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A new autoinflammatory syndrome related to somatic mutations of UBA1 was recently described and called VEXAS syndrome ('Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome').To describe clinical characteristics, laboratory findings and outcomes of VEXAS syndrome.One hundred and sixteen patients with VEXAS syndrome were referred to a French multicentre registry between November 2020 and May 2021. The frequency and median of parameters and vital status, from diagnosis to the end of the follow-up, were recorded.The main clinical features of VEXAS syndrome were found to be skin lesions (83%), noninfectious fever (64%), weight loss (62%), lung involvement (50%), ocular symptoms (39%), relapsing chondritis (36%), venous thrombosis (35%), lymph nodes (34%) and arthralgia (27%). Haematological disease was present in 58 cases (50%): myelodysplastic syndrome (MDS; n = 58) and monoclonal gammopathy of unknown significance (n = 12; all patients with MGUS also have a MDS). UBA1 mutations included p.M41T (45%), p.M41V (30%), p.M41L (18%) and splice mutations (7%). After a median follow-up of 3 years, 18 patients died (15·5%; nine of infection and three due to MDS progression). Unsupervised analysis identified three clusters: cluster 1 (47%; mild-to-moderate disease); cluster 2 (16%; underlying MDS and higher mortality rates); and cluster 3 (37%; constitutional manifestations, higher C-reactive protein levels and less frequent chondritis). The 5-year probability of survival was 84·2% in cluster 1, 50·5% in cluster 2 and 89·6% in cluster 3. The UBA1 p.Met41Leu mutation was associated with a better prognosis.VEXAS syndrome has a large spectrum of organ manifestations and shows different clinical and prognostic profiles. It also raises a potential impact of the identified UBA1 mutation.

Recent evidence showed greater efficacy of tocilizumab (TCZ) in the subgroups of COVID-19 patients who presented with symptoms for less than 7 days and in those only receiving oxygen. We retrospectively analyzed a compassionate use cohort to determine the best timing for TCZ injection. We showed no association between the timing of injection after symptom onset and the efficacy of TCZ on mortality. We then investigated whether the oxygen level at the time of TCZ injection impacted the mortality rate. Our study finally suggested that TCZ could be less effective when oxygen requirement is >11 L/min and we hypothesized that earlier administration could be associated with better outcome. However, randomized clinical trials are required to confirm this hypothesis.

Inside a cell, mitochondria are organelles that exhibit dynamic locomotion and spatial rearrangement (Cai and Sheng 2009; Sheng 2017). This movement is necessary for a cell to maintain basic metabolic functions, and disruption of this motility often results in cell death. In fruit flies and mammals, one protein complex is primarily responsible for trafficking mitochondria along microtubules. This protein complex typically, but not always, consists of three proteins: Miro, Trak, and a motor protein (Stowers et al. 2002; Guo et al. 2005; Glater et al. 2006; Macaskill et al. 2009; Koutsopoulos et al. 2010; Brickley and Stephenson 2011; van Spronsen et al. 2013; Barel et al. 2017; López-Doménech et al. 2018; Henrichs et al. 2020).

Resume Introduction Les troubles trophiques des extremites sont une complication frequente de la sclerodermie systemique (ScS), principalement lies a une atteinte microcirculatoire. Cependant, la ScS semble etre un facteur de risque d’atteinte athero-thrombotique precoce qui peut toucher les arteres peripheriques, participer a la survenue de troubles trophiques et favoriser la survenue de complications infectieuses. L’objectif de ce travail etait d’evaluer la prevalence de l’atteinte arterielle des membres chez les patients ScS. Methodes Inclusions consecutives dans le cadre d’une consultation pluridisciplinaire centree sur le handicap de la main avec recueil de donnees cliniques [facteurs de risque cardiovasculaire (FRCV), antecedent de troubles trophiques d’origine ischemique, palpation des pouls peripheriques, manœuvre d’Allen aux membres superieurs (MS) et inferieurs (MI)], et hemodynamiques (flux des arteres radiales, ulnaires, tibiales anterieures et posterieures enregistres par Doppler continu, et mesure des indices de pression systolique aux chevilles). Resultats Quatorze patients ont ete inclus (11 droitiers, 2 gauchers et 1 ambidextre). Le sex-ratio homme/femme etait de 0,27 et l’âge moyen de 58,1 ± 10,4 ans. Les principaux FRCV etaient l’âge et le tabagisme. Aux MS, 42,8 % des patients avaient un antecedent de troubles trophiques, la manœuvre d’Allen etait anormale pour 35,7 % des arcades palmaires superficielles, 42,9 % des pouls ulnaires n’etaient pas percus et aucun flux n’etait enregistrable pour 25 % des arteres ulnaires. Aux MI, 14,3 % des patients avaient deja presente des troubles trophiques des orteils, la manœuvre d’Allen etait anormale pour 15,4 % des arteres tibiales posterieures, 25,6 % des pouls tibiaux posterieurs n’etaient pas percus et le flux de 15,4 % des arteres tibiales posterieures etait pathologique. Conclusion L’atteinte macrovasculaire distale touchait preferentiellement les arteres ulnaires et tibiales posterieures avec une frequence elevee aux MS et deux fois moindre aux MI. La physiopathologie est mal connue mais il pourrait s’agir d’une manifestation propre de la ScS. Il semble necessaire que les patients ScS beneficient d’un equilibre strict de leurs FRCV et d’un depistage des atteintes arterielles macrovasculaires. Se pose aussi la question de la place d’un traitement anti-atheromateux chez ces patients.

INTRODUCTION AND OBJECTIVES Cardiac manifestations in Fabry disease are mainly characterized by left ventricular hypertrophy (LVH). The aims of this study were (1) to describe the pattern of regional strain in patients with Fabry disease and (2) to assess whether this pattern may help differentiate patients with Fabry disease from patients with sarcomeric hypertrophic cardiomyopathy (HCM). METHODS Seventy-seven subjects were investigated: patients with Fabry disease (n=37; 57% with LVH), patients with HCM (n=21), and healthy controls (n=19). Global and segmental longitudinal and circumferential strain (CS) analyses were performed by two-dimensional speckle strain imaging. Base-to-apex longitudinal and CS gradient, defined as the peak gradient difference between averaged basal and apical strain, was calculated. RESULTS Longitudinal strain gradient did not differ between controls and Fabry patients without hypertrophy (respectively: -10±3.2 vs -8±4.3, P=.41) or between the HCM group and Fabry patients with hypertrophy (respectively: -7.5±4.5 vs -9±4.5, P=.37). The CS gradient was lower in Fabry patients without hypertrophy compared to the controls (respectively: 1±8 vs 14.2±9.5, P

Figure S1. Funnel plots, forest plots, and meta-regression for the meta-analysis of SMR. Figure S2. Funnel plots and forest plots of the risk factors related with mortality. (PDF 948 kb)

Table S1. Survival rates and survival curve for lcSSc and dcSSc. Table S2. Main characteristics of studies in the SMR meta-analysis. Table S3. Main characteristics of studies in the prognosis factors meta-analysis. (DOCX 51 kb)

Resume Introduction Le syndrome d’Erasmus se definit comme l’association d’une sclerodermie systemique et d’une exposition a la silice. Observation Nous en rapportons un cas chez un foreur-artificier en carrieres de roches massives, expose a la silice et aux derives nitres contenus dans les explosifs. Conclusion La physiopathologie et les etiologies de la sclerodermie systemique sont encore imparfaitement connues mais il s’avere que le monoxyde d’azote, libere notamment lors de la metabolisation des derives nitres, est aussi implique dans la genese de la maladie : il semble donc licite de s’interroger sur les consequences d’une co-exposition au monoxyde d’azote non maitrisee et non quantifiee sur l’etat vasculaire, deja degrade par la sclerodermie. Plus largement, notre observation souligne l’importance d’un recueil soigneux et exhaustif des expositions professionnelles pour les patients atteints de sclerodermie systemique.

Calcinosis cutis constitutes a heterogeneous group of chronic disorder. It can be associated with disturbance of calcium and/or phosphate metabolism (metastatic, tumor calcinosis, calciphylaxis) but may also develop without any metabolic disorder, in particular during the course of connective tissue diseases. Among these, the most common are dermatomyositis and the limited form of systemic sclerosis. The physiopathology of calcinosis cutis is poorly known. It can cause pain, chronic ulcerations, infections, which are sources of sometimes major disability. Treatment of calcinosis is challenging because no drug has been shown to be reliably effective in stopping the progression or decreasing dystrophic calcifications in controlled trials. Calcium blocker and colchicine are generally prescribed as the first line systemic therapy. In the localized forms of small lesions, surgical excision is often effective and sometimes preceded by local treatments (laser therapy, extracorporeal shock wave lithotripsy, topical sodium thiosulfate, etc.) or systemic treatment (minocycline, warfarine). When calcinosis is disseminated, it may require additional treatments (aluminium hydroxyde, bisphosphonates) possibly associated with surgery in case of large lesions. Time to response may be prolonged from weeks to months. The calcinosis cutis can lead to secondary infection, pain and functional disability that have to be prevented.

Melanoma-associated retinopathy (MAR) is a rare autoimmune syndrome in patients with melanoma characterized by visual disorders. MAR is induced by the degeneration of bipolar cells of the retina and the presence of serum autoantibodies against retina proteins. Ipilimumab, an anti-cytotoxic T lymphocyte-associated antigen 4 antibody, improves survival in previously treated patients with metastatic melanoma, but is responsible for a spectrum of immune-related adverse events. Administration of ipilimumab to patients with autoimmune diseases (such as MAR or vitiligo) is actually not recommended. We report a patient presenting with MAR occurring during a melanoma relapse. Surgery and chemotherapy had no effect on visual acuity and melanoma increased. In the absence of alternative antitumoral treatment, we focused on the vital prognosis and treated the patient with ipilimumab. Two years after the treatment the patient is free from new metastasis but has presented with exacerbation of vitiligo and MAR. In the very rare case of melanoma with autoimmune disease without a therapy option, ipilimumab could be discussed, taking into account the fact that it can be effective on tumor burden but can also increase autoimmunity.

Resume Introduction Le syndrome d’Ehlers-Danlos de type hypermobile (SED-TH) est une maladie genetique rare dont le diagnostic repose sur une association de criteres cliniques decrite dans la classification de Villefranche. Ce diagnostic est difficile a etablir du fait de l’absence de signes cliniques specifiques et de test genetique. Le SED-TH se manifeste principalement par des douleurs musculosquelettiques et une hyperlaxite articulaire. Des dysfonctions de l’appareil manducateur (DAM) sont egalement decrites. Notre but etait d’objectiver la presence et de qualifier le type de DAM associe au SED-TH afin de proposer un argument diagnostique supplementaire. Materiel et methodes Une etude cas-temoin, prospective, monocentrique, a compare une cohorte de patients atteints d’un SED-TH a un groupe temoin apparie de patients volontaires sains. L’examen clinique etait standardise, comprenant un interrogatoire general, un examen oral et un examen de l’articulation temporo-mandibulaire, realise selon les recommandations de la TMD/RDC (temporomandibular disorders/research diagnostic criteria). Resultats Quatorze patients SED-TH et 58 patients temoins ont etes examines. La prevalence de DAM (n = 13 ; 92,9 % vs n = 4 ; 6,9 % ; p = 10−11) etait significativement plus importante dans le groupe SED-TH. Les DAM du groupe SED-TH etaient complexes associant plusieurs mecanismes contrairement au groupe temoin dont le mecanisme etait toujours unique (n = 13 ; 92,9 % vs n = 0 ; 0,0 %). Discussion Les DAM sont fortement associees au SED-TH. Elles pourraient etre utilisees dans l’etablissement du diagnostic de cette pathologie.

Background IgG replacement therapy (IgRT) in primary immunodeficiencies (PID) is a lifelong treatment which may be administered intravenously (IVIg) or subcutaneously (SCIg), at hospital or at home. The objective of the VISAGE study was to investigate if route and/or place for IgRT impact patients’ satisfaction regarding IgRT and quality of life (QoL) in real-life conditions. Methods The study enrolled PID patients at least 15 years old receiving IgRT for at least 3 months. Satisfaction and QoL were evaluated at enrollment and over a 12-month follow-up period by Life Quality Index (LQI) which measures 3 dimensions of satisfaction: treatment interference, therapy related problems and therapy settings (factors I, II and III) and SF-36 v2 questionnaire. Results The study included 116 PID patients (mean age 42 ± 18 years, 44 % males, 58 % with scholar or professional occupation) receiving IgRT for a mean of 8.5 ± 8.4 years. At enrollment they were receiving either home-based SCIg (51 %), hospital-based IVIg (40 %) or home-based IVIg (9 %). Patients exhibited a high degree of satisfaction regarding IgRT whatever the route and place for administration. LQI factor I was higher for home-based SCIg (86 ± 2) than for hospital-based IVIg (81 ± 3) and home-based IVIg (73 ± 5; p = 0.02 versus home-based SCIg); no difference was found for LQI factor II; LQI factor III was higher for home-based SCIg (92 ± 2) than for hospital-based IVIg (87 ± 5) and hospital-based IVIg (82 ± 3; p = 0.005 versus home-based SCIg). By contrast, every dimension of QoL was impaired. Over the follow-up period, 10 patients switched from hospital-based IVIg to home-based SCIg and improved LQI factor I (p = 0.004) and factor III (p = 0.02), while no change was noticed in LQI factors II and QoL. Meanwhile, no change in satisfaction or QoL was found in patients with stable route of IgRT. When asked on their preferred place of treatment all but one patient with home-based treatment would choose to be treated at home and 29 % of patients treated at hospital would prefer home-based IgRT. Conclusion PID patients expressed a high degree of satisfaction regarding IgRT, contrasting with impaired QoL. In real-life conditions awareness of patient’s expectations regarding the route or place of IgRT may be associated with further improvement of satisfaction.

Trophic disorders of the extremities are a common complication of systemic sclerosis (SSc), mainly related to microvascular damage. However, SSc seems to be a risk factor for premature athero-thrombotic disease that can affect the peripheral arteries, participate in the occurrence of trophic disorders and promote the occurrence of infectious complications. The objective of this study was to assess the prevalence of arterial disease of the limbs in SSc patients.Consecutive inclusions in the context of a multidisciplinary consultation centered on disability of the hand with collection of clinical data [cardiovascular risk factors (CVRF), history of trophic disorders of ischemic origin, peripheral pulse palpation, Allen maneuver the upper (UL) and lower limbs (LL)], and hemodynamic data (flow recorded by Doppler in radial, ulnar, anterior and posterior tibial arteries, and measurement of systolic indices ankles).Fourteen patients were included (11 right-handers, 2 left-handers, 1 ambidextrous). The sex-ratio male/female was 0.27 and the average age of 58.1±10.4 years. The main CVRF were age and smoking. In the UL, 42.8% of patients had a history of trophic disorders, Allen maneuver was abnormal for 35.7% of the superficial palmar arch, 42.9% of ulnar pulse were not perceived and there was no recordable flow in 25% of ulnar artery. In the LL, 14.3% of patients had already presented trophic disorders toes, Allen maneuver was abnormal for 15.4% of the posterior tibial artery, 25.6% of posterior tibial pulse were not perceived and flow of 15.4% of posterior tibial arteries was pathological.The distal macrovascular disease preferentially affecting the ulnar and posterior tibial arteries with a high frequency to the UL and two times less at LL. The pathophysiology is unclear but it could be a proper manifestation of SSc. It seems necessary that SSc patients have a strict balance of their CVRF and a screening of macrovascular arterial lesions. There is also the question of the place of an anti-atherosclerotic therapy in these patients.

The Ehlers-Danlos syndrome, hypermobility type (EDS-HT) is a rare genetic disease. Diagnosis is based on a combination of clinical criteria described in the classification of Villefranche. Diagnosis is difficult to make because of the lack of specific clinical signs and the absence of genetic testing. The EDS-TH manifests itself manly by musculoskeletal pain and joint hypermobility. Temporomandibular disorders (TMD) are also reported. Our aim was to objectify the presence and to qualify the type of TMD associated with the EDS-HT in order to propose an additional diagnostic argument.A prospective, monocenter case-control study, comparing a cohort of patients suffering from EDS-HT to a paired control group of healthy volunteers has been conducted. Clinical examination was standardized, including a general questioning, an oral examination and a temporomandibular joint examination following the TMD/RDC (temporomandibular disorders/research diagnostic criteria).Fourteen EDS-HT patients and 58 control patients were examined. The prevalence of TMDs (n=13; 92.9% vs. n=4; 6.9%; P=10(-11)) was significantly higher in the EDS-HT group. TMDs occurring in the EDS-HT group were complex, combining several mechanisms in contrast to the control group, where only one mechanism was found in all the patients (n=13; 92.9% vs. n=0; 0.0%).TMDs are strongly associated with RDS-HT. TMDs could therefore be used in the diagnosis of this disease.

To define parameters predictive of lymphoma development in patients with primary Sjögren's syndrome (SS).A multicenter case-control survey was performed to identify predictors of lymphoma. Cases were patients who developed lymphoma after diagnosis of primary SS and were mainly recruited through the Club Rhumatismes et Inflammation network. For each case, 2 controls (matched for disease duration and age) were randomly selected among patients with primary SS and without lymphoma. Cases and controls were compared using univariate analysis and then using multivariate analysis to identify independent predictors of lymphoma.One hundred one patients with primary SS and lymphoma were included. Eighty-seven patients were women (86.1%), and the mean ± SD age at lymphoma diagnosis was 57.4 ± 12.6 years. The most frequent histologic type was B cell non-Hodgkin's lymphoma (NHL) in 99 of 101 patients, with marginal-zone lymphoma in 76 of the 99 patients (76.8%) including 58 (58.6%) with lymphoma of the mucosa-associated lymphoid tissue type. Lymphomas were most frequently located in the salivary glands (43 patients). A specific treatment was initiated at diagnosis in 87 patients with B cell NHL, and 61 patients (61.6%) achieved complete sustained remission after the first line of treatment. In the multivariate analysis, salivary gland enlargement, the presence of rheumatoid factor (RF), low C4, cryoglobulinemia, lymphopenia, and disease activity according to the European League Against Rheumatism Sjögren's Syndrome Disease Activity Index (excluding the lymphoma domain) were found to be predictors of lymphoma. No previous treatment for primary SS was associated with any effect on lymphoma occurrence.In addition to previously known factors predictive of lymphoma occurrence, the independent roles of RF and disease activity were demonstrated in this case-control study of primary SS-associated lymphoma. Our findings highlight the roles of chronic antigenic stimulation and disease activity in the development of this severe complication.

22q11 deletion is a common genetic disorder which associates a polymalformative syndrome to dysimmune features. Autoimmunity and immune deficiency manifestations are often associated, resulting in a therapeutic challenge for this disease.We report a 28-year-old patient who presented with hemorrhagic manifestations leading to the diagnosis of severe thrombocytopenia (15,000/mmRituximab may be an alternative to corticosteroid for the treatment of auto-immune manifestations associated with minor forms of 22q11 deletion syndrome without significant worsening of the immune deficiency.

Introduction Les troubles trophiques des extremites sont une complication frequente de la sclerodermie systemique (ScS), principalement lies a une atteinte microcirculatoire. Cependant la ScS semble etre un facteur de risque d’atteinte atherothrombotique precoce qui peut toucher les arteres peripheriques, participer a la survenue de troubles trophiques et favoriser la survenue de complications infectieuses. Objectif Evaluer la prevalence de l’atteinte arterielle des membres chez les patients ScS. Materiel et methode Inclusions consecutives dans le cadre d’une consultation pluridisciplinaire centree sur le handicap de la main avec recueil de donnees cliniques (facteurs de risque cardiovasculaire [FRCV], antecedent de troubles trophiques d’origine ischemique, palpation des pouls peripheriques, manœuvre d’Allen aux membres superieurs [MS] et inferieurs [MI]), et hemodynamiques (flux des arteres radiales, ulnaires, tibiales anterieures et posterieures enregistres par Doppler continu, et mesure des indices de pression systolique aux chevilles). Resultats Quatorze patients ont ete inclus (11 droitiers, 2 gauchers et 1 ambidextre). Le sex-ratio homme/femme etait de 0,27 et l’âge moyen de 58,1 ± 10,4 ans. Les principaux FRCV etaient l’âge et le tabagisme. Aux MS : 42,8 % des patients avaient un antecedent de troubles trophiques, la manœuvre d’Allen etait anormale pour 35,7 % des arcades palmaires superficielles, 42,9 % des pouls ulnaires n’etaient pas percus et aucun flux n’etait enregistrable pour 25 % des arteres ulnaires. Aux MI : 14,3 % des patients avaient deja presente des troubles trophiques des orteils, la manœuvre d’Allen etait anormale pour 15,4 % des arteres tibiales posterieures, 25,6 % des pouls tibiaux posterieurs n’etaient pas percus et le flux de 15,4 % des arteres tibiales posterieures etait pathologique. Conclusion L’atteinte macrovasculaire distale touchait preferentiellement les arteres ulnaires et tibiales posterieures avec une frequence elevee aux MS et deux fois moindre aux MI mais non negligeable. La physiopathologie est mal connue mais il pourrait s’agir d’une manifestation propre de la ScS. Il semble necessaire que les patients ScS beneficient d’un equilibre strict de leurs FRCV, d’un depistage des atteintes arterielles macrovasculaires. Se pose aussi la question de la place d’un traitement antiatheromateux chez ces patients.

The Tasman International Geospace Environment Radars (TIGER) form part of an international network of similar HF radars called Super Dual Auroral Radar Network (SuperDARN) which explore the impact of solar disturbances on Earth by monitoring the location and velocity of plasma of plasma irregularities and related phenomena occurring in the ionosphere. These radars utilise an Auto Correlation Function (ACF) to measure the changing phase of the ACF between lag times to determine the Doppler frequency and thus the target velocity. With the development of TIGER-3, an all digital radar platform, a novel method of determining target velocities has been proposed. In the proposed method, a comparison of the transmit and receive signal magnitude spectrums is performed to determine the Spectrum Difference Function (SDF). It has been shown that the gradient of SDF in the vicinity of the carrier frequency is proportional to the target Doppler shift. In this paper we consider the constraints of hardware processing on the implementation of the technique and suggest an alternate architecture for the TIGER-3 radar that will allow a dramatic reduction in computational complexity to allow the real-time determination of velocity in conjunction with the normal operation of the receivers. The proposed technique moves the processing from the RF frequency band to a low frequency IF band to reduce the computational length of the Fast Fourier transform (FFT) without compromising the validity of the technique.

Calcinosis cutis constitutes a heterogeneous group of chronic disorder. It can be associated with disturbance of calcium and/or phosphate metabolism (metastatic, tumor calcinosis, calciphylaxis) but may also develop without any metabolic disorder, in particular during the course of connective tissue diseases. Among these, the most common are dermatomyositis and the limited form of systemic sclerosis. The physiopathology of calcinosis cutis is poorly known. It can cause pain, chronic ulcerations, infections, which are sources of sometimes major disability. Treatment of calcinosis is challenging because no drug has been shown to be reliably effective in stopping the progression or decreasing dystrophic calcifications in controlled trials. Calcium blocker and colchicine are generally prescribed as the first line systemic therapy. In the localized forms of small lesions, surgical excision is often effective and sometimes preceded by local treatments (laser therapy, extracorporeal shock wave lithotripsy, topical sodium thiosulfate, etc.) or systemic treatment (minocycline, warfarine). When calcinosis is disseminated, it may require additional treatments (aluminium hydroxyde, bisphosphonates) possibly associated with surgery in case of large lesions. Time to response may be prolonged from weeks to months. The calcinosis cutis can lead to secondary infection, pain and functional disability that have to be prevented.

Erasmus' syndrome is the association between systemic sclerosis and silica exposure.We report a case of this syndrome in a driller-powderman exposed to silica and nitro compounds contained in explosives.Physiopathology and etiologies of systemic sclerosis are still not well known. However, nitric oxide, a product of nitro compounds metabolism, is involved in the physiopathology of the disease: it seems thus licit to wonder about the consequences of an uncontrolled occupational exposure to nitric oxide on the vascular function, already damaged by systemic sclerosis. To a wider extent, our report highlights the importance of a comprehensive and detailed collection of occupational exposures for patients diagnosed with systemic sclerosis.

L’arterite a cellules geantes (ACG) est une vascularite granulomateuse touchant les vaisseaux de moyen et de gros calibres, chez des sujets âges en moyenne de 70 a 80 ans. En 2015, son traitement restera la corticotherapie generale delivree, dans les formes non compliquees, a la dose de 0,7 mg/kg de prednisone. Les formes graves exposent au risque de cecite bilaterale par ischemie retinienne et justifient un traitement precoce par fortes doses en bolus de methylprednisone. Les rechutes sont frequentes, rarement sous une forme ischemique et repondent classiquement a une augmentation de la dose. Les enjeux tiennent neanmoins au developpement de nouvelles strategies d’epargne cortisonique car les suivis de cohorte ont pu montrer qu’a 10 ans, pres de 80 % des patients avaient au moins une complication de la corticotherapie generale. Aucune molecule n’a recu a ce jour d’autorisation de mise sur le marche pour cette indication. Le recours au methotrexate, utilise avec un faible niveau de preuve, pourrait s’enrichir de l’utilisation d’autres immunosuppresseurs dans les formes cortico-resistantes ou dependantes mais la encore, avec une experience tres limitee. Les anti-TNF alpha n’ont definitivement pas d’indication, ceci est confirme par la publication de l’etude HECTOR (adalimumab). Les lesions inflammatoires passent par deux principales voies : les axes interleukine 12-cellules Th1– interferon gamma et interleukine-6-cellules Th17-interleukine-17 ou interleukine-21. De nouvelles voies therapeutiques sont ouvertes par l’utilisation de biotherapies anti-IL6 recepteur (tocilizumab), anti-IL1s, avec des essais therapeutiques de phase 2-3. Cependant, alors meme que la reaction biologique inflammatoire est rapidement maitrisee par le tocilizumab, rien ne permet d’etre certain qu’il en soit de meme pour les lesions vasculaires. Or, la prevention de la morbidite cardio-vasculaire constitue un autre axe majeur d’amelioration de la prise en charge de l’ACG. L’utilisation de l’aspirine a la dose de 75 a 160 mg par jour est classique meme si elle ne repose pas sur des donnees d’essais randomises (etude de la Cochrane en 2014). La comprehension du role de certains marqueurs (microparticules par exemple) pourrait aider a prevenir les complications ischemiques en les ciblant. Le recours aux examens d’imagerie comme le PET-scanner au 18FDG doit etre precise, dans le but d’identifier les patients a haut risque de dilatation–anevrisme et en adapter le traitement.

This paper presents a novel antenna impedance matching system used in the latest Australian SuperDARN class HF radar, at Buckland Park, South Australia. Earlier radar designs used an off-the-shelf log-periodic wideband antenna that is significantly easier to match over the SuperDARN frequency band, but expensive to buy and mount, and had limited capability for azimuthal beamforming. The newer TTFD antenna, used in many recent SuperDARN radars, offers improvement in these areas, but is in essence a narrow band antenna. It is capable of wideband operation at the cost of being difficult to match, frequency dependant, high-impedance and complex load. Previous TTFD matching transformers utilising toroids have been measured and evaluated for their suitability for the Buckland Park radar. A new system based on an LC matching network circuit has been devised to replace them. The design approach and results of the new matching circuit are detailed.

Corticosteroids (CS) remain the main treatment for giant cell arteritis (GCA). The choice of initial prednisone dosage (from 0.5 to 1 mg/kg/d) takes into account the disease severity and comorbidities in order to reduce the drug side effects. Low-dose aspirin may benefit to patients suffering ischemic complications or with multiple cardiovascular risk factors. Randomised controlled trials are necessary to precise its benefit-to-risk ratio. Methotrexate has a moderate corticosteroid sparing effect but it does not prevent cephalic complications and there is no evidence of a reduced frequency of CS adverse effects with this drug. Hydroxychloroquine and infliximab or adalimumab did not prevent relapses in double blind controlled trials. High doses of intravenous methylprednisolone are often prescribed for severe ischemic complications though there is no evidence that such doses are superior to classical doses. In corticosteroid dependent patients, the benefit-to-risk ratio of immunosuppressive drugs is unknown. Dapsone is no longer prescribed due to severe adverse effects. Efficacy of tocilizumab is very promising but its benefit-to-risk ratio in old people is largely unknown. Finding a well tolerated corticosteroid sparing drug remains a challenge and further studies are necessary to reduce the long term rate of cardiovascular events and the burden of CS adverse effects.

Giant cell arteritis, temporal arteritis is a systemic vasculitis, which involves large and medium-sized arteries, especially the extracranial branches of the carotid arteries, in patients above the age of 50. Ischemic complications may involve the eye, orbit, or visual pathway, with blindness being the most feared complication. Visual ischemic complications are observed at least in 25% of patients with giant cell arteritis. Irreversible visual loss is mainly due to acute anterior ischemic optic neuropathy. Thus, in any patient above the age of 50 with any manifestation of ocular ischemia, transient or permanent, giant cell arteritis must be considered and ruled out by an emergent targeted investigation and inflammatory work-up. Steroids remain the treatment of choice for giant cell arteritis and must be instituted immediately upon suspicion of the diagnosis, even in the physician's office. The goal is to protect the eye and visual pathways from irreversible blindness or to prevent contralateral disease. Thus, steroid treatment does not constitute a cure for already-incurred visual loss, which will still carry an unfavourable prognosis.

Background The internal medicine and rheumatology departments of our hospital receive patients with autoimmune diseases whose firstline treatment is long term corticotherapy, responsible for many adverse events (AE). Purpose We conducted a survey in internal medicine and rheumatology departments to assess patients’ needs and expectations concerning therapeutic education with a view to creating an adapted therapeutic education programme (TEP) about long term corticotherapy. Material and methods Our survey has conducted in patients receiving long term oral corticotherapy (≥7.5 mg/day for ≥3 months), hospitalised or followed in internal medicine or rheumatology departments of our hospital. We interviewed patients, assessing their knowledge (by creation of a specific score) and opinion about corticosteroid AE to evaluate their needs about TEP. Patient information sources were collected. Results 110 patients were included. Their average score was 12.5/30 points. The most troublesome AE described by patients were weight gain, lipodystrophy and neuropsychiatric manifestations. Diet induced constraints were the most inconvenient effect for 8% of patients. Most patients were informed about corticosteroid AE by their doctor. Information was sufficient according to 75% of patients and was clear for 89%. 51% of patients would like to receive a personal interview to clarify the information and 49% would prefer a written document. 6 patients suggested designing an internet platform or a smartphone application to help them manage their treatment. Information about corticosteroid AE, role of adjuvant treatments and diet were requested. Conclusion Patients feel well informed about corticosteroid AE but their scores reflect their lack of knowledge. Diet is troublesome for many patients and could lead to poor compliance or refusal of treatment. After discussion with internists and collaboration with the dietetic team, it was agreed to systematically propose a meeting with patients at the start of corticotherapy. A clear and lucid information sheet about the recommended diet is being developed. Patients’ expectations towards TEP are wide and confirm the interest in creating an educational process or a TEP dedicated to patients receiving long term corticotherapy in our hospital. References and/or Acknowledgements Hervier B, et al. Evaluation des attentes des patients pour un programme d’education therapeutique au cours du lupus systemique. Rev Med Interne 2014;35:297-302. No conflict of interest.

Background Corticosteroids are widely prescribed drugs in current practice but their use may be limited by their clinical and biological adverse events (AEs), affecting about two­-thirds of treated patients. Patients can be distrustful towards corticotherapy because of its AEs, probably because of a lack of information. Purpose We conducted a prospective study in departments of internal medicine and rheumatology to assess knowledge about corticosteroid AEs by a cohort of patients treated with long term oral corticotherapy. Material and methods Patients treated with long term oral corticotherapy (≥7.5 mg/day for ≥3 months), hospitalised or followed in internal medicine or rheumatology departments were included over a 4 month period. A score of patients’ knowledge about corticosteroid AEs has been created. Its scale has been fixed according to the frequency and gravity of corticosteroid AEs described in literature. A statistical analysis has defined the variables influencing significantly the knowledge patients’ score about corticosteroid AEs and the predictive variables of this score. Results 110 patients were included in the study. The average score obtained by patients was 12.5/30 points. 81% of patients scored below average. The main variables influencing our score were the patients’ school level, a long period of corticotherapy, patients’ general knowledge about corticotherapy and their diet, and patients’ opinion about AEs. Predictive variables of this score were patients’ general knowledge about corticotherapy and diet, and the number of AEs felt by the patients. Conclusion Scores obtained by our patients reflect a real ignorance of corticosteroid AEs. The predictive and influencing variables of this score showed the importance of patient information and education. This will allow us to target the patients’ gaps and to create suitable educational tools as part of a therapeutic educational programme (TEP). Implementation of a TEP is primordial to improve patients’ knowledge and opinion about the AEs of long term corticotherapy. Our score could allow assessment of the impact of a TEP on patients’ opinion and adherence to their treatment. References and/or Acknowledgements Fardet L, et al . Corticosteroid-induced clinical adverse events:frequency, risk factors and patient’s opinion, Br J Dermatol 2007;157:142–8 Fardet L, et al . Internal medicine physicians’ perception of frequency and impact of corticosteroid-induced adverse events, Rev Med Interne 2009;30:113–18 No conflict of interest.

Giant cell arteritis, temporal arteritis is a systemic vasculitis, which involves large and medium-sized arteries, especially the extracranial branches of the carotid arteries, in patients above the age of 50. Ischemic complications may involve the eye, orbit, or visual pathway, with blindness being the most feared complication. Visual ischemic complications are observed at least in 25% of patients with giant cell arteritis. Irreversible visual loss is mainly due to acute anterior ischemic optic neuropathy. Thus, in any patient above the age of 50 with any manifestation of ocular ischemia, transient or permanent, giant cell arteritis must be considered and ruled out by an emergent targeted investigation and inflammatory work-up. Steroids remain the treatment of choice for giant cell arteritis and must be instituted immediately upon suspicion of the diagnosis, even in the physician's office. The goal is to protect the eye and visual pathways from irreversible blindness or to prevent contralateral disease. Thus, steroid treatment does not constitute a cure for already-incurred visual loss, which will still carry an unfavourable prognosis.

Lipomas usually extend in subcutaneous tissues and rarely may be compressive. We report a case of neck lipoma resulting in jugular vein thrombosis and pulmonary embolism in a patient treated by clozapine. Clozpine may be considered an associated risk factor for thrombosis. This case suggests that performing a regional evaluation may be particularly important when thrombophlebitis occurs.

Lipomas usually extend in subcutaneous tissues and rarely may be compressive. We report a case of neck lipoma resulting in jugular vein thrombosis and pulmonary embolism in a patient treated by clozapine. Clozpine may be considered an associated risk factor for thrombosis. This case suggests that performing a regional evaluation may be particularly important when thrombophlebitis occurs.

Summary. Background: A strong association between bilateral deep vein thrombosis (DVT) and cancer had been found in one retrospective study. To confirm this finding, consecutive patients with an objective diagnosis of bilateral DVT were followed over 12 months. Patients and methods: One-hundred and three patients, hospitalized for bilateral DVT, were included in the study. Twenty-six patients (25.2%) were already known to have a cancer, 26 (25.2%) had a previous history of venous thromboembolic disease, 44 (42.7%) had a symptomatic pulmonary embolism. The patients were scheduled to be prospectively followed up at 3, 6 and 12 months as outpatients. Information on recurrence, evidence of a new overt cancer and the cause of death were recorded for all patients. Results: A new cancer was diagnosed in 20 (26%) of the 77 patients without known cancer at admission. The risk of cancer was significantly more important in idiopathic thrombosis than in patients with secondary thrombosis (40.5% vs. 12.5%; odds ratio 4.8, 95% confidence interval 1.4, 18.8). Seventy percent of the cancers discovered had already spread. Age, gender, presence of pulmonary embolism, recurrence and location of the thrombosis were not statistically associated with the risk of cancer. The 1-year survival rates of patients with a previously known cancer and patients with a newly discovered cancer were, respectively, 26% and 35% (P = 0.33). Conclusion: Bilateral DVT is a significant risk indicator of malignancy. Cancer is present in 45% of patients with bilateral DVT and is associated with a poor prognosis.