two different criteria to classify patients with Sjogren’s syndrome could be available may introduce some difficulties. New criteria are being discussed by expert groups from the ACR and the European League Against Rheumatism to obtain consensual classification criteria. We describe the objective tests that are useful in practice for a positive diagnosis and assessment of pSS. Furthermore, we present other tests that might be included in future classification criteria.

International audience; OBJECTIVES: To assess the diagnostic value of minor salivary gland biopsy (MSGB) for primary Sjögren's syndrome (pSS). METHODS: Systematic review of studies retrieved from PUBMED and EMBASE using the terms 'salivary glands' AND 'Sjögren's syndrome' AND 'biopsy', conducted in patients with suspected pSS, and defining positive biopsies as a focus score (FS)≥1. Sensitivity and specificity of MSGB were abstracted from the articles or calculated when possible. RESULTS: Of 238 publications identified initially, 9 were included in the study. MSGB sensitivity ranged from 63.5% to 93.7% and specificity from 61.2% to 100%. Specificity was >89% in six studies. An attempt to separate patients with and without pSS without using MSGB findings or via clinical re-evaluation was made in only two studies, in 73 and 120 patients, respectively, with sicca syndrome in the first study and suspected pSS in the other. The reference standard for diagnosing pSS was a set of criteria that did not include MSGB in the first and patient re-evaluation by three experienced rheumatologists who were aware of MSGB findings in the other. In these studies, sensitivity was 63.9% and 85.7% and specificity was 91.9% and 89.7%, respectively. CONCLUSIONS: Few published studies have evaluated the diagnostic usefulness of MSGB in pSS. Only two studies used a methodology that precluded circular reasoning. Our study indicates a lack of information about the diagnostic value of MSGB. Specificity and positive predictive values (PPV) are high and sensitivity is variable.

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.

Joint Bone Spine - In Press.Proof corrected by the author Available online since mardi 11 decembre 2012

Joint Bone Spine - In Press.Proof corrected by the author Available online since jeudi 26 juillet 2012

Introduction L’oligoarthrite est une atteinte inflammatoire de 2 ou 3 articulations. Elle a de nombreuses etiologies qu’il faut s’acharner, a l’aide de l’anamnese, de la clinique, des examens paracliniques, a rechercher, gage d’un traitement etiologique adapte. Nous rapportons ici une etiologie exceptionnelle d’oligoarthrite associee a une eruption cutanee. Observation Monsieur T., educateur canin de 50 ans, consulte pour fievre depuis 72 heures associee a une douleur du coude gauche depuis 24 heures. Ses comorbidites sont une silicose, un asthme, des lombalgies chroniques, une gonarthrose bilaterale traitees par formoterol, montelukast, ebastine, budesonide, sulfate de terbutaline. L’examen retrouve une fievre a 40 °C, une arthrite du coude gauche et du genou droit mais ni souffle cardiaque, ni lesion cutanee. Le lendemain, il presentera trois lesions d’erytheme annulaire centrifuge. Devant cette oligoarthrite aigue febrile associee a un syndrome inflammatoire biologique avec une hyperleucocytose et l’absence de signes radiographiques notables, nous realisons un bilan etiologique. L’analyse du liquide articulaire du coude retrouve un liquide inflammatoire avec une predominance de PNN, sans microcristaux ni germe a l’examen direct et a la culture avec une PCR ARN 16S negative. Les serologies : Campylobacter, Yersinia pseutobuberculosis et Y. enterocolitica, Chlamydia trachomatis et C. pneumoniae, Mycoplasma pneumoniae, Francisella tularensis, Treponema pallidum, Borrelia burgdorferi, VIH, parvovirus B19, hepatitis B et C virus reviennent negatives. De meme que les marqueurs de rhumatisme inflammatoire (AAN positifs au 1/320e mouchetes sans specificite, anti-CCP, FR et ANCA negatifs). L’origine iatrogene est ecartee. C’est apres 7 jours de culture qu’une des cinq hemocultures revient positive a Capnocytophaga canimorsus. Cette infection est traitee par ceftriaxone (2 g IV/jour, 7 jours) puis levofloxacine (500 mg × 2/jour per os 4 semaines) avec efficacite. Le patient ne presente pas de facteur majeur d’immunodepression. Seul un diabete a ete decouvert, mais il a une exposition professionnelle patente avec notion de lechage de plaies du visage par ses chiens. Discussion C. canimorsus est un Bacille gram negatif [1] a culture lente [1] . Difficile a identifier [1] , on peut dorenavant s’aider de la PCR universelle [1] ou de la spectrometrie de masse. Contrairement aux autres Capnocytophaga d’origine bucco-dentaire et frequemment opportunistes, C. canimorsus est un germe commensal de la gueule du chien mais aussi du chat et du lapin qui se transmet par morsure, griffure et autres contacts [1] . Son traitement repose sur une antibiotherapie notamment par penicilline. T. Butler a recense 484 cas dans sa revue de la litterature en 2015 [1] , il s’agit en general d’hommes [1] de plus de 50 ans [1] en contact avec des chiens ou des chats [1] , presentant comme autres facteurs de risque une splenectomie [1] ou une asplenie fonctionnelle [1] et/ou une consommation excessive d’alcool [1] . Cependant, la plupart des cas decrits sont survenus chez des patients apparemment en bonne sante [1] . L’infection par C. canimorsus associe des symptomes varies, domines toutefois par le sepsis et la meningite [1] , et se place dorenavant juste derriere Pasteurella multocida pour les infections apres morsure de chien et Streptococcus pneumoniae pour le sepsis chez les splenectomises [1] . Par contre, l’atteinte rhumatologique reste exceptionnelle : une spondylodiscite chez un homme de 54 ans [2] , une bi-arthrite des genoux chez un homme de 59 ans a distance d’une arthroplastie [5] , une mono-arthrite du genou avec exantheme chez un homme de 59 ans apres arthroplastie [4] et une mono-arthrite de la premiere metatarso-phalangienne chez un homme de 67 ans [3] . Chez tous [2] , [3] , [4] , [5] , un contact avec un chien a ete retrouve. Cette observation a ete l’occasion de recenser sur les 15 dernieres annees les infections a C. canimorsus diagnostiquees dans notre CHRU soit 5 cas : une endocardite avec purpura, deux plaies infectees, une spondylodiscite et l’oligoarthrite avec erytheme annulaire centrifuge. Chez quatre, un contact avec un chien a ete retrouve et chez un, un contact avec un chat. Trois patients presentaient un diabete et un etait splenectomise. Conclusion Nous rapportons ici une cause inhabituelle d’oligoarthrite avec eruption cutanee sous la forme d’erythemes annulaires centrifuges. Il s’agit de l’expression clinique rare d’une bacteriemie a C. canimorsus, chez un patient en bonne sante apparente mais ayant une exposition patente. Cette observation rappelle la necessite d’un bilan exhaustif des oligoarthrites incluant la PCR16S sur le liquide de ponction, la pratique d’hemocultures systematiques sur milieu performant cultivees au moins 7 jours.

Background Primary Sjogren syndrome (pSS) is an autoimmune disease which may be associated to several systemic manifestations. Neurological manifestations seem to be frequent but prevalence is inconstant in literature (1). Objectives To assess neurological involvement prevalence in a national multi-center prospective cohort and to study epidemiological aspects of neurological involvement. Methods The ASSESS (Assessment of Systemic Signs and Evolution in Sjogren9s Syndrome) cohort is a French, multi-centre, prospective cohort, set up in 2006, including 395 pSS patients fulfilling American- European Consensus Criteria (AECG). Demographic and clinical data were compared between patients with and without neurological manifestations, and between patients with peripheral nervous system (PNS) manifestations, central nervous system manifestations (CNS) and no manifestations. Medical records of 26 patients with CNS manifestations and/or cranial nerve involvement were reviewed by both local investigator and our group to confirm or invalidate the association between pSS and the neurological manifestations. Results Data at inclusion were available for 392 patients. Mean follow-up was 33.8 months. Mean age at inclusion was 58 (±12) years. Mean age at diagnosis was 51 (±12) years. Seventy-four of 392 patients (65 females and 9 males) presented neurological manifestations (18.9%). Sixty-three had PNS manifestations (16%) and 14 had CNS manifestations (3.6%). Most frequent peripheral manifestations were pure sensitive neuropathy (9.2%) and sensory-motor neuropathy (5.3%). Only 2 patients (0.6%) had isolated ganglionopathy. Most frequent central manifestations were cerebral vasculitis (5 patients, 1.3%) and myelitis (4 patients, 1.0%) Patients with neurological involvement had more severe disease according to the ESSDAI scoring system, 8.9 (±6.6) versus 3.9 (±4.8). They had more frequently immunomodulatory/immunosuppressive drugs, 32.4% (24/74) versus 13.8% (44/318) (p=0003). Vasculitis was seen in 21 of 73 patients with neurological manifestations (28.8%) and in 30 of 318 patients without (9.4%) (p Conclusions The prevalence of neurological manifestations in the ASSESS cohort was about 20% and is comparable to data in the literature. Isolated ganglionopathy, which is frequently considered as the most specific neurological manifestation in pSS, was rare ( References Carvajal Alegria G et al. Joint Bone Spine. 2014 Jun 20 Disclosure of Interest None declared

Background Primary Sjogren9s syndrome (pSS) is a systemic autoimmune disease characterized by B-cell hyperactivation. In some patients, multiple autoantibodies develop, including anti-neutrophil cytoplasmic antibody (ANCA), which could lead to the development of ANCA-associated vasculitis (AAV). Objectives To describe clinical presentation, therapy and prognosis of patients diagnosed with both pSS and AAV. Methods Nation-wide survey in France collecting information on cases of AAV associated with pSS. The study was completed by a systematic review of the literature. Results This work identified 6 new cases of coexisting pSS and AAV: 2 microscopic polyangiitis (MPA), 2 MPO-ANCA renal-limited AAV, 1 granulomatosis with polyangiitis (GPA), and 1 eosinophilic granulomatosis with polyangiitis (EGPA). The systematic literature search identified 15 previously published cases. Among the 21 patients, 18 were females. Mean age at diagnosis of AAV was 64±10 years with mean pSS duration of 76±122 months. All individuals with pre-existent pSS experienced at least one extra-glandular manifestation attributable to pSS before AAV diagnosis. p-ANCA with anti-MPO specificity were found in 80% (16/20), c-ANCA with anti-PR3 specificity in 9.5% (2/20) and isolated c-ANCA in 14.3% (3/21). Vasculitis involved kidneys (n=13), lungs (n=6), peripheral nerves (n=5), skin (n=5), central nervous system (n=2), small bowel (n=1), muscle (n=1), and sinuses (n=1). The mean follow-up duration of AAV was 20.5 (±31.1) months. A favourable response to treatment was observed in 76.2% of cases (16/21), stabilization in 19% of cases (4/21) and one death was reported. Conclusions This work shows that AAV may occur in patients with pSS. These are most commonly p-ANCA associated vasculitis with anti-MPO specificity. The AAV may reveal an underlying pSS or arise during its evolution. In this latter case, its occurrence appears to be correlated with extra-glandular manifestations of pSS. Disclosure of Interest None declared

Background Osteoporosis (OP) is a frequent complication of ankylosing spondylitis (AS), even in early stages of the disease (1). Assessment of bone mineral density (BMD) remains complicated in these generally male and young patients and consequently, therapeutic indications and issues are not clear (2). Objectives The objective of this study is to assess the prevalence of low BMD and to detect the predictive clinical or biological parameters of OP in AS patients. Methods We searched Pubmed, EMBASE and Cochrane databases to perform a meta-analysis of prevalence of low bone mineral density in AS using data from new published observational studies or baseline data from longitudinal studies up to June 2014. The recommendations of PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses) were applied (3). The outcome was the BMD measurement assessed with dual x-ray absorptiometry (DEXA) at the lumbar spine or femoral neck in absolute value or T-score. Our comparator population in case-control studies was composed by sex and age-matched healthy subjects. The standard difference in means between AS patients and controls were estimated using DerSimonian and Laird random-effects models. Results Fourty-six studies were included and concerned 3420 patients with AS. At the femoral neck, the mean BMD was at 0.813 g/cm 2 (IC95% 0.784 – 0.842 g/cm 2 ) with T-score at -1.055 SD (IC95% -1.325-0.785 SD). At the lumbar spine, the mean BMD was at 1.020 g/cm 2 (IC95% 0.999 – 1.040) with T-score at –0.904 SD (IC95% -1.085-0.723). BMD was correlated with age (p=0.02) and AS duration (p=0.007). None statistically significant correlation between BMD and gender (p=0.61), BASDAI (p=0.43), BASFI (p=0.51), CRP (p=0.32), ESR (p=0.64), Body Mass Index (p=0.68) was found. In 21 case-control studies, BMD in AS patients (n=1271) was significantly decreased comparing to control group (n=1858) at the femoral neck (-0.814±0.134; p Conclusions This first meta-analyses confirmed significant increase of low BMD at the femoral neck in patients with AS compared with age-and-sex-matched healthy controls. This difference disappears at the lumbar spine. Reflection about the OP, vertebral fracture assessment and about the benefit of the OP treatment in AS should be supported and should promote future prospective studies. References Will R, Palmer R, Bhalla AK et al. Osteoporosis in early ankylosing spondylitis: a primary pathological event? Lancet. 1989 Dec 23-30;2(8678-8679):1483-5. Karberg K, Zochling J, Sieper J et al. Bone loss is detected more frequently in patients with ankylosing spondylitis with syndesmophytes. J Rheumatol. 2005 Jul;32(7):1290-8. Moher D, Liberati A, Tetzlaff J et al; PRISMA Group. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement.BMJ. 2009 Jul 21;339:b2535. Disclosure of Interest None declared

Background Congenital dislocation of the hip (CDH), which is one of the most common skeletal congenital anomalies, results from an abnormality of the seating of the femoral head in the acetabulum. Importance of misalignment of femoral head defines different phenotypes ranging from luxation to mild forms of acetabular dysplasia (AD). CDH is a multifactorial disease with a strong genetic component attested by ethnical predispositions and familial aggregation. Recently, several studies reported the association between functional polymorphism of the 59-untranslated region of GDF5 (Growth/Differentiation Factor 5) and CDH, a region that have also been reported to be associated with osteoarthritis. Objectives The main goal of our study was to assess whether GDF5 is associated with mild forms of AD in adult patients with hip osteoarthritis. Methods 131 Caucasian patients from the KHOALA (Knee or Hip Osteoarthritis Long Term Assessment) cohort with hip osteoarthritis were included in this study. All patients met both American College of Rheumatology and Kellgren and Lawrence (≥2) criteria for osteoarthritis. Patients underwent a morphological evaluation at both hips, based on standard anteroposterior and lateral X-rays. The morphological parameters retained here were the acetabular depth, VCE and HTE angles. Cut-off values to define AD were: VCE angle ≤20 °, HTE angle >12 °, or acetabular depth Results 45.8% (60/131) of patients had at least one morphological parameter consistent with AD. 29.8% (39/131) had abnormal HTE, 25.2% (33/131) had abnormal acetabular depth, and 17.6% (23/131) had abnormal VCE. The case-control study revealed a significant association between rs143383 variant and AD, when defined by insufficient acetabular depth. Individuals carrying CT genotype had a 5 fold higher prevalence of AD compared to those carrying CC genotype (95% CI: [0.95-35.63], p=0.030). Prevalence of AD was not significantly increased among patients carrying TT genotype (OR TT vs. CC =4.03, 95% CI: [0.72-29.33], p=0.138), whereas a significant association was observed under a dominant model (OR =4.57, 95% CI: 0.93-30.53], p=0.035). No significant association with out of range HTE and VCE values was observed. For each SNP, mean VCE, HTE, and acetabular depth values were compared among the three groups defined by their genotypes.For rs143383 variant, a significant difference in acetabular depth was observed among individuals presenting at least one morphological parameter consistent with AD (ANOVA: F =6.19, p=0.004 in the right hip and F =7.59, p=0.001 in the left hip). Conclusions This study confirms the association between GDF5 polymorphisms and CDH. It is the first publication to report an association between GDF5 and adult AD in context of hip osteoarthritis. Our results suggest an association between rs143383 and insufficient acetabular depth that should be assessed in further studies. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.2371

Background The minor salivary gland biopsy (MSGB) plays a preponderant role in the American European Consensus Group (AECG) criteria set for primary and secondary Sjogren’s syndrome (SS).However, its diagnostic value remains partially unclear and some authors have pointed out the lack of reproducibility of the results, and the lack of specificity of the test. Objectives To estimate the diagnostic value of MSGB for primary-SS, we performed a systematic review of published studies on MSGB for investigating SS. Methods PUBMED and EMBASE databases were searched. The following association of MeSh terms was used: “SJOGREN’S SYNDROME” in conjunction with “SALIVARY GLANDS” AND “BIOPSY”. All publications published between January 1966 and June 2011 were considered. Selected publications were screened for additional relevant studies. We selected studies that considered a Focus Score (FS) ≥1 (or equivalent) as cut-off for a positive biopsy and concerning patients addressed for suspected SS. Sensibility and specificity of MSGB were collected, or calculated when possible. Results From the 237 publications identified, 8 were finally included in the study (table 1). Among these 8 studies, sensitivity ranged from 63.5% to 93.7% and specificity from 61.2% to 100%. Specificity was >89% in 6 studies (Table 1). The differentiation between SS and non-SS patients was made without the help of MSGB or through a clinical reevaluation in only 2 studies. In these studies, sensitivity was respectively 63.9% and 85.7%, specificity was 91.9% and 89.7%. These 2 studies concerned 73 and 120 patients, evaluated for sicca symptoms in the first case and suspicion of primary-SS in the second. The gold standard for diagnosis of primary-SS was a set of criteria that did not include the MSGB in the first case and a reassessment of each patient by three experienced rheumatologists in the second (but not blinded to the MSGB). Conclusions Few studies are available in the literature to estimate the diagnostic value of MSGB for primary-SS. Only 2 studies used a methodology that avoids circular reasoning. Our study shows the lack of knowledge about the diagnostic value of MSGB, but confirm the good diagnosis value. The specificity of the test seems to be high, but the data about sensitivity is more heterogeneous. Disclosure of Interest None Declared

Background Early arthritis is often undifferentiated and may resolve spontaneously, remain undifferentiated, or progress to rheumatoid arthritis or to another chronic joint disease such as SpA. There is a need for improving the early diagnosis of ankylosing spondylitis (AS) and other spondyloarthritis (SpAs). Objectives To 1- assess the level of agreement between a SpA at baseline and 10 years later in a cohort of patients with recent-onset arthritis and 2- evaluate the ability of baseline clinical, laboratory, and radiographic features to predict a final diagnosis of SpA. Methods Patients with swelling of at least one joint between 1995 and 1997 were included. Ten years later, the physicians of each patient were contacted to determine the final diagnosis. Baseline pelvic radiographs were subjected to centralized blinded review. The statistical analysis used the chi-square test, Mann-Whitney test, and Cohen’s kappa coefficient. Results The diagnosis at baseline and 10 years later was known for 164 patients. At baseline, a diagnosis of SpA was suggested in 25 (15.2%) patients, of whom only 10 had a diagnosis of SpA 10 years later, in addition to 10 patients not diagnosed with SpA at baseline (20/164, 12.2%, Kappa=0.36±0.1). The 10-year diagnoses were ankylosing spondylitis (n=3), psoriatic arthritis (n=7), reactive arthritis (n=4), undifferentiated SpA (n=4), SAPHO syndrome, and Crohn’s disease (n=1). Of the 20 patients with a 10-year diagnosis of SpA, only 4 had inflammatory back pain at baseline (versus 11/144 non-SpA patients, p =0.72), 5 had psoriasis (versus 9/144 non-SpA patients, p =0.005), and 1 had uveitis ( p =0.12); 10 of the 20 SpA patients were HLA-B27-positive versus 8/144 non-SpA patients ( p Conclusions SpA is relatively rare and extremely challenging to diagnose in patients with early peripheral arthritis. Disclosure of Interest None Declared

Background Patients with rheumatoid arthritis (RA) are at increased risk for cardiovascular mortality and may therefore benefit from early atherosclerosis detection. Ankle brachial pressure index (ABPI) measurement can detect asymptomatic peripheral atherosclerosis, and isolated ABPI abnormalities can warrant treatment changes according to French recommendations. Objectives To evlauate the prevalence of ABPI abnormalities in RA in routine pratice. Methods We studied consecutive 100 patients meeting 1987 American College of Rheumatology criteria for RA when evaluated as inpatients or outpatients in November 2010 at a single rheumatology department. ABPI was measured routinely by a vascular physician. Patients were separated into three groups based on whether the ABPI was 1.3 (arterial stiffness). The three groups were compared using chi-square and Mann-Whitney tests. Results Of the 100 patients, 85 had normal ABPI values, 10 had DAF, and 5 had stiff arteries. Patients with DAF were older (69.1±12.6 years, P=0.002) and more often had a history of coronary artery disease (40%, P=0.004) compared to patients with normal arteries. The group with incompressible arteries had a larger proportion of patients with diabetes (40%, P=0.034) compared to the group with normal ABPI values. Conclusions The prevalence of DAF is relatively high in RA, especially in older patients. DAF is strongly associated with coronary artery disease in RA patients. Disclosure of Interest None Declared