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International audience; ObjectivesLupus nephritis (LN) is a frequent complication of systemic lupus erythematosus (SLE). Severe (proliferative) forms of LN are treated with induction immunosuppressive therapy (IST), followed by maintenance IST, to target remission and avoid relapses. The optimal duration of maintenance IST is unknown. The WIN-Lupus trial tested whether IST discontinuation after 2‒3 years was non-inferior to IST continuation for two more years in proliferative LN.MethodsWIN-Lupus was an investigator-initiated multicentre randomised controlled trial. Patients receiving maintenance IST with azathioprine or mycophenolate mofetil for 2–3 years, and hydroxychloroquine, were randomised (1:1) into two groups: (1) IST continuation and (2) IST discontinuation. The primary endpoint was the relapse rate of proliferative LN at 24 months. Main secondary endpoints were the rate of severe SLE flares, survival without renal relapse or severe flare, adverse events.ResultsBetween 2011 and 2016, 96 patients (out of 200 planned) were randomised in WIN-Lupus: IST continuation group (n=48), IST discontinuation group (n=48). Relapse of proliferative LN occurred in 5/40 (12.5%) patients with IST continuation and in 12/44 (27.3%) patients with IST discontinuation (difference 14.8% (95% CI −1.9 to 31.5)). Non-inferiority was not demonstrated for relapse rate; time to relapse did not differ between the groups. Severe SLE flares (renal or extrarenal) were less frequent in patients with IST continuation (5/40 vs 14/44 patients; p=0.035). Adverse events did not differ between the groups.ConclusionsNon-inferiority of maintenance IST discontinuation after 2‒3 years was not demonstrated for renal relapse. IST discontinuation was associated with a higher risk of severe SLE flares.Trial registration numberNCT01284725.

Chikungunya nephropathy is an uncommon etiology of acute kidney injury, associated with the mosquito-borne chikungunya arbovirus (CHIKV). The very limited number of pathologic reports to date have only involved postmortem analyses. We here report 5 cases of acute kidney injury for which kidney biopsies were performed in patients with confirmed acute CHIKV infection, during the recent outbreak of chikungunya disease in the French West Indies. The patients ranged from 42 to 76 years of age. All of the patients developed kidney injury, 3 of whom required short-term dialysis and underwent a kidney biopsy. Analysis of kidney biopsies revealed 2 main histopathologic patterns: acute interstitial nephritis with predominant lymphoid inflammation and acute tubular injury. Epithelioid granulomas were observed in 2 cases. There were no glomerular lesions, except in biopsies from 2 patients, including 1 with a previous known primary focal segmental glomerulosclerosis. CHIKV antigen immunofluorescence microscopy revealed staining in tubular cells. In all of the cases, the short-term outcome was favorable, with recovery of kidney function.

Rapidly progressive interstitial lung disease (RP-ILD) is a frequent and severe manifestation of anti-MDA5 dermatomyositis (MDA5-DM) associated with poor outcome. The optimal treatment regimen for MDA5-DM RP-ILD is yet to be determined. Specifically, the value of adding plasma exchange (PLEX) to corticosteroids and immunosuppressants remains unclear. We aimed to evaluate the effect of PLEX on the outcome of patients with MDA5-DM RP-ILD.This French nationwide multicentre retrospective study included all MDA5-DM RP-ILD patients from 2012 to 2021 admitted to 18 centres. The primary endpoint was one-year transplant-free survival.51 patients with MDA5-DM RP-ILD (female 67%; mean age at disease onset: 51 ± 11.6 years) were included. Thirty-two (63%) patients required mechanical ventilation and twenty-five (49%) received PLEX. One-year mortality or lung transplant occurred in 63% cases after a median follow-up of 77 [38-264] days. The Cox proportional hazards multivariable model only retained mechanical ventilation but not PLEX (p = 0.7) as independent predictor of the primary endpoint. One-year transplant-free survival rates in PLEX + vs. PLEX-were 20% vs. 54% (p = 0.01), respectively. The Kaplan-Meier estimated probabilities of one-year transplant-free survival was statistically higher in PLEX-compared to PLEX + patients (p = 0.05). PLEX + compared to PLEX-patients more frequently received mechanical ventilation and immunosuppressants suggesting PLEX + patients had a more severe disease.MDA5-DM RP-ILD is associated with poor rate of one-year transplant-free survival. The use of PLEX was not associated with a better outcome albeit they were mainly given to more severe patients. While our study reports the largest series of MDA5-DM RP-ILD given PLEX, these results needs to be interpreted with caution owing the numerous selection, indication and interpretation bias. Further studies are needed to evaluate their efficacy in this setting.

Introduction: Systemic diseases of pediatric onset are more frequent in the Afro-Caribbean population. We performed a study of patients followed in French overseas departments of America (FOAD) for pediatric systemic lupus (pSLE). The aims were to describe their clinical and biological specificities during childhood.Methods: A retrospective study was conducted between January 2000 and September 2021. Listings of patients with pediatric onset were obtained through multiple sources: computerized hospital archives, registry of referent pediatricians and adult specialists in internal medicine and French National Registry for rare diseases. The spectrum of diseases studied was systemic lupus according to international criteria with pediatric onset.Results: Overall, 2148 patients were identified, and 54 patients were included. The average follow-up was 8.3 years (range: 0.3 - 25 years). We found an increase in new diagnoses throughout the years. At onset, pSLE patients had a median of 10 SLICC criteria (range: 4-12), and the median EULAR/ACR 2019 score was 38 (12 - 54). At onset, one-third of patients had renal involvement, 15% neurolupus and 41% cardiac involvement. During childhood, 54% had renal involvement, and 26% suffered from neurolupus. Patients suffered a median of 3 flares during childhood and 26% from more than 5 flares. Younger patients at onset had worst outcomes; they had more flares (median at 5 p = 0,02) and needed an average of 4 background therapies (p = 0,04).Conclusion: The outcomes of Afro-Caribbean patients, where pSLE is more frequent, were similar to those in Western countries with worse disease activity at onset. Further studies should be performed to identify the genetic and environmental factors in this population.

Transplacental passage of maternal anti-SSA and anti-SSB antibodies, potentially associated with maternal autoimmune diseases, can cause neonatal lupus syndrome. Given the paucity of data in this setting, we report short- and long-term outcomes of mothers of offspring with congenital heart block (CHB).This retrospective study included anti-SSA/SSB antibody-positive mothers of fetuses with high-degree CHB and focused on their health status before pregnancy, at CHB diagnosis, and thereafter.We analyzed 215 women with at least 1 pregnancy with CHB. Prior to this diagnosis, only 52 (24%) mothers had been diagnosed with an autoimmune disease, mainly systemic lupus erythematosus (SLE; n = 26, 12%) and Sjögren syndrome (SS; n = 16, 7%). Six more were diagnosed with an autoimmune disease during the index pregnancy. Of the 157 mothers (73%) with no such diagnosis at childbirth, 77 (49%) developed one after a median follow-up of 11 years (range: 21 days to 54 years). By the end of follow-up, 135 women (63%) had an autoimmune disease diagnosis, mainly SLE (n = 54, 25%) and SS (n = 72, 33%). Three patients with SLE had renal involvement, and only 6 (3%) had required an immunosuppressive drug at any point. The symptoms best predicting autoimmune disease development were arthralgia and myalgia (One-quarter of the patients had an autoimmune disease diagnosis at the time of the fetal CHB diagnosis. Nearly half of those without an initial diagnosis progressed during follow-up, most without severe manifestations. Severe diseases such as lupus nephritis were rarely seen, and immunosuppressive drugs were rarely required.

BACKGROUND AND AIMS Lupus nephritis (LN) is a frequent complication of systemic lupus erythematosus (SLE). Severe (proliferative) forms of LN are treated with an induction immunosuppressive therapy (IST), followed by a maintenance IST, to target remission and avoid relapses. The optimal duration of maintenance IST for proliferative LN is unknown. The WIN-Lupus trial tested whether IST discontinuation after 2–3 years in proliferative LN was non-inferior to IST continuation for 2 more years. METHOD WIN-Lupus is an investigator-initiated academic randomized controlled trial, conducted in 28 French centres. Patients on maintenance IST with azathioprine or mycophenolate mofetil for a minimum of 2 years and a maximum of 3 years, and those who were taking hydroxychloroquine were randomized (1:1) between two groups: IST continuation and IST discontinuation. The primary endpoint was the relapse rate of proliferative LN at 24 months. Secondary endpoints were the rate of severe SLE flares, survival without renal relapse or severe flare, adverse events, kidney function, disease activity, corticosteroid exposure, patient-reported outcome and medico-economic impact. RESULTS Between 2011 and 2016, 125 patients were screened and 96 were randomized in the trial: 48 in the IST continuation group and 48 in the IST discontinuation group. In the per-protocol population, a relapse of proliferative LN occurred in 5/40 (10.4%) patients with IST continuation, and in 12/44 (25%) patients with IST discontinuation (difference 14.8%, 95% CI [−1.9; 31.5]). Noninferiority was not demonstrated for relapse rate. Time to renal relapse did not differ between groups (P = 0.092). Severe SLE flares (renal or extrarenal) were less frequent in patients with IST continuation compared to IST discontinuation (5/40 versus 14/44 patients; P = 0.035). IST discontinuation was associated with lower health-related costs. Adverse events did not differ between groups. CONCLUSION Noninferiority of maintenance IST discontinuation after 2 to 3 years was not demonstrated for renal relapse. IST discontinuation was associated with a higher risk of severe SLE flare.

Objective Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by asthma, hypereosinophilia and ANCA positivity in 40% of patients. Renal involvement is rare and poorly described, leading to this renal biopsy-proven based study in a large EGPA cohort. Methods We conducted a retrospective multicentre study including patients fulfilling the 1990 ACR criteria and/or the 2012 revised Chapel Hill Consensus Conference criteria for EGPA and/or the modified criteria of the MIRRA trial, with biopsy-proven nephropathy. Results Sixty-three patients [27 women, median age 60 years (18–83)] were included. Renal disease was present at vasculitis diagnosis in 54 patients (86%). ANCA were positive in 53 cases (84%) with anti-MPO specificity in 44 (83%). All patients had late-onset asthma. Peripheral neuropathy was present in 29 cases (46%), alveolar haemorrhage in 10 (16%). The most common renal presentation was acute renal failure (75%). Renal biopsy revealed pauci-immune necrotizing GN in 49 cases (78%). Membranous nephropathy (10%) and membranoproliferative GN (3%) were mostly observed in ANCA-negative patients. Pure acute interstitial nephritis was found in six cases (10%); important interstitial inflammation was observed in 28 (44%). All patients received steroids with adjunctive immunosuppression in 54 cases (86%). After a median follow-up of 51 months (1–296), 58 patients (92%) were alive, nine (14%) were on chronic dialysis and two (3%) had undergone kidney transplantation. Conclusion Necrotizing pauci-immune GN is the most common renal presentation in ANCA-positive EGPA. ANCA-negative patients had frequent atypical renal presentation with other glomerulopathies such as membranous nephropathy. An important eosinophilic interstitial infiltration was observed in almost 50% of cases.

Aseptic abscess (AA) syndrome is a rare type of inflammatory disorder involving polymorphonuclear neutrophils (PMNs), often associated with inflammatory bowel disease (IBD). This study sought to describe the clinical characteristics and evolution of this syndrome in a large cohort. We included all patients included in the French AA syndrome register from 1999 to 2020. All patients fulfilled the criteria outlined by André et al. in 2007. Seventy-one patients were included, 37 of which were men (52.1%), of a mean age of 34.5 ± 17 years. The abscesses were located in the spleen (71.8%), lymph nodes (50.7%), skin (29.5%), liver (28.1%), lung (22.5), and rarer locations (brain, genitals, kidneys, ENT, muscles, or breasts). Of all the patients, 59% presented with an associated disease, primarily IBD (42%). They were treated with colchicine (28.1%), corticosteroids (85.9%), immunosuppressants (61.9%), and biologics (32.3%). A relapse was observed in 62% of cases, mostly in the same organ. Upon multivariate analysis, factors associated with the risk of relapse were: prescription of colchicine (HR 0.52; 95% CI [0.28–0.97]; p = 0.042), associated IBD (HR 0.57; 95% CI [0.32–0.99]; p = 0.047), and hepatic or skin abscesses at diagnosis (HR 2.14; 95% CI [1.35–3.40]; p = 0.001 and HR 1.78; 95% CI [1.07–2.93]; p = 0.024, respectively). No deaths occurred related to this disease. This large retrospective cohort study with long follow up showed that AA syndrome is a relapsing systemic disease that can evolve on its own or be the precursor of an underlying disease, such as IBD. Of all the available treatments, colchicine appeared to be protective against relapse.

Lupus nephritis (LN) has been described as having worse survival and renal outcomes in African-descent patients than Caucasians. We aimed to provide long-term population-based data in an Afro-descendant cohort of LN with high income and easy and free access to specialized healthcare. Study design: We performed a retrospective population-based analysis using data from 2002–2015 of 1140 renal biopsies at the University Hospital of Martinique (French West Indies). All systemic lupus erythematosus patients with a diagnosis of LN followed for at least 12 months in Martinique or who died during this period were included. Results: A total of 89 patients were included, of whom 68 (76.4%) had proliferative (class III or IV), 17 (19.1%) had membranous (class V), and 4 (4.5%) had class I or II lupus nephritis according to the ISN/RPS classification. At a mean follow-up of 118.3 months, 51.7% of patients were still in remission. The rates of end-stage renal disease were 13.5%, 19.1%, and 21.3% at 10, 15, and 20 years of follow-up, respectively, and mortality rates were 4.5%, 5.6%, and 7.9% at 10, 15, and 20 years of follow-up, respectively. Conclusions: The good survival of our Afro-descendant LN patients, similar to that observed in Caucasians, shades the burden of ethnicity but rather emphasizes and reinforces the importance of optimizing all modifiable factors associated with poor outcome, especially socioeconomics.

National audience; Objectif Évaluer la performance de l’échographie des glandes salivaires pour le diagnostic du syndrome de Gougerot-Sjögren primitif (SGSp) ou secondaire (SGSs). Méthodes Étude transversale multicentrique sur 97 patients présentant un syndrome sec clinique. Le SGSp (n = 39) et le SGSs (n = 22) remplissaient les critères de classification du groupe de consensus américano-européen (AECG, American-European Consensus Group). Les témoins (n = 36) étaient des patients présentant un syndrome sec mais ne remplissant pas les critères de l’AECG. L’échostructure des quatre glandes salivaires principales a été évaluée selon quatre méthodes : le score de Salaffi (0–16), le score de Jousse-Joulin (0–4), le score de Hocevar (0–48) et le score de Milic (0–12). Résultats Les résultats médians des scores échographiques étaient plus élevés dans les groupes SGSp et SGSs que chez les témoins (p < 0,001). Selon les courbes ROC et le rapport de vraisemblance positif (RV + ), les quatre scores ont montré une bonne performance diagnostique pour le SGSp et le SGSs. L’aire sous la courbe (ASC) du SGSp et du SGSs était respectivement de 0,891 (IC 95 % 0,812–0,970) et de 0,824 (IC 95 % 0,695–0,954) pour le score de Hocevar, de 0,885 (IC 95 % 0,804–0,965) et de 0,808 (IC 95 % 0,673–0,943) pour le score de Milic, de 0,915 (IC 95 % 0,848–0,982) et de 0,844 (IC 95 % 0,724–0,965) pour le score de Salaffi et de 0,897 (IC 95 % 0,821–0,973) et de 0,851 (IC 95 % 0,735–0,968) pour le score de Jousse-Joulin. Cette étude a mis en évidence une reproductibilité interobservateur intéressante (kappa 0,714 ± 0,131) de l’évaluation échographique, avec un accord entre les lecteurs de 85,7 % pour la détermination du caractère pathologique des glandes salivaires. Conclusion L’échographie des glandes salivaires est une procédure d’imagerie simple, non invasive et performante pour le diagnostic du SGSp et du SGSs selon les scores de Salaffi, de Milic et de Jousse-Joulin.

ObjectivesThe predominance of extramuscular manifestations (e.g., skin rash, arthralgia, interstitial lung disease [ILD]) as well as the low frequency of muscle signs in anti–melanoma differentiation-associated gene 5 antibody–positive (anti-MDA5+) dermatomyositis caused us to question the term myositis-specific antibody for the anti-MDA5 antibody, as well as the homogeneity of the disease.MethodsTo characterize the anti-MDA5+ phenotype, an unsupervised analysis was performed on anti-MDA5+ patients (n = 83/121) and compared to a group of patients with myositis without anti-MDA5 antibody (anti-MDA5−; n = 190/201) based on selected variables, collected retrospectively, without any missing data.ResultsWithin anti-MDA5+ patients (n = 83), 3 subgroups were identified. One group (18.1%) corresponded to patients with a rapidly progressive ILD (93.3%; p < 0.0001 across all) and a very high mortality rate. The second subgroup (55.4%) corresponded to patients with pure dermato-rheumatologic symptoms (arthralgia; 82.6%; p < 0.01) and a good prognosis. The third corresponded to patients, mainly male (72.7%; p < 0.0001), with severe skin vasculopathy, frequent signs of myositis (proximal weakness: 68.2%; p < 0.0001), and an intermediate prognosis. Raynaud phenomenon, arthralgia/arthritis, and sex permit the cluster appurtenance (83.3% correct estimation). Nevertheless, an unsupervised analysis confirmed that anti-MDA5 antibody delineates an independent group of patients (e.g., dermatomyositis skin rash, skin ulcers, calcinosis, mechanic's hands, ILD, arthralgia/arthritis, and high mortality rate) distinct from anti-MDA5− patients with myositis.ConclusionAnti-MDA5+ patients have a systemic syndrome distinct from other patients with myositis. Three subgroups with different prognosis exist.

(1) Background: The prevalence of Spondyloarthritis (SpA) varies significantly in different regions and ethnic groups due several factors such as heterogeneity in study populations, the diversity of classification criteria used in epidemiological studies, the prevalence variability of HLA-B27 or disparity in healthcare access. To our knowledge, there is no data on SpA in Martinique, a French region in the Caribbean with a predominantly Afro-descendant population and a high level of healthcare. (2) Methods: This was a retrospective study of all SpA patients treated at the Fort de France University Hospital between 1 January 1997 and 1 January 2008. (3) Results: In our cohort of 86 SpA patients, age at diagnosis was late (41 years old), ankylosing spondylitis (AS) was the most frequent sub-type (60.5%), inflammatory bowel disease was the most frequent extra articular feature (23.3%) and no one had personal familial history of the disease. Inflammatory syndrome concerned 55.6% of patients, no one was positive for HIV and HLA-B27 positivity was low (42.2%). However, HLA-B27 was statistically associated with AS. Out of 64 patients, 41 had sacroiliitis. (4) Conclusion: To our knowledge, this is the first comprehensive descriptive study of SpA subtypes in Martinique, a French region in the Caribbean. We report clinical and biological similarities in our SpA cohort with those of sub-Saharan Africa and with SpA subtypes reported in Afro-descendant populations.

Objective.To assess the reproducibility and the utility of the 6-minute walk test (6MWT) in systemic sclerosis (SSc).Methods.All patients with SSc who underwent at least two 6MWT within a minimum 3-month interval plus simultaneous routine clinical, biological, and functional evaluations were consecutively enrolled in this observational study over 6 years. Following American Thoracic Society guidelines, each 6MWT was repeated twice to assess the 6-minute walk distance (6MWD) reproducibility, with the highest value being reported for subsequent analysis.Results.Among 56 (38 female) included patients aged 46 ± SD 12.7 years, with 17 ± 10 modified Rodnan skin score (mRSS) and 1 ± 0.8 Scleroderma Health Assessment Questionnaire (SHAQ) at first referral, 277 6MWT evaluations (5 ± 3.9 6MWT per patient) were performed over 23 ± 22.5 months followup. Meanwhile, 8 deaths (87.5% SSc-related) occurred. The mean 6MWD absolute value was 457 ± 117 m with a 4 ± 2.2 mean Borg dyspnea score. The 6MWD intraclass correlation coefficient was 0.996 (95% CI 0.995–0.999, p < 0.0001). In multivariate linear regression analysis, these factors were independently associated with a lower 6MWD: sex (R2 = 0.47, p < 0.0001), mRSS (R2 = 0.47, p = 0.008), tendon friction rub (R2 = 0.47, p = 0.003), SHAQ (R2 = 0.47, p = 0.02), muscle disability score (R2 = 0.47, p = 0.03), DLCO% (R2 = 0.47, p = 0.0008), and left ventricular ejection fraction (R2 = 0.47, p = 0.006). The 6MWD at first referral was an independent predictor for the overall mortality (HR 0.99, 95% CI 0.988–0.999) and the SSc-related mortality (HR 0.99, 95% CI 0.988–0.999).Conclusion.We show strong reproducibility for the 6MWD and confirm the 6MWT utility to assess the overall prognosis of patients with SSc.

Resume Objectifs Des etudes ont releve une frequence moins elevee de la polyarthrite rhumatoide (PR) dans la population africaine. Il n’existe cependant aucune donnee precise concernant la population d’origine afro-caribeenne. Le projet EPPPRA est une etude epidemiologique prospective visant a definir la prevalence et les caracteristiques cliniques de la PR aux Antilles francaises (Martinique, Guadeloupe, Guyane francaise). Methodes EPPPRA regroupe l’ensemble des rhumatologues exercant aux Antilles francaises auxquels il a ete propose d’inclure pendant une periode d’un an tous les patients ayant un diagnostic clinique de PR. Nous rapportons ici les resultats obtenus en Martinique. Resultats D’apres les resultats de l’EPPPRA, la prevalence globale de la PR standardisee selon l’âge (population mondiale de reference) est estimee a 0,10 % [IC 95 % : 0,09 % a 0,11 %] en Martinique avec une nette predominance feminine (88,1 %) et 93,1 % des patients auto-declares d’origine ethnique afro-caribeenne. L’âge moyen au moment du diagnostic etait de 49,6 ± 16,0 ans. Dans la majorite des cas, les sujets remplissaient au moins 4 criteres (94,4 %) de la classification 1987 de l’American College of Rheumatology (ACR) et obtenaient un minimum de 6 points (78,2 %) selon la classification 2010 de l’ACR/European League Against Rheumatism (EULAR). Un taux eleve de seropositivite a ete retrouve (84,2 %). Malgre le retentissement fonctionnel observe chez 40,5 % des patients, la maladie etait faiblement active chez 71,4 % d’entre eux. Le traitement le plus courant etait le Methotrexate (73 %), suivi des biotherapies (24,4 %). De nombreux patients (68,6 %) ont egalement beneficie d’une corticotherapie. Les facteurs de risque cardiovasculaire tres frequents contrastaient avec une consommation de tabac particulierement basse (8,7 %). Conclusion Ce travail met en evidence la faible prevalence standardisee de la PR observee dans une population francaise d’origine afro-caribeenne et en souligne les caracteristiques specifiques (forte predominance feminine, taux de seropositivite eleve, faible consommation de tabac). Malgre la facilite d’acces aux soins de sante et aux biotherapies, la PR est d’evolution destructrice au retentissement fonctionnel invalidant dans environ la moitie des cas de cette etude.

The Francophone Society of Bone Marrow Transplantation and Cellular Therapy (SFGM-TC) organized the 7th allogeneic hematopoietic stem cell transplantation clinical practices harmonization workshop series in September 2017 in Lille, France and updated recommendations for indications and follow-up in autologous hematopoietic stem cell transplantation in autoimmune and autoinflammatory diseases, previously published under the auspices of SFGM-TC.

Background Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear. Methods We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies. Results Among 187 neonates with CHB, 35 (18.8%, one missing data) had DCM and 22 (11.8%) died during a median follow-up of 7years [range: birth–36years]. On multivariate analysis, factors associated with postnatal DCM were in utero DCM ( P =0.0199; HR=3.13 [95% CI: 1.20–8.16]), non-European origin ( P =0.0052; HR=4.10 [95% CI: 1.81–9.28]) and pacemaker implantation ( P =0.0013; HR=5.48 [95% CI: 1.94–15.47]). Postnatal DCM could be categorized in two subgroups: neonatal DCM (n=13, diagnosed at a median age of 0day [birth–4days]) and late-onset DCM (n=22, diagnosed at a median age of 15.2months [3.6months–22.8years]). Factors associated with neonatal DCM were in utero DCM, hydrops, endocardial fibroelastosis and pericardial effusion, whereas those associated with late-onset DCM were non-European origin, in utero mitral valve insufficiency, and pacemaker implantation. Fluorinated steroids showed no protective effect against late-onset DCM ( P =0.27; HR=1.65 [95% CI: 0.63–4.25]). Probability of survival at 10years was 23.1% for newborns diagnosed neonatally with DCM, 53.9% for those who developed late-onset DCM, and 98.6% for those without DCM. Conclusion Neonatal and late-onset DCM appear to be two different entities. None of the known risk factors associated with neonatal DCM predicted late-onset DCM. Long-term follow-up of cardiac function is warranted in all children with CHB.

Introduction Le canal lombaire etroit est (CLE) est une pathologie invalidante qui touche principalement les populations vieillissantes et represente un cout de sante important. La presentation clinique associant claudication neurogene et lombalgies resulte d’un retrecissement congenital ou degeneratif du canal rachidien. Ce retrecissement plus important en population Africaine contraste avec une incidence plus faible de la maladie dans cette population. Dans les principaux essais cliniques et meta-analyses portant sur les traitements du CLE, 70 a 95 % des patients etudies sont d’origine caucasienne et aucune difference d’efficacite entre chirurgie, reeducation ou infiltrations epidurales de corticosteroides n’est observee. A ce jour, il n’existe aucune etude therapeutique comparative en population d’origine africaine (afro descendante : AD). Nous souhaitons comparer trois approches therapeutiques dans le CLE en Martinique, ile francaise des Caraibes avec un niveau de soins eleve comparativement aux autres iles de la region et une population vieillissante majoritairement AD. Patients et methodes Cohorte prospective de 138 patients martiniquais, AD, presentant un CLE, evalues a 3 mois d’une premiere serie d’infiltration epidurales de corticosteroides associe a une reeducation standardisee, puis affectes dans 3 bras therapeutiques en fonction de leur evolution clinique et de leurs souhaits : reeducation (R : kinesitherapie non standardisee et traitements oraux), deuxieme serie d’infiltrations epidurales de corticosteroides (EPI2) ou chirurgie de decompression (CD). Critere principal d’evaluation : evolution de l’Oswestry Disability Index (ODI) a 3 mois (M3), M12, M18, M24. Criteres secondaires d’evaluation : evolution des douleurs radiculaires et lombaires, mesuree par l’EVA (respectivement EVA-R et EVA-L). Resultats 71 patients ont ete affectes dans le bras R, 37 dans le bras EPI2 et 30 dans le bras CD. Tous les bras etaient comparables en âge (62,3 ± 13,1 ans), sexe (ratio homme/femme 0,75) et IMC avec une moyenne de 27,0 ± 5,97 kg/m2. L’ODI et l’EVA-R etaient significativement plus eleves dans le bras CD a baseline (p Discussion Nous observons une superiorite de la CD sur l’evolution de la EVA-R et de l’ODI, score fonctionnel composite, a M3 et M12. Ces resultats sont similaires a ceux des principales etudes comparant traitements chirurgicaux et non chirurgicaux du CLE. Si les resultats favorables a long terme (M24) sur l’ODI et l’EVA-R ne sont pas repliques, il existe une tendance en faveur de la chirurgie. L’absence de significativite statistique pourrait s’expliquer par un nombre important de perdus de vu. Conclusion Il s’agit de la premiere etude comparative des traitements du CLE realisee en population AD, montrant une superiorite de la CD sur l’evolution clinique des patients.

Nonadherence to treatment is a major cause of lupus flares. Hydroxychloroquine (HCQ), a major medication in systemic lupus erythematosus, has a long half-life and can be quantified by high-performance liquid chromatography. This international study evaluated nonadherence in 305 lupus patients with flares using drug levels (HCQ

Objectives Studies suggest that rheumatoid arthritis (RA) is less frequent in African populations. However, no recent precise data exists for Afro-Caribbeans. The EPPPRA project is a prospective epidemiological survey to describe prevalence and clinical aspects of RA in the French West Indies (Martinique, Guadeloupe, French Guiana). Methods EPPPRA involved all rheumatologists from the French West Indies who included all patients with a known clinical diagnosis of RA, during a one-year period. We outline here results for Martinique. Results EPPPRA estimated an overall world age-standardized prevalence of RA at 0.10% [95% CI 0.09% to 0.11%] in Martinique, with a high female predominance (88.1%) and 93.1% of self-reported Afro-Caribbeans. Mean age at diagnosis was 49.6 ± 16.0 years. A majority of subjects presented at least 4 criteria points from the 1987 American College of Rheumatology (ACR) classification (94.4%) and at least 6 points (78.2%) from the 2010 ACR/European League Against Rheumatism (EULAR) classification. A high immune seropositivity rate was highlighted (84.2%). Despite functional impact observed in 40.5% of patients, 71.4% presented a low disease activity level. Methotrexate was the most common ongoing treatment (73%), followed by biotherapies (24.4%). Numerous patients (68.6%) received a steroid regimen. Cardiovascular risk factors were very frequent, contrasting with a very low tobacco use (8.7%), Conclusion This work outlines low standardized prevalence of RA in a French Afro-Caribbean population with specific characteristics (high female predominance, high immune seropositivity, low tobacco use). Despite easy access to care and biotherapies, approximately half of RA patients still present destructive disease with functional impact.

Resume Introduction La cellulite de Wells est une dermatose eosinophilique rare associant des lesions erythemateuses inflammatoires, une hypereosinophilie sanguine frequente et des images histologiques evocatrices. Elle pose le probleme du diagnostic differentiel avec les autres dermatoses eosinophiliques. Classiquement, le traitement repose sur la corticotherapie systemique. Observation Nous rapportons l’observation d’une patiente de 63 ans qui consultait pour une eruption maculo-papuleuse prurigineuse des membres et du tronc, d’extension rapide, confluente en placards erythemateux bien limites, indures et douloureux. Le bilan biologique revelait une hypereosinophilie sanguine. L’histologie montrait un infiltrat inflammatoire eosinophile perivasculaire et autour des fibres de collagene. Un traitement par dermocorticoides seuls permettait la regression des symptomes. Conclusion Le diagnostic positif de la cellulite de Wells est difficile en l’absence de signe specifique ; elle doit etre evoquee devant l’association de donnees cliniques, biologiques et histologiques evocatrices. L’efficacite et la bonne tolerance des dermocorticoides locaux doit conduire a les utiliser en premiere intention.

International audience; BACKGROUND:As intravenous immunoglobulins (IVIG) exhibit immunomodulatory and antifibrotic properties, they may be a relevant treatment for systemic sclerosis (SSc). The objectives of this work were thus to report on the efficacy and safety of IVIG in a population of SSc patients and to review the available literature.METHODS:46 patients from 19 French centers were retrospectively recruited. They were included if they had a diagnosis of SSc and received at least 1 IVIG infusion at a dosage >1g/kg/cycle. Relevant data collected at IVIG discontinuation were compared to those collected at IVIG initiation. A comprehensive literature review was performed.RESULTS:We observed a significant improvement of muscle pain (74% vs. 20%, p

Introduction La gale est une infection a ectoparasites, causee par Scarcoptes scabiei variete hominis, principalement transmise par contact physique. La prevalence mondiale est estimee a 300 millions de cas par an [1] . La gale crouteuse norvegienne est une forme grave de l’infestation tres contagieuse, qui se manifeste surtout chez les immunodeprimes. L’atteinte cutanee se presente sous forme de lesions etendues, inflammatoires et hyperkeratosiques. Nous rapportons ici un cas de gale crouteuse norvegienne chez une patiente suivie pour un lupus erythemateux systemique, traite par belimumab. Observation Il s’agit d’une patiente âgee de 53 ans d’origine afro-antillaise, travaillant comme aide-soignante aupres d’enfants handicapes. Elle est suivie pour un lupus systemique depuis 20 ans avec des lesions cutanees lupiques chroniques, une polyarthrite non erosive, une atteinte renale de type glomerulonephrite classe V en 2014 puis classe IV en 2017, une lymphopenie chronique, des anticorps antinucleaires et anti-ADN positifs. Elle a recu differents traitements immunosuppresseurs : une corticotherapie au long cours, hydroxychloroquine (arrete en 2018 du fait d’une toxicite oculaire), cyclophosphamide avec relais par mycophenolate mofetil puis azathioprine. Elle recoit un traitement par ivermectine en tant que cas-contact pour la gale. Un mois apres, devant une poussee cutanee severe avec des lesions cutanees severes diffuses de lupus discoides et apres une hepatite a l’azathioprine, un traitement par belimumab est introduit. Trois mois apres, elle developpe un prurit associe aux lesions de lupus cutane, non modifiees. Un nouveau traitement par ivermectine est prescrit. Un mois plus tard, la patiente developpe un prurit generalise, a predominance nocturne, principalement localise sur les espaces interdigitaux des mains, les faces palmaires des poignets, le tronc et les plis sous-mammaires. Il n’y avait pas de symptomes similaires dans l’entourage familial et professionnel. Elle consulte un dermatologue qui prescrit des dermocorticoides et des antihistaminiques, sans efficacite. Nous constatons un aspect hyperkeratosique sans croutes jaunâtres et parfois excorie des lesions cutanees de lupus cutane chronique du decollete, du cuir chevelu, des oreilles et du dos. Aucun sillon scabieux n’a ete observe au dermatoscope. Un prelevement par grattage d’une lesion du dos hyperkeratosique a ete realise pour examen microscopique direct : il est mis en evidence a la fois des acariens et ses œufs. La patiente a ete traitee trois fois avec de l’ivermectine et de la creme a la permethrine, associe a des antihistaminiques. Des mesures de controle ont egalement ete mises en place dans le milieu familial et professionnel. Un mois apres le traitement local et general, on observe une nette amelioration cutanee avec disparition du prurit et de l’hyperkeratose. Discussion Quelques cas de gale crouteuse norvegienne au cours du lupus systemique ont ete rapportes dans la litterature [2] . La presentation peut etre atypique, se localiser sur des lesions cutanees lupiques preexistantes. Chez notre patiente, le prurit et l’apparition de l’hyperkeratose ont incite a rechercher la gale. Les antecedents de contact dans le contexte d’immunodepression (succession de traitements immunosuppresseurs, lymphopenie chronique) ont pu favoriser la survenue d’une gale severe. Dans le cas rapporte, malgre 2 cures d’ivermectine avant et pendant la biotherapie par belimumab, la patiente a developpe une forme norvegienne. Cette variante clinique peut etre associee a une morbidite importante (notamment liee a une septicemie secondaire). A l’heure actuelle, le belimumab n’est pas associe a un sur-risque d’infection opportuniste par rapport a l’incidence habituelle chez les patients atteints de lupus systemique, ni par rapport a la duree d’exposition [3] . D’autres cas ont ete signales avec d’autres biotherapies (anti-TNF, tocilizumab). Conclusion Il s’agit du premier cas de gale crouteuse norvegienne rapportee associee a l’utilisation de belimumab. Ce diagnostic peut etre difficile car la presentation clinique peut etre atypique et compliquer des lesions cutanees du lupus pre-existantes. Devant l’apparition d’un prurit ou d’un aspect hyperkeratosique, des prelevements sont necessaires afin de reconnaitre cette affection, dont le traitement general et local par ivermectine et permethrine est assez simple.

Cardiac involvement is the leading cause of death in systemic sclerosis (SSc), although the condition may remain clinically asymptomatic for a long time [(1)][1]. SSc remains underdiagnosed despite repeated transthoracic echocardiography (TTE). This prospective longitudinal study included all

Retroperitoneal fibrosis (RPF) is defined by the presence of a fibro-inflammatory tissue that surrounds the retroperitoneal structures, including abdominal aorta and its branches. Although RPF resu...

The use of autologous haematopoietic stem cell transplantation (AHSCT) in autoimmune disease (AD) patients has increased progressively worldwide. We retrospectively analysed the long-term outcome of AHSCT for AD reported to the French Society for Bone Marrow Transplantation and Cellular Therapy (SFGM-TC). All French AD patients (≥ 18 years at transplant) with a first AHSCT between 1997 and 2013 were included. Primary data were derived from the European Society for Blood and Marrow Transplantation (EBMT) registry, and additional data were obtained through a specific questionnaire designed for the study. Primary end-point was overall survival (OS). Secondary end points were progression-free survival (PFS) and non-relapse mortality (NRM). Ninety-four AD patients were included, of whom 71% suffered from rheumatologic diseases (n = 67, including 56 systemic sclerosis (SSc)), 16% from neurological disease (n = 15, including 14 multiple sclerosis (MS)) and 13% from various other AD (n = 12). After a median (interquartile range, IQR) follow-up of 83 months (38–130), OS at 5 and 10 years were 77% (95% CI 68.5–86.2) and 64% (95% CI 51.7–76.3), and for PFS 51% (95% CI 40.4–61.6) and 44% (95% CI 32.8–55.3), respectively. Overall, NRM was 8.7% (95% CI 4.0–15.5) at day 100, 9.8% (95% CI 4.8–16.9) at 5 years and 13.6% (95% CI 6.9–22.5) at 10 years. This first SFGM-TC retrospective report shows long-term benefit of AHSCT in AD patients with acceptable toxicity.

Older age is associated with increased mortality in immune thrombotic thrombocytopenic purpura (iTTP). Yet, data are scarce regarding iTTP occurring among older patients. To assess clinical features and long-term impact of iTTP on mortality in older patients (>60 years old), characteristics and prognoses of adult iTTP patients enrolled in the French Reference Center for Thrombotic Microangiopathies registry between 2000 and 2016 were described according to age (/=60 years old). Long-term mortality of iTTP older survivors was compared with that of non-iTTP geriatric subjects. Comparing, respectively, older iTTP patients (N = 71) with younger patients (N = 340), time from hospital admission to diagnosis was longer (P < .0001); at diagnosis, delirium (P = .034), behavior impairment (P = .045), renal involvement (P < .0001), and elevated troponin level (P = .025) were more important whereas cytopenias were less profound (platelet count, 22 x 103/mm3 [9-57] vs 13 x 103/mm3 [9-21], respectively [P = .002]; hemoglobin level, 9 g/dL [8-11] vs 8 g/dL [7-10], respectively [P = .0007]). Short- and mid-term mortalities were higher (P < .0001) and increased for every 10 years of age range. Age >/=60 years, cardiac involvement, increased plasma creatinine level, and total plasma exchange volume were independently associated with 1-month mortality. Compared with a non-iTTP geriatric population, older survivors showed an increased long-term mortality (hazard ratio = 3.44; P < .001). In conclusion, older iTTP patients have atypical neurological presentation delaying the diagnosis. Age negatively impacts short-term but also long-term mortality.

Introduction Les dermatomyosites a anticorps anti-MDA5 (DM MDA5+) ont des atteintes extramusculaires au premier plan (lesions cutanees severes, arthralgies/arthrite, fievre) pouvant engager le pronostic vital (atteinte pulmonaire), alors que l’atteinte musculaire est peu marquee. Leur pronostic est sombre (25 % de mortalite) et il est important de definir des facteurs de risque de mortalite pour mieux definir la strategie therapeutique. L’objectif de cette etude est d’identifier des facteurs pronostiques chez les DM MDA5+. Materiels et methodes Nous avons realise une etude nationale multicentrique retrospective grâce au Reseau francais des myosites pour les DM MDA5+. Les criteres d’inclusion etaient : – un diagnostic de DM (definie selon les criteres de l’ENMC en 2004 : un debut progressif subaigu, une faiblesse musculaire plutot proximale, une atteinte cutanee typique comme un erytheme lilace des paupieres, des papules de Gottron ou un erytheme du decollete et une atrophie perifasciculaire a la biopsie musculaire) ; – la positivite des anticorps anti-MDA5. Les recueils des donnees cliniques, paracliniques et evolutives ont ete realises a partir des dossiers des patients. Resultats Quarante et un patients ont ete identifies. L’âge moyen etait de 44 ans (± 16,5) avec un sex-ratio femme/homme de 1,4. Au diagnostic 92 % patients presentaient une atteinte pulmonaire et 87 % une atteinte cutanee. Un tiers (31 %) des patients ont presente une atteinte pulmonaire rapidement progressive (RPILD definie par une aggravation des lesions au scanner thoracique ou une aggravation des EFR pendant une duree inferieure ou egale a 3 mois). Les atteintes articulaires et musculaires (deficit moteur ou elevation des CK) etaient observees respectivement dans 75 % et 51 % des cas. La duree moyenne de suivi etait de 41,2 mois (± 61,3). La plupart des patients ont beneficie de corticosteroides (98 %) et d’immunosuppresseurs (83 %, dont 24 % du cyclophosphamide et 15 % des inhibiteurs des calcineurines) ou de rituximab (7 %), plus ou moins associes a des echanges plasmatiques (20 %) et/ou des immunoglobulines intraveineuses (15 %) selon leur severite. Le taux de mortalite etait de 22 %, avec des 44 % des deces survenus dans les 3 mois suivants le diagnostic. Les deces etaient dus a l’atteinte respiratoire dans 67 % des cas. Parmi les patients vivants, 52 % ont presente au moins une rechute, avec le plus frequemment une rechute pulmonaire (45 % des patients rechuteurs). L’analyse univariee identifie 3 variables liees au deces. Comme attendu les signes de gravite immediate respiratoire ont ete identifies avec une hypoxie severe (paO2 Conclusion Comme attendu, la severite initiale de l’atteinte pulmonaire (RPILD et hypoxie profonde) est un facteur pronostique pejoratif. Cette etude suggere pour la premiere fois que le sexe feminin et l’existence d’une atteinte articulaire seraient des elements de bon pronostic a prendre en compte dans la strategie therapeutique chez les patients sans signe de gravite pulmonaire immediate.

Introduction Le caplacizumab, un nanocorps anti-facteur Willebrand, s’est revele efficace dans le purpura thrombotique thrombocytopenique autoimmun (PTTi) dans deux essais cliniques. Il a beneficie d’une autorisation de mise sur le marche pour les patients atteints d’un PTTi au diagnostic. A ce jour, aucune donnee de vraie vie (hors protocole therapeutique) n’est disponible. L’objectif de ce travail est de d’evaluer l’efficacite et la tolerance du caplacizumab chez des patients atteints de PTTi non selectionnes. Materiels et methodes En France, le caplacizumab a beneficie d’une autorisation temporaire d’utilisation depuis septembre 2018. Nous avons traite selon un schema consensuel au plan national 90 patients avec un diagnostic clinique de PTTi en 15 mois a partir de 27 centres nationaux. Le traitement a consiste en une « triplette » associant des le diagnostic (en premiere ligne) des echanges plasmatiques (EP), une immunosuppression par corticoides et rituximab, et du caplacizumab. L’evolution des patients a ete comparee a celle de 180 patients historiques apparies en termes de severite et traites selon le schema historique, par EP et corticoides au diagnostic, et rituximab en cas de reponse defavorable au traitement standard. Le critere principal de jugement etait un critere composite associant la mortalite et la survenue d’une forme refractaire de PTT. Resultats Le pourcentage de patients dans le groupe « triplette » avec le critere de jugement principal composite etait de 2,2 % contre 12,2 % chez les patients historiques (p = 0,01). Par consequent, les patients traites avec le schema triplette etaient 6,2 fois moins susceptibles d’avoir un deces lie a un PTTi ou a un PTTi refractaire que les patients de la cohorte historique (IC a 95 %, 1,4 a 26,3, p = 0,013). Le taux de mortalite etait de facon inattendu extremement faible (6,7 % ; n = 12) dans la cohorte historique, ne permettant pas d’atteindre une difference siginificative sur la mortalite entre les 2 groupes. (p = 0,09). Pourtant un seul patient âge (83 ans) est decede dans le groupe triplette d’une embolie pulmonaire. Une diminution significative des exacerbations a ete notee dans la groupe triplette par rapport a la cohorte historique (3,4 % vs 44 %, p 0,25 μg/l) ou un score de mortalite eleve (atteinte cerebrale clinique, âge > 60 ans et LDH > 10 N) que chez ceux sans ces atteintes d’organe. Des evenements indesirables lies au caplacizumab sont survenus chez 46 patients (51 %), dont 13 evenements hemorragiques majeurs ou non majeurs mais cliniquement pertinents. Onze patients (12 %) ont eu un evenement thromboembolique veineux (une EP n = 5 ; une TVP n = 4 et une thrombose liee au catheter veineux central n = 4) sans difference entre les deux groupes (p = 0,79). Aucun d’entre eux n’avait recu de thromboprophylaxie meme lorsque la numeration plaquettaire etait superieure a 50,103/mm3. La duree totale de traitement par caplacizumab a ete de 33 jours consecutifs (29–37). Une activite ADAMTS13 detectable (activite ≥ 20 %) a ete le plus souvent observee aux semaines 2 ou 3 apres le dernier EP. Une analyse medico-economique reposant uniquement sur les couts lies a la prise en charge en aigue de la maladie montre un cout 3 fois plus eleve de la prise en charge par la triplette. Conclusion Dans cette premiere etude de « vraie vie », nous montrons qu’une « triplette », associant des EP, un traitement immunosuppresseur par corticoides et rituximab, et du caplacizumab en premiere ligne previent les evolutions defavorables de la maladie et diminue tres significativement la charge de soin. Le caplacizumab, par son action immediate, protege les patients jusqu’a l’amelioration de l’activite ADAMTS13 par le rituximab. En gommant le pronostic historiquement pejoratif des atteintes cardiaque et cerebrale, il ameliore pour la premiere fois en plus de 20 ans le devenir des patients.

The standard four-rituximab infusions treatment in acquired thrombotic thrombocytopenic purpura (TTP) remains empirical. Peripheral B cell depletion is correlated with the decrease in serum concentrations of anti-ADAMTS13 and associated with clinical response. To assess the efficacy of a rituximab regimen based on B cell depletion, 24 TTP patients were enrolled in this prospective multicentre single arm phase II study and then compared to patients from a previous study. Patients with a suboptimal response to a plasma exchange-based regimen received two infusions of rituximab 375 mg/m2 within 4 days, and a third dose at day +15 of the first infusion if peripheral B cells were still detectable. Primary endpoint was the assessment of the time required to platelet count recovery from the first plasma exchange. Three patients died after the first rituximab administration. In the remaining patients, the B cell-driven treatment hastened remission and ADAMTS13 activity recovery as a result of rapid anti-ADAMTS13 depletion in a similar manner to the standard four-rituximab infusions schedule. The 1-year relapse-free survival was also comparable between both groups. A rituximab regimen based on B cell depletion is feasible and provides comparable results than with the four-rituximab infusions schedule. This regimen could represent a new standard in TTP. This trial was registered at www.clinicaltrials.gov (NCT00907751). This article is protected by copyright. All rights reserved.

Objective To provide an epidemiologic description of Kikuchi-Fujimoto disease (KFD), and to describe its relationship with systemic lupus erythematosus (SLE) in a population of sub-Saharan origin. Methods Patients were retrospectively included on the basis of lymph node histology compatible with KFD reported in Martinique from 1991 until 2013. In order to describe the characteristics of the disease in a larger cohort, we subsequently included more patients of Afro-Caribbean origin from Guadeloupe and French Guiana. Results In Martinique, mean annual incidence between 1991 and 2013 was 2.78 cases for 1 million inhabitants (95% confidence interval 1.73-3.93). A total of 36 Afro-Caribbean patients from the 3 French American regions were included. Mean age was 30.5 years (range 5-59 years) and the female:male ratio was 3:1. The main characteristics were cervical adenopathies (88.8%), fever (83.3%), asthenia (73.0%), weight loss (64.4%), and recurrence in 33.3%. KFD was associated with lupus (n = 9 for SLE, n = 2 for cutaneous lupus) in 36.6% (11 of 30). Conclusion We report the first epidemiologic description of KFD in a population of sub-Saharan origin. According to our data, this disease is present in the black African diaspora and is strongly associated with autoimmune diseases, particularly lupus.

Objective To describe chronic chikungunya manifestations seen during the outbreak in the Caribbean from December 2013 to January 2015. Methods Patients were seen at our center, the only rheumatology department in Martinique Island, and were examined by a senior rheumatologist using a standard care report form. Chikungunya was diagnosed collectively based on consensus among all clinicians. The median time from onset of acute chikungunya to the first rheumatology consultation was calculated, severity was evaluated based on clinical scales and the degree of joint destruction, and each patient's treatment was recorded. Results For the 147 patients analyzed, the median time between onset of acute chikungunya and the first rheumatology consultation was 8 months. After review of each patient's medical record, 19 (12.9%) were diagnosed as having epidemic-influenced chikungunya. Four distinct rheumatologic patterns were observed in the remaining patients (those with compatible history and positive serologic findings): 47 patients (32%) had reactivation of painful chronic mechanical manifestations, 9 patients (6.1%) had fibromyalgia, 45 patients (30.6%) met criteria for spondyloarthritis (as evaluated before the chikungunya virus infection in all patients) and experienced a flare, and 27 patients (18.4%), with no history of joint disease, developed de novo bilateral symmetric chronic inflammatory joint disease in response to chikungunya virus infection. For inflammatory arthritis, most patients were treated with methotrexate (up to 25 mg/week), with good response and tolerance. Thirteen patients were treated with conventional doses of anti–tumor necrosis factor agents, with good tolerance and efficacy as expected. Conclusion The term “chronic chikungunya syndrome” covers multiple etiologies. Compliance with the French Society of Rheumatology recommendations, careful recording of patient histories, and serologic verification help prevent errors inherent to the epidemic context and ensure early therapeutic intervention for these patients. To avoid late initiation of treatment, patients should receive rheumatologic consultation as early as possible.

PURPOSE To describe the retinal manifestations in adult T-cell leukemia (ATL) related to an infection by the human T-cell lymphotropic virus type-1 (HTLV-1). METHODS Retrospective case series of patients with ATL with retinal findings. RESULTS A total of 175 patients were diagnosed with ATL in Martinique between 1983 and 2013. Three of them showed intraocular findings related to ATL. They were bilateral deep retinal infiltrates associated with intermediate uveitis. In two cases, the ATL diagnosis was known. In the third, fluorescein angiography was remarkable for deep retinal infiltrates although fundus examination was unremarkable. The ATL cells were found in the blood of this patient. Despite chemotherapy, infiltrates progressed from the retinal periphery to the posterior pole in two patients, thus reducing visual acuity to light perception. They were associated with vasculitis. CONCLUSION Retinal involvement in ATL is very rare. It can occur at any point during the natural course of the disease. Human T-cell lymphotropic virus type-1 carriers should benefit from a regular ophthalmic examination, and a fluorescein angiography must be performed in all patients with human T-cell lymphotropic virus type-1 with vitreous cells. The presence of deep retinal infiltrates must raise suspicion for ATL in a patient with human T-cell lymphotropic virus type-1.

Cardiac neonatal lupus syndrome is due to anti-SSA or SSB antibodies and mainly includes congenital heart block (CHB) and dilated cardiomyopathy (DCM). Its optimal management is still debated. We report a large series of autoimmune high degree CHB.Inclusion criteria in this retrospective study were fetuses or neonates with high-degree CHB associated with maternal anti-SSA/SSB antibodies.214 CHB were included: 202 detected in utero at a median term of 23 weeks' gestation (WG) [range 16 to 39 WG] and 12 neonatal cases diagnosed at a median age of 0 days [range birth to 8 days]. The 214 cases of CHB included 202 (94.4%) third-degree CHB, 8 (3.7%) second-degree CHB, and 4 (1.9%) intermittent CHB. In multivariate analysis, the factors associated with feto-neonatal deaths (15.7%) were hydrops (p0.001; hazard ratio [HR] 12.4 [95% confidence interval (95%CI) 4.7-32.7]) and prematurity (p=0.002; HR 17.1 [95%CI 2.8-103.1]). During a median follow-up of 7 years [birth to 36 years], 148 of 187 children born alive (79.1%) had a pacemaker, 35 (18.8%, one missing data) had DCM, and 22 (11.8%) died. In multivariate analysis, factors associated with child death were in utero DCM (p=0.0157; HR 6.37 [95%CI: 1.25-32.44]), postnatal DCM (p0.0001; HR 227.58[95%CI: 24.33-2128.46]) and pacemaker implantation (p=0.0035; HR 0.11[95%CI: 0.02-0.51]). The use of fluorinated steroids was neither associated with survival nor with regression of 2nd degree CHB.In this second largest series of CHB, we confirm some of the previous results. We were unable to find data supporting the routine use of in utero fluorinated steroids.

Objectives In this large nationwide French case-control study, we describe the features of TTP and ANCA-positive vasculitis; compare to vasculitis without associated TTP; describe the outcome and treatments. Methods We collected all cases with TTP and associated vasculitis. We conducted a literature review using PubMed, Web of Science, congress posters from January 2005 to August 2017 of PTT and ANCA-positive vasculitis. A control group of MPA without TTP during all the disease foillow-up was extracted from the Saint Antoine and Montfermeil Hospital patients with vasculitis. Firstly we compared our PTT cases and the literature review cases and secondary with a control group ANCA vasculitis without MAT. Results 8 patients with MAT secondary to ANCA associated vasculitides were included in our French series 75% of Women with a median age 45 years,21–76 positive ANCA in 50% and 37.5% is MPO, BVSA score at 16, FFS at 1. In 10 literature cases 90% Women, a median age 60 years [17–77], positive ANCA in 100% and 30% is MPO, BVSA score at 39, FFS at 1. The clinical features at the diagnosis of vasculitis were fever (n=5; 62%), ENT involvement (25%), kidney cresescnt glomerular involvment (n=6; 75%) with kidney failure in joint involvement (n=2; 25%) polyarthralgia type, with gastrointestinal involvement type mesenteric ischemia and pericarditis and lung involvement (nodules and alveolar haemorrhage) (n=1; 12.5%), in one case each Median C- reactive protein levels were at 49 mg/L [1–204], with creatininemia at 170 mg/dl [80–588]. ANCA were present in 4 patients (50%), MPO in 3 cases (37.5%). The time between the diagnosis of vasculitis and TTP was 9 months [0–51]. TTP features were: Hb at 7.8 g/dl [4.8–10], LDH 1658 [777–3110], platelets 33000 [3000–1 25 000], haptoglobin at 0 [0–0.05], creatinine at 205 [80–757]. ADAMTS 13 levels were at 10 [1–138], and x patients have normal ADAMTS13 levels, with anti ADAMTS 13 antibodies in 1 case. Plasma exchanges were done in all patients with median of 10 exchanges [4–23], 37.5% are dialyzed. Conclusions In comparison to our cases, the literature patients have similar organs involvement, but median creatininemia levels and BVAS levels were higher in the literature cases. Considering TTP features, our cases have less frequent active vasculitis, less important creatinine levels and thus less recurs to kidney dialysis. In vasculitis associated with TTP, there was no significant differences in organ involvements, BVAS and FFS scales values, laboratory data and ANCA levels. Only creatininemia as expected was higer in vasculitis associated with TTP (225 Mmol vs 150 Mmol, p=0.0044). Disclosure of Interest None declared

Preemptive rituximab infusions prevent relapses in immune thrombotic thrombocytopenic purpura (iTTP) by maintaining normal ADAMTS13 activity. However, the long-term outcome of these patients and the potential adverse events of this strategy need to be determined. We report the long-term outcome of 92 patients with iTTP in clinical remission who received preemptive rituximab after identification of severe ADAMTS13 deficiency (activity 1 iTTP episode, and the median cumulative relapse incidence before preemptive rituximab was 0.33 episode per year (interquartile range [IQR], 0.23-0.66). After preemptive rituximab, the median cumulative relapse incidence in the whole population decreased to 0 episodes per year (IQR, 0-1.32; P < .001). After preemptive rituximab, ADAMTS13 activity recovery was sustained in 34 patients (37%) during a follow-up of 31.5 months (IQR, 18-65), and severe ADAMTS13 deficiency recurred in 45 patients (49%) after the initial improvement. ADAMTS13 activity usually improved with additional courses of preemptive rituximab. In 13 patients (14%), ADAMTS13 activity remained undetectable after the first rituximab course, but retreatment was efficient in 6 of 10 cases. In total, 14 patients (15%) clinically relapsed, and 19 patients (20.7%) experienced benign adverse effects. Preemptive rituximab treatment was associated with a change in ADAMTS13 conformation in respondent patients. Finally, in the group of 23 historical patients with iTTP and persistently undetectable ADAMTS13 activity, 74% clinically relapsed after a 7-year follow-up (IQR, 5-11). In conclusion, persistently undetectable ADAMTS13 activity in iTTP during remission is associated with a higher relapse rate. Preemptive rituximab reduces clinical relapses by maintaining a detectable ADAMTS13 activity with an advantageous risk-benefit balance. ispartof: Blood vol:132 issue:20 pages:2143-2153 ispartof: location:United States status: published

International audience; Objective: To evaluate the performance of salivary gland ultrasonography for the diagnosis of primary and secondary Sjögren's syndromes (pSS and sSS).Method: Multicenter cross-sectional study on 97 patients with clinical sicca symptoms. The pSS (n = 39) and the sSS (n = 22) met the American-European Consensus Group (AECG) classification criteria. The control patients (n = 36) with sicca symptoms did not fulfill the AECG criteria. Four scores were used to evaluate the 4 major salivary gland echostructure: the Salaffi score (0-16), Jousse-Joulin score (0-4), Hocevar score (0-48) and Milic score (0-12).Results: The medians of ultrasonographic (US) scores were higher in the pSS and sSS groups than in the control group (p

Nonadherence to treatment is a major cause of lupus flares. Hydroxychloroquine (HCQ), a major medication in systemic lupus erythematosus, has a long half-life and can be quantified by high-performance liquid chromatography. This international study evaluated nonadherence in 305 lupus patients with flares using drug levels (HCQ200 ng/ml or undetectable desethylchloroquine), and self-administered questionnaires (MASRI80% or MMAS-86). Drug levels defined 18.4% of the patients as severely nonadherent. In multivariate analyses, younger age, nonuse of steroids, higher body mass index, and unemployment were associated with nonadherence by drug level. Questionnaires classified 39.9% of patients as nonadherent. Correlations between adherence measured by questionnaires, drug level, and physician assessment were moderate. Both methods probably measured two different patterns of nonadherence: self-administered questionnaires mostly captured relatively infrequently missed tablets, while drug levels identified severe nonadherence (i.e., interruption or erratic tablet intake). The frequency with which physicians miss nonadherence, together with underreporting by patients, suggests that therapeutic drug monitoring is useful in this setting. (Trial registration: ClinicalTrials.gov: NCT01509989.).

Introduction La dermatomyosite a anticorps anti-MDA5 (Melanoma differentiation-associated gene 5) (DM MDA5 + ) est une maladie systemique touchant la peau, les articulations et le poumon. L’atteinte pulmonaire peut etre rapidement progressive et fatale. L’anticorps anti-MDA5 cible un recepteur reconnaissant les ADN/ARN viraux. La frequence de l’atteinte respiratoire (> 80 % des cas) et la nature de la cible auto-antigenique suggerent un facteur declenchant viral de la maladie. Pour explorer cette question nous avons fait l’hypothese que le debut de la maladie etait rythme par les infections virales saisonnieres a tropisme respiratoire. Ce travail avait pour objectif de determiner si le debut des symptomes etait saisonnier. Materiels et methodes Nous avons realise une etude observationnelle, retrospective et multicentrique (n = 37) sur une cohorte francaise de patients atteinte de DM MDA5 + . Les donnees cliniques, biologiques et radiographiques ont ete etudiees, ainsi que la date d’apparition des premiers symptomes respiratoires (definis par le mois d’apparition des premiers symptomes reportes dans le premier compte rendu). Apres une analyse de la serie temporelle (stationnaire, autocorrelation) la modelisation des resultats (SARIMA) a pu etre appliquee. Les resultats ont ete compares a ceux de la declaration des cas de grippe recueillis par l’institut de veille sanitaire (INVS). Resultats Les patients inclus (n = 121) etaient majoritairement des femmes (diagnostic 2002–2017) d’âge moyen 49 ans. La majorite des patients presentaient des lesions cutanees (87,5 %), une pneumopathie interstitielle (77 %) et des arthralgies (69 %). Un tiers des patients etait febrile au diagnostic. L’atteinte pulmonaire etait rapidement progressive dans un tiers des cas. Un quart des patients sont decedes. La survenue des symptomes a ete classee par trimestre. Dans un premier temps, l’analyse de la repartition des premiers symptomes par trimestre montre qu’ils surviennent plus frequemment en periode hivernale (p = 0,05). Dans un second temps, la modelisation a permis de montrer que la repartition des premiers symptomes etait saisonniere (periodique) (p Conclusion Cette etude a permis de mettre en evidence que le debut des symptomes etait saisonnier avec un pic hivernal comparable a celui des cas declares de grippe. Ensemble, ces donnees suggerent indirectement l’implication d’un virus dans la physiopathologie de la maladie.

Background Cogan syndrome is mainly treated with steroids. We aimed to determine the place of DMARDs and biologic-targeted treatments. Patients and methods We conducted a French nationwide retrospective study of patients with Cogan syndrome (n = 40) and a literature review of cases (n = 22) and analyzed the efficacy of disease-modifying anti-rheumatic drugs (DMARDs) and tumor necrosis factor α (TNF-α) antagonists. Results We included 62 patients (31 females) (median age 37 years [range 2–76]. At diagnosis, 61 patients (98%) had vestibulo-auditory symptoms, particularly bilateral hearing loss in 41% and deafness in 31%. Ocular signs were present in 57 patients (92%), with interstitial keratitis in 31 (51%). The first-line treatment consisted of steroids alone (n = 43; 70%) or associated with other immunosuppressive drugs (n = 18; 30%). Overall, 13/43 (30%) and 4/18 (22%) patients with steroids alone and with associated immunosuppressive drugs, respectively (p = 0.8), showed vestibulo-auditory response; 32/39 (82%) and 15/19 (79%) ocular response; and 23/28 (82%) and 10/14 (71%) general response. Overall 61 patients had used a total of 126 lines of treatment, consisting of steroids alone (n = 51 lines), steroids with DMARDs (n = 65) and infliximab (n = 10). Vestibulo-auditory response was significantly more frequent with infliximab than DMARDs or steroids alone (80% vs 39% and 35%, respectively), whereas ocular, systemic and acute-phase reactant response rates were similar. Infliximab was the only significant predictor of vestibulo-auditory improvement (odds ratio 20.7 [95% confidence interval 1.65; 260], p = 0.019). Conclusion Infliximab could lead to vestibulo-auditory response in DMARDS and steroid-refractory Cogan syndrome, but prospective studies are necessary.

Resume Introduction Nous rapportons un cas de pyoderma gangrenosum (PG) associe a une aortite neutrophilique compliquee d’une dissection aortique ascendante, et suivi de l’apparition d’une polyarthrite rhumatoide (PR). Observation Une femme de 79 ans etait hospitalisee fin 2009 pour un PG vegetant. Un traitement par corticotherapie generale, puis colchicine et dermocorticoides, permettait une cicatrisation complete des lesions. Au cours de l’hospitalisation, la patiente presentait une dissection de l’aorte ascendante, traitee avec succes par une intervention chirurgicale en urgence. L’examen anatomopathologique de la piece operatoire trouvait une aortite neutrophilique diffuse. Les diagnostics de maladie de Takayasu et de lupus etaient ecartes. La tomodensitometrie (TDM) thoracique objectivait un syndrome interstitiel pulmonaire. Il existait une discrete lymphocytose dans le liquide bronchiolo-alveolaire, sans germe pathogene isole. Les lesions cutanees recidivaient trois, puis quatre ans plus tard, associees a l’apparition d’une PR. Une aggravation de la pneumopathie etait mise en evidence par TDM en 2013, avant d’observer une stabilisation en 2014. Le PG n’a plus recidive par la suite. Conclusion A notre connaissance, aucune autre observation d’aortite neutrophilique associee avec un PG ou une PR n’a ete publiee a ce jour. Tandis que dans la litterature emerge le concept de « systemicite » des dermatoses neutrophiliques, la presence d’une localisation cutanee et aortique de la pathologie neutrophilique chez une meme patiente conforte cette approche.