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SSc is an auto-immune disease characterized by life-threatening manifestations such as lung fibrosis or pulmonary arterial hypertension. Symptoms with a detrimental impact on quality of life are also reported and sicca syndrome (xerostomia, xeropthalmia) is present in up to 80% of patients with SSc. Sicca syndrome can occur in the absence of overlap with Sjögren’s disease and recent studies highlight that fibrosis of minor and major salivary glands, directly linked to the pathogenesis of SSc, could be a major contributor of xerostomia in SSc. This narrative review provides an overview of the clinical presentation, diagnostic strategies, management and future perspectives on sicca syndrome in patients with SSc.

To evaluate the diagnosis performances of halo and compression signs alone and combined, assessed by a high frequency 22-MHz probe, and test their agreement in giant cell arteritis (GCA).In this cross-sectional study on patients suspected with GCA, halo sign was defined as hypo or iso-echogenic circumferential aspect of the vessel wall in transverse or longitudinal view; and compression sign was defined as visibility of the vessel wall upon transducer-imposed compression of the artery. Agreement of the two signs was tested using the Cohen's kappa statistic.A total of 80 patients (50% women) were included with a mean age of 74.4 years. Twenty participants (25%) were ultimately treated for GCA. Halo and compression signs have respective prevalences of 35% and 48%, with respective sensitivity and specificity of 80% and 80% for the halo sign; and 85% and 65% for the compression sign. The kappa coefficient for the global agreement of the two signs was 0.67 (95% confident interval: 0.54-0.85). Combination of the two signs give a sensitivity of 80% and a specificity of 81.7%.Halo and compression signs assessed by a high frequency probe, show a good level of agreement for the diagnosis of GCA and improve ultrasound specificity when combined together.

Resume L’echographie des glandes salivaires est un examen simple, peu invasif et reproductible qui s’est developpe depuis une trentaine d’annees. Principalement utilisee dans le syndrome de Gougerot-Sjogren (SGS), elle permet une etude du parenchyme des glandes parotides et submandibulaires. Ce dernier est homogene et iso-echogene en conditions normales. Le caractere pathologique est marque par une heterogeneite en lien avec la presence de zones hypoechogenes (aspect en nid d’abeille) ou d’un aspect fibreux de la glande en lien avec le depot d’un tissu fibro- adipeux se traduisant en echographie par de multiples bandes hyperechogenes (aspect fibreux). Malgre la presence de donnees de plus en plus robustes sur sa fiabilite et son utilite, l’echographie des glandes salivaires n’est actuellement pas associee aux criteres de classification ACR-EULAR 2016 du SGS, probablement par faute de consensus quant a son utilisation. En 2019, l’OMERACT a publie un score semi-quantitatif grade de 0 a 3 dont la fiabilite a ete confirmee dans plusieurs etudes. L’uniformisation des pratiques devrait permettre une diffusion et une validation de l’utilisation de l’echographie des glandes salivaires dans le syndrome de Gougerot-Sjogren a l’echelle internationale.

Resume L’arterite gigantocellulaire touche entre 15 et 45 personnes sur 100 000 personnes de plus de 50 ans en Europe du Nord. La presentation cranio-cephalique represente plus de deux tiers des cas et impacte le pronostic fonctionnel en raison de l’atteinte ophtalmologique et du risque de cecite. De nouvelles recommandations ont permis de faciliter le diagnostic en privilegiant le recours a l’echographie des troncs supra-aortiques et des arteres temporales devant une probabilite clinique forte.

Objective: Major salivary gland ultrasonography (SGUS) is widely used for the diagnosis of primary Sjögren’s syndrome (pSS). Our objective was to assess the contribution of SGUS compared to other items of the 2016 ACR/EULAR pSS classification criteria, based on expert opinion. Methods: A secure web‐based relational database was used by 24 experts from 14 countries to assess 512 realistic vignettes developed from data of patients with suspected pSS. Each vignette provided classification criteria items and information on history, clinical symptoms and SGUS findings. Each expert assessed 64 vignettes, and each vignette was assessed by 3 experts. A diagnosis of pSS was defined according to at least 2 of 3 experts. Validation was performed in the independent French DiapSS cohort of patients with suspected pSS. Results: A criteria‐based pSS diagnosis and SGUS findings were independently associated with an expert diagnosis of pSS (P < 0.001). The derived diagnostic weights of individual items in the 2016 ACR/EULAR criteria including SGUS were as follows: anti‐SSA, 3; focus score ≥ 1, 3; SGUS score ≥ 2, 1; positive Schirmer’s test, 1; dry mouth, 1; and salivary flow rate < 0.1 mL/min, 1. The corrected C statistic area under the curve for the new weighted score was 0.96. Adding SGUS improves the sensitivity from 90.2 % to 95.6% with a quite similar specificity 84.1% versus 82.6%. Results were similar in the DiapSS cohort: adding SGUS improves the sensitivity from 87% to 93%. Conclusion: SGUS had similar weight compared to minor items, and its addition improves the performance of the 2016 ACR/EULAR classification criteria. acceptedVersion

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.

International audience; Il est bien établi que l’activité physique (AP) a un impact positif sur les patients atteints de polyarthrite rhumatoïde (PR) [1]. Cependant, le confinement prolongé déclenché par la COVID-19 de mars à mai 2020 a considérablement réduit les possibilités d’AP en France. Cette étude avait pour objectif de décrire et de mesurer l’impact de ce confinement sur la pratique d’AP, la qualité de vie et les relations avec les rhumatologues des patients atteints de PR.L’étude a été menée sur www.carenity.com, une communauté mondiale de patients en ligne où les patients atteints de maladies chroniques peuvent partager leur expérience et obtenir des informations de santé. Les patients volontaires peuvent également contribuer à la recherche médicale en participant à des études en ligne. Les patients atteints de PR ont été invités à répondre à un questionnaire en ligne du 25 mai au 15 juin 2020. Les patients ont donné leur consentement en ligne au début de l’étude pour confirmer leur participation. Les analyses statistiques ont été réalisées à l’aide de tests du X2 pour analyser les différences entre les variables catégorielles. Dans l’ensemble de l’étude, le niveau d’activité de la PR a été défini par le nombre de symptômes et la gêne occasionnée.Carenity.com compte 698 patients activement impliqués dans sa communauté PR en France, dont 204 (29 %) ont participé (86 % de femmes, âge moyen 54 ans, 84 % diagnostiqués il y a plus de 3 ans).La proportion de patients pratiquant une AP a diminué pendant le confinement : de 74 % à 42 % pour la mobilité active, de 22 % à 11 % pour les sports modérément intenses et de 16 % à 6 % pour les sports très intenses (Tableau 1). Pendant le confinement, 50 % des patients ont réduit significativement la quantité d’AP qu’ils pratiquaient, de 57 % en termes de fréquence et de 48 % en termes d’intensité (p < 0,05, diminution significative par rapport aux patients qui ont augmenté ou sont restés stables pendant le confinement). Pour maintenir une AP, la plupart des patients ont déclaré utiliser une méthode spécifique, principalement numérique (vidéos d’exercices en ligne, applications informatiques…).

Resume Objectifs Cette etude, menee dans une population de patients atteints de LES, avait pour objectifs (1) de decrire les atteintes articulaires echographiques (US), (2) d’estimer la correlation du nombre d’articulations cliniquement gonflees (NAG-C) et du SLEDAI clinique (SLEDAI-C) avec le NAG echographique (NAG-US) et le SLEDAI echographique (SLEDAI-US), (3) de mettre en evidence les caracteristiques specifiques des patients lupiques selon les observations en mode Doppler puissance (DP). Methode Nous avons inclus dans cette etude transversale multicentrique 151 patients adultes consecutifs atteints de LES. L’evaluation comprenait un examen clinique standardise des articulations, une echographie en mode B et en mode Doppler puissance (DP) sur 40 articulations et 26 tendons, en aveugle des resultats de l’examen clinique. La correlation entre l’examen clinique et l’echographie en mode B a ete calculee au moyen du coefficient de correlation intra-classe (ICC [Intervalle de confiance a 95 %]). Resultats Un taux tres eleve d’anomalies infracliniques echographiques a ete observe chez les patients asymptomatiques : l’existence d’au moins une manifestation echographique a ete retrouvee chez 85 % des patients qui ne presentaient aucun symptome articulaire. Parmi eux, 46 patients (87 %) avaient un antecedent d’atteinte articulaire. L’epanchement articulaire (108 patients), l’hypertrophie synoviale (HS, 109 patients) et la synovite (61 patients) etaient les anomalies les plus frequemment retrouvees. Un signal DP positif des articulations ou des tendons (grade > 1) a ete retrouve dans 44 % des cas (67/151). Des synovites ont ete detectees principalement aux articulations MCP et des poignets. Malgre la faible correlation de l’examen clinique au NAG-US en mode B, une bonne correlation du SLEDAI clinique au SLEDAI echographique a ete retrouvee. La comparaison des patients atteints de LES avec et sans signal DP n’a pas mis en evidence de caracteristiques specifiques du LES. Conclusion L’echographie est une methode d’imagerie qui a prouve son utilite pour l’evaluation des atteintes articulaires dans le LES mais qui ne modifiait pas de maniere significative le score SLEDAI.

Ultrasound is a useful imaging modality for the diagnosis of giant cell arteritis. Ultrasound of temporal arteries is recommended as the first imaging modality in patients with suspected predominantly cranial giant cell arteritis. The thickening of the arterial wall (halo sign) is the ultrasound finding most suggestive of giant cell arteritis. In patients in whom there is a high clinical suspicion of giant cell arteritis and a halo sign, the diagnosis of giant cell arteritis may be made without temporal artery biopsy.

International audience; Despite major recent advances in the therapeutic management of Giant cell arteritis (GCA), the diagnosis accuracy of temporal artery ultrasound remains controversial in this disease. We performed a systematic review to determine the sensitivity, specificity, and summary positive (LR+) and negative (LR-) likelihood ratios of temporal artery ultrasound for the diagnosis of GCA. For this, we searched EMBASE, MEDLINE and the Cochrane Database of Systematic Reviews without language restriction. Original articles reporting on diagnostic accuracy of temporal artery ultrasound compared to temporal artery biopsy, for the diagnosis of GCA, were selected. Sensitivity and specificity from each study were used to fit a bivariate diagnosis accuracy model. Of 1280 articles identified, 48 underwent full-text review, and 25 were included. Based on a total of 20 studies, the sensitivity and specificity of hypoechoic halo compared to positive temporal artery biopsy were respectively of 68% (95% CI: 57-78) and 81% (95%CI: 75-86). The summary mean positive and negative likelihood ratios were respectively of 3.64 (95%CI: 2.76-4.73) and 0.40 (0.28-0.52). Taking into account 11 studies reporting on the presence of any abnormal sign on temporal artery ultrasound yielded similar results with largely overlapping 95% confidence interval regions. This study provides the summary estimates of the diagnostic properties of temporal artery ultrasound compared to temporal artery biopsy, for the diagnosis of GCA. Those parameters allow the calculation of the post-test probability of GCA in a given patient, based on the results of temporal artery ultrasound and will help improving the diagnosis strategy for this common disease.

OBJECTIVE: Clinical heterogeneity, a hallmark of systemic autoimmune diseases, impedes early diagnosis and effective treatment, issues that may be addressed if patients could be classified into groups defined by molecular pattern. This study was undertaken to identify molecular clusters for reclassifying systemic autoimmune diseases independently of clinical diagnosis. METHODS: Unsupervised clustering of integrated whole blood transcriptome and methylome cross-sectional data on 955 patients with 7 systemic autoimmune diseases and 267 healthy controls was undertaken. In addition, an inception cohort was prospectively followed up for 6 or 14 months to validate the results and analyze whether or not cluster assignment changed over time. RESULTS: Four clusters were identified and validated. Three were pathologic, representing "inflammatory," "lymphoid," and "interferon" patterns. Each included all diagnoses and was defined by genetic, clinical, serologic, and cellular features. A fourth cluster with no specific molecular pattern was associated with low disease activity and included healthy controls. A longitudinal and independent inception cohort showed a relapse-remission pattern, where patients remained in their pathologic cluster, moving only to the healthy one, thus showing that the molecular clusters remained stable over time and that single pathogenic molecular signatures characterized each individual patient. CONCLUSION: Patients with systemic autoimmune diseases can be jointly stratified into 3 stable disease clusters with specific molecular patterns differentiating different molecular disease mechanisms. These results have important implications for future clinical trials and the study of nonresponse to therapy, marking a paradigm shift in our view of systemic autoimmune diseases. ispartof: ARTHRITIS & RHEUMATOLOGY vol:73 issue:6 pages:1073-1085 ispartof: location:United States status: published

Objectives The aim of this study was to show the usefulness of a mid-infrared fibre evanescent wave spectroscopy point of care device in the identification of septic arthritis patients in a multicentre cohort, and to apply this technology to clinical practice among physicians. Methods SF samples from 402 patients enrolled in a multicentre cohort were frozen for analysis by mid-infrared fibre evanescent wave spectroscopy. The calibration cohort was divided into two groups of patients (septic arthritis and non-septic arthritis) and relevant spectral variables were used for logistic regression model. Model performances were tested on an independent set of 86 freshly obtained SF samples from patients enrolled in a single-centre acute arthritis cohort and spectroscopic analyses performed at the patient’s bedside. Results The model set-up, using frozen–thawed SFs, provided good performances, with area under the curve 0.95, sensitivity 0.90, specificity 0.90, positive predictive value 0.41 and negative predictive value 0.99. Performances obtained in the validation cohort were area under the curve 0.90, sensitivity 0.92, specificity 0.81, positive predictive value 0.46 and negative predictive value 0.98. The septic arthritis probability has been translated into a risk score from 0 to 4 according to septic risk. For a risk score of 0, the probability of identifying a septic patient is very low (negative predictive value of 1), whereas a risk score of 4 indicates very high risk of septic arthritis (positive predictive value of 1). Conclusion Mid-infrared fibre evanescent wave spectroscopy could distinguish septic from non-septic synovial arthritis fluids with good performances, and showed particular usefulness in ruling out septic arthritis. Our data supports the possibility of technology transfer. Trial registration ClinicalTrials.gov, http://clinicaltrials.gov, NCT02860871.

International audience; The diagnostic modalities for giant cell arteritis (GCA) have evolved significantly in recent years. Among the different diagnostic tools developed, Doppler ultrasound of the temporal arteries, with a sensitivity and specificity reaching 69% and 82%, respectively, is now recognized as superior and, therefore, is a first-line diagnostic tool in GCA. Moreover, with the increasing development of new ultrasound technologies, the accuracy of Doppler ultrasound in GCA seems to be constantly improving. In this article, we describe in detail the scanning technique to perform while realizing Doppler ultrasound of temporal arteries to assess GCA, as well as the diagnostic performance of this tool according to current literature.

ObjectiveTo evaluate the relevance of salivary gland ultrasound (SGUS) and its place in the diagnostic algorithm in patients referred with dry syndrome (DS) for a suspicion of Sjögren’s syndrome (SS).MethodsWe included all patients assessed at our dedicated DS clinic from June 2015 to September 2019 for which a SGUS has been carried out. Images were read blindly and the worst salivary gland was scored according to OMERACT classification. Clinical features, disease activity and treatments were collected.Results337 patients were seen from June 2015 to September 2019. 269 patients underwent SGUS. 77 patients were diagnosed with SS and 192 did not meet the ACR/EULAR criteria for SS: non-Sjögren’s DS (NSDS). Of these 192 patients, 60 had another possible cause of DS, and 132 patients were diagnosed with SAPS (sicca, asthenia, polyalgia syndrome).SGUS abnormalities were significantly higher in patients with SS versus NSDS: 51% vs 8% for a score ≥2 (pConclusionSGUS is useful for SS diagnosis. If anti-SSA antibodies are negative and SGUS score

Introduction Oral administration of pilocarpine enhances salivary flow in sicca patients but its effect upstream on ultrasound (US) of salivary glands (SG) and downstream on periodontium remain unknown. Methods Sicca patients were prospectively included. Echostructural and vascularization of SG were assessed using B mode and pulsed Doppler (USPD). Vascularization of SG was measured using resistive index (RI) before and after stimulation by lemon juice. Echostructure (measure of glandular length in cm2, evaluation of parotid and submandibular glands parenchymal abnormalities) was assessed at baseline (M0) and after 3 months (M3) of treatment with pilocarpine. A dental consultation was performed at M0 and M3 to evaluate changes in unstimulated salivary flow (USSF), stimulated salivary flow (SSF), and periodontal parameters such as modified gingival index (Lobene), plaque index (Silness), bleeding index, pocket depth, and pH. Results Nineteen patients were included but only 11 received pilocarpine treatment for 3 months, as six stopped pilocarpine due to side effects and two were excluded for other causes. Among the 11 patients who completed the 3-month follow-up, five had primary Sjögren’s syndrome according to the American-European’s classification criteria. As expected, statistical differences were found concerning SSF (p = 0.018) and USSF (p = 0.027) between M0 and M3 while no statistical change in both SG echostructure and vascularization or periodontal evaluation was shown. Conclusions Pilocarpine improved SSF and USSF measurements in sicca syndrome but no ultrasonography of major salivary glands (SGUS) structural and vascular changes were detected as well as periodontal evaluation. Supplementary Information The online version contains supplementary material available at 10.1007/s40744-020-00263-y.

Introduction Les atteintes cutanees sont habituelles au cours du syndrome de Gougerot-Sjogren primitif (SSp), mais leur prevalence et leurs caracteristiques sont difficiles a etablir precisement en raison du nombre limite de patients etudies dans la plupart des cohortes, de la variabilite des signes fonctionnels et cliniques evalues dans chaque cohorte, de la rarete de certains de ces signes et de l’heterogeneite des evaluations des etudes precedentes. L’objectif principal de cette etude etait de determiner, a partir de 3 groupes francais de patients souffrant de SSp (TEARS, ASSESS, DiapSS) dans lesquels differents criteres d’evaluation ont ete utilises, la prevalence des atteintes dermatologiques, les parametres cliniques et paracliniques qui leur sont associes. Materiel et methodes Nous avons utilise les donnees de deux cohortes francaises (ASSESS, qui a evalue la prevalence des atteintes cutanes chez 395 patients atteints de SSp, et DiapSS, qui comprend 139 patients atteints du syndrome de Gougerot-Sjogren diagnostique selon les criteres ACR-EULAR de 2016 parmi 325 patients suspectes d’etre atteints du SSp) et les donnees de base de l’essai randomise TEARS (110 patients atteints de SSp recent ou actif traites par rituximab ou placebo, evalues pour la secheresse cutanee a l’aide d’une echelle visuelle analogique sur 100). Resultats Les manifestations cutanees incluses dans l’indice EULAR d’activite du syndrome de Gougerot-Sjogren (ESSDAI) etaient rares dans la cohorte ASSESS (n = 16/395, 4,1 %), mais elles etaient associees a une activite dans les autres domaines de l’ESSDAI. L’etude TEARS a montre une forte prevalence de la secheresse cutanee (EVA > 50 ; 48,2 %) et a constate que les patients ayant la peau seche presentaient des scores EVA de douleur et de secheresse globale plus eleves (p = 0,003 et p Discussion Les signes dermatologiques inclus dans l’ESSDAI sont rares. Ces atteintes cutanees actives sont associees a l’activite de l’ESSDAI dans divers domaines. Un examen systematique par un dermatologue permettrait de mieux estimer les manifestations cutanees specifiques et non specifiques du syndrome de Gougerot-Sjogren. L’atteinte cutanee la plus courante est la secheresse cutanee mais elle n’est pas incluse dans les criteres d’evaluation de la maladie. Elle devrait donc etre evaluee par une EVA specifique.

We read with great interest the results of Couderc and colleagues concerning ultrasonographic (US) involvement of major salivary glands (SGs) in systemic sclerosis (SSc).1 We fully agree with their comment concerning the high prevalence of sicca syndrome in SSc and its underestimated impact on quality of life. US evaluation of major SGs is a non-irradiating, easy-to-use and largely available tool. Its diagnostic performances have been properly evaluated in primary Sjogren syndrome (SS)2 and can be extended to secondary SS. In their study, Couderc and colleagues highlight the high prevalence of overlap syndrome SSc/primary SS according to US evaluation. Nonetheless, this overlap syndrome may not be the only mechanism underlying sicca syndrome in SSc. As initially suggested by Couderc and colleagues, a specific SSc-associated fibrosis of major SGs could also explain sicca syndrome in SSc.1 3 This hypothesis is all the more relevant as such glandular fibrosis has already been described in minor SGs of SSc patients.3 Although the US Cornec’s score used by Couderc and colleagues …

Introduction Ulnar tunnel syndrome at the elbow is a common pathology. The ultrasound cross-sectional area is a well-known metric widely accepted in radiology for the description of nerve entrapment. However, the pathological cut-off value remains challenging. The objectives of this study were to (1) describe the ultrasound cross-sectional area measurement of the ulnar nerve at three locations, and (2) to evaluate the inter-observer reliability by two independent ultrasonographers. Methods One-hundred ulnar nerves of 50 asymptomatic individuals were scanned using B-mode and power Doppler ultrasonography. The ultrasound cross-sectional area measurements of the ulnar nerve were performed at three different levels: 2 cm proximal to the epicondyle, at the level of the epicondyle, and 2 cm distal to the epicondyle. Results In our healthy population, we found 21, 24 and 7% of ultrasound cross-sectional area ulnar nerve > 8 mm2, respectively, at three different levels of measurement and 4, 7, and 0% US-CSA ulnar nerve > 10 mm2. The intraclass correlation coefficient measured at three different site levels were good (0.7943, 0.7509) to moderate (0.5701). Conclusions Almost one-quarter of our healthy population had an ultrasound cross-sectional area ulnar nerve more than 8 mm2 and few more than 10 mm2. A cut-off of ultrasound cross-sectional area ulnar nerve measurement more than 10 mm2 could be considered as pathological. No abnormal elbow ulnar nerve vascularization has been seen. This is the first step towards normal B-mode ulnar nerve values at the elbow to further detect pathological US findings as ulnar nerve entrapment.

To determine the prevalence and significance of dermatological disorders in primary Sjögren syndrome (pSS).We used 2 pSS French cohorts (ASSESS, in which prevalence of skin disorders in 395 patients was evaluated; and diapSS, in which 76 on 139 pSS patients had an examination by a dermatologist) and baseline data of the TEARS randomized trial (110 patients with recent or active pSS treated with rituximab or placebo and evaluated for skin dryness using a visual analogue scale (VAS) out of 100).Skin manifestations included in the EULAR Sjögren syndrome disease activity index (ESSDAI) were rare in the ASSESS cohort (n=16/395, 4.1%, mainly purpuras; only 3 had high activity), but they were associated with activity in the other ESSDAI domains (peripheral neurological (P0.001), muscular (P0.01), haematological (P0.05), biological (P0.05), history of arthritis (P0.01), splenomegaly (P0.05) and higher gamma globulin levels (P0.01)). In the diapSS cohort, compared to pSS patients not receiving a dermatological consultation, the pSS patients who had a dermatological consultation had significantly more dermatological involvement outside the ESSDAI score [38.2% (29/76) versus 15.9% (10/63); P0.01]. The TEARS study showed a high prevalence of cutaneous dryness (VAS50; 48.2%) and found that patients with dry skin had higher VAS pain (P0.01) and drought (P0.01) scores.ESSDAI skin activity is rare and associated with hypergammaglobulinemia and ESSDAI activity. Systematic dermatological examination is informative for non-specific lesions. The most common skin disorder is skin dryness, which is associated with a higher pain and overall subjective dryness.

Objectives To evaluate the reliability of the OMERACT paediatric ultrasound (US) synovitis definitions and scoring system in JIA. Methods Thirteen sonographers analysed 75 images for the presence/absence of elementary lesions (binary scoring) and for grading synovitis, synovial hypertrophy, effusion and Doppler signals. Static US images of the second metacarpophalangeal joint (MCP-II), wrist, elbow, knee and ankle in JIA patients at different ages and different disease stages were collected with standardized scanning by two experienced sonographers. Intra- and inter-reader reliability were analysed with kappa coefficients. Results Intra-reader reliability was good for binary scoring (Cohen’s kappa 0.62, range 0.47–0.75), synovitis and synovial hypertrophy; excellent for Doppler signals (quadratic weighted kappa 0.77, 0.66–0.86; 0.76, 0.61–0.84; and 0.87, 0.77–0.94, respectively); and moderate for effusion (0.55, 0.24–0.76). Inter-reader reliability was good for synovitis and synovial hypertrophy (Light’s kappa 0.68, 95% CI: 0.61, 0.75 and 0.63, 0.54–0.71, respectively), excellent for Doppler signals (0.85, 95% CI: 0.77, 0.90), and moderate for binary scoring and effusion (0.48, 95% CI: 0.36, 0.64 and 0.49, 0.40–0.60, respectively). We obtained the best scores for the knee (0.71, 0.54–0.85) except for Doppler signals, with reliability higher for MCP-II. We found a trend toward better results in older children. Conclusions This is the first study establishing the reliability of the OMERACT paediatric US synovitis definitions and scoring system in the five most commonly affected joints in JIA. The reliability was good among a large group of sonographers. These results support the applicability of these definitions and scoring system in clinical practice and multicentre studies.

Resume Objectif La parotidomegalie est un critere du score d’activite du syndrome de Gougerot-Sjogren primitif (ESSDAI, EULAR Sjogren Syndrome Disease Activity Index) defini par l’EULAR. La valeur seuil de longueur a ete fixee a 3 cm pour les glandes parotides et a 2 cm pour les glandes submandibulaires. Cependant la fiabilite de l’evaluation clinique des glandes salivaires demeure douteuse. L’objectif principal de cette etude est d’evaluer la reproductibilite interobservateur de la mesure des parotides par palpation. L’objectif secondaire est de comparer la fiabilite de cet examen a celle de l’echographie. Methodes Ont ete inclus des patients en consultation externe dans un meme centre de reference presentant un syndrome de Gougerot-Sjogren primitif (SGSp) avere ou suspecte. Deux investigateurs independants (V.D.-P. et D. C.) ont pratique un examen clinique pour evaluer le gonflement des glandes parotides, leur taille (mesure directe sous l’angle mandibulaire a l’aide d’un decimetre) et la douleur. Le coefficient kappa de Cohen a ete calcule pour determiner l’accord interobservateur quant au gonflement des parotides ; le coefficient de correlation intraclasse a ete calcule pour determiner l’accord interobservateur quant a la taille mesuree des glandes. Resultats Trente-quatre patients (33 femmes, un homme) ont ete inclus. Les donnees cliniques etaient completes pour 33 patients. Le coefficient kappa d’accord interobservateur etait de 0,90 [0,76–1,00] pour la detection d’une parotidomegalie sur les 66 parotides. Pour la longueur de la glande, il etait de 0,60 [0,42–0,73]. Le seuil median determinant la parotidomegalie etait de 4,15 cm pour l’un des observateurs et de 4,92 cm pour le second. Aucune correlation entre les investigateurs n’a ete etablie concernant la palpation des glandes submandibulaires. Une association significative a ete constatee entre parotidomegalie clinique et augmentation de la superficie observee a l’echographie. Conclusion L’evaluation clinique de la parotidomegalie, en mode binaire (presence/absence), etait concordante entre les deux observateurs. En revanche, la concordance etait faible pour la mesure directe. L’echographie pourrait constituer un examen complementaire utile.

Resume La biopsie des glandes salivaires accessoires est un examen simple, frequemment realise par les rhumatologues dans un contexte diagnostique (syndrome de Sjogren, sarcoidose, amylose, etc.). Les glandes salivaires accessoires representent plusieurs centaines de glandes disseminees dans la cavite buccale et sont facilement accessibles dans la levre inferieure. Toutefois, un certain nombre de precautions sont necessaires pour eviter les complications liees a cette petite chirurgie. Etant donne qu’elles ne representent que 10 % de la production de salive, la biopsie de quatre a six glandes (selon les recommandations EULAR) sur les centaines existantes a peu d’impact sur la production de salive. La biopsie de glandes salivaires principales a ete rapportee, en particulier pour les parotides qui etaient les premieres glandes prelevees, et de facon plus anecdotique pour les glandes sublinguales. L’evolution des techniques, en particulier de l’echographie, remet probablement au gout du jour ces biopsies des glandes parotides en limitant considerablement les risques de complication.

Resume Les modalites diagnostiques de l’arterite a cellules geantes (ACG-ex maladie de Horton) ont beaucoup evolue au cours de ces dernieres annees. Toutefois, la biopsie de l’artere temporale (BAT), malgre sa sensibilite moderee, garde une place de choix dans le diagnostic des formes cephaliques. Nous decrivons dans cet article la technique de realisation de la BAT ainsi que les performances diagnostiques de cet examen.