Maladie de Still de l'adulte (manifestations, traitements, évolution et facteurs pronostiques chez 57 patients)

Le but de cette étude observationnelle rétrospective est de décrire une cohorte française de maladies de Still de l'adulte (MSA) et d'en identifier les facteurs pronostiques. Les patients devaient remplir les critères de classification de Yamaguchi ou Fautrel à l'inclusion. Les variables candidates ont été analysées en régression multivariée non ajusté puis ajusté sur les variables potentiellement confondantes. Cinquante-sept patients sont suivis en moyenne 8,4 ans en médecine interne (75%) ou rhumatologie (25%). Le délai diagnostic médian est de 4 mois. L'évolution est monocyclique chez 17 patients, intermittente chez 25 et chronique chez 15. Le dosage de la ferritine glycosylée (FG) réalisé chez 37 patients est corrélé à un diagnostic plus précoce. Neuf 18FDG-PET scanners montrent un hypermétabolisme des tissus lymphatiques et glandulaires. Les complications, dont le syndrome d'activation lymphohistiocytaire (n=8), sont fréquentes (n=19). Aucun des trois décès répertoriés n'est attribuable à la MSA. La corticodépendance, prédite par une FG basse, concerne 23 patients (45%). Un quart des patients a reçu des anti-TNFa ou de l'anakinra, bien tolérés. La fièvre > 39,5C est prédictive d'une évolution monocyclique de la MSA alors que les arthrites et la thrombopénie sont respectivement associées à une évolution chronique et compliquée de la maladie. Les patients les plus jeunes sont les plus à risque de résistance aux premières lignes de traitement. La MSA reste de diagnostic difficile, sa mortalité est faible malgré des complications fréquentes. La FG et le 18FDG-PET scanner sont utiles pour le diagnostic. Les patients initialement très symptomatiques évolueront vers une forme systémique de MSA alors que ceux présentant seulement des arthrites évolueront vers une forme articulaire chronique.LYON1-BU Santé (693882101) / SudocSudocFranceF

Still’s Disease Mortality Trends in France, 1979–2016: A Multiple-Cause-of-Death Study

Still’s disease (SD) is often considered a benign disease, with low mortality rates. However, few studies have investigated SD mortality and its causes and most of these have been single-center cohort studies. We sought to examine mortality rates and causes of death among French decedents with SD. We performed a multiple-cause-of-death analysis on data collected between 1979 and 2016 by the French Epidemiological Center for the Medical Causes of Death. SD-related mortality rates were calculated and compared with the general population (observed/expected ratios, O/E). A total of 289 death certificates mentioned SD as the underlying cause of death (UCD) (n = 154) or as a non-underlying causes of death (NUCD) (n = 135). Over the study period, the mean age at death was 55.3 years (vs. 75.5 years in the general population), with differences depending on the period analyzed. The age-standardized mortality rate was 0.13/million person-years and was not different between men and women. When SD was the UCD, the most frequent associated causes were cardiovascular diseases (n = 29, 18.8%), infections (n = 25, 16.2%), and blood disorders (n = 11, 7.1%), including six cases (54%) with macrophage activation syndrome. As compared to the general population, SD decedents aged <45 years were more likely to die from a cardiovascular event (O/E = 3.41, p < 0.01); decedents at all ages were more likely to die from infection (O/E = 7.96–13.02, p < 0.001)

Adult-onset Still's disease

International audienceFirst described in 1971, adult-onset Still's disease (AOSD) is a rare multisystemic disorder considered as a complex (multigenic) autoinflammatory syndrome. A genetic background would confer susceptibility to the development of autoinflammatory reactions to environmental triggers. Macrophage and neutrophil activation is a hallmark of AOSD which can lead to a reactive hemophagocytic lymphohistiocytosis. As in the latter disease, the cytotoxic function of natural killer cells is decreased in patients with active AOSD. IL-18 and IL-1β, two proinflammatory cytokines processed through the inflammasome machinery, are key factors in the pathogenesis of AOSD; they cause IL-6 and Th1 cytokine secretion as well as NK cell dysregulation leading to macrophage activation. The clinico-biological picture of AOSD usually includes high spiking fever with joint symptoms, evanescent skin rash, sore throat, striking neutrophilic leukocytosis, hyperferritinemia with collapsed glycosylated ferritin (\textless20%), and abnormal liver function tests. According to the clinical presentation of the disease at diagnosis, two AOSD phenotypes may be distinguished: i) a highly symptomatic, systemic and feverish one, which would evolve into a systemic (mono- or polycyclic) pattern; ii) a more indolent one with arthritis in the foreground and poor systemic symptomatology, which would evolve into a chronic articular pattern. Steroid- and methotrexate-refractory AOSD cases benefit now from recent insights into autoinflammatory disorders: anakinra seems to be an efficient, well tolerated, steroid-sparing treatment in systemic patterns; tocilizumab seems efficient in AOSD with active arthritis and systemic symptoms while TNFα-blockers could be interesting in chronic polyarticular refractory AOSD

Mortality associated with Behçet’s disease in France assessed by multiple-cause-of-death analysis

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Pathogenesis of adult-onset Still's disease: new insights from the juvenile counterpart

Adult-onset Still's disease (AOSD) is a rare inflammatory disease characterized by the classical triad of daily fever, arthritis, and typical salmon-colored rash. Recent accumulation of knowledge, mostly arising from hereditary autoinflammatory diseases and from the systemic-onset juvenile idiopathic arthritis (sJIA), has given raise to new hypotheses on the pathophysiology of AOSD. In this review, we first discuss on the continuum between AOSD and sJIA. Then, we summarize current hypotheses on the underlying pathogenesis: (1) an infectious hypothesis; (2) an autoinflammatory hypothesis; (3) a lymphohistiocytic hypothesis; and (4) a hyperferritinemic hypothesis. Finally, we present the recent data suggesting that patients with AOSD fall into two distinct subgroups with different courses, one with prominent systemic features and one with chronic arthritis

Management of Non-Infectious Uveitis, a Selection of Topical Items Updating

First of all, we would like to thank all of the authors for their contributions and the editorial staff who enabled the achievement of this «Diagnosis and Management of Non-infectious Uveitis: Old and New Challenges» Special Issue [...

Uveitis as an Open Window to Systemic Inflammatory Diseases

Spondyloarthritis (Spa), Behçet’s disease (BD) and sarcoidosis are major systemic inflammatory diseases worldwide. They are all multisystem pathologies and share a possible ocular involvement, especially uveitis. We hereby describe selected cases who were referred by ophthalmologists to our internal medicine department for unexplained uveitis. Physical examination and/or the use of laboratory and imaging investigations allowed to make a diagnosis of a systemic inflammatory disease in a large proportion of patients. In our tertiary referral center, 75 patients have been diagnosed with Spa (n = 20), BD (n = 9), or sarcoidosis (n = 46) in the last two years. There was a significant delay in the diagnosis of Spa-associated uveitis. Screening strategies using Human Leukocyte Antigen (HLA)-B27 determination and sacroiliac magnetic resonance imaging in patients suffering from chronic low back pain and/or psoriasis helped in the diagnosis. BD’s uveitis affects young people from both sexes and all origins and usually presents with panuveitis and retinal vasculitis. The high proportion of sarcoidosis in our population is explained by the use of chest computed tomography (CT) and 18F-fluorodeoxyglucose positron emission tomography CT that helped to identify smaller hilar or mediastinal involvement and allowed to further investigate those patients, especially in the elderly. Our results confirm how in these sight- and potentially life-threatening diseases a prompt diagnosis is mandatory and benefits from a multidisciplinary approach

Occupational hypersensitivity pneumonitis in a baker: a new cause.

International audienceBakers are exposed daily to flour and may be susceptible to immunologic occupational diseases. A 30-year-old, nonsmoking, female baker was referred for progressive dyspnea on exertion, basal crackles on auscultation, restrictive lung function, decreased diffusing capacity of the lung for carbon monoxide, ground glass hyperdensities with a mosaic pattern on high-resolution CT scan, 25% lymphocytosis by BAL, and cellular chronic bronchiolitis with peribronchiolar interstitial inflammation by lung biopsy specimen. Cultures from flours isolated nine species, including Aspergillus fumigatus. Twenty-six antigens were tested. Serum-specific precipitins were found against A fumigatus, the flour mite Acarus siro, and total extracts from maize and oat. Outcome was favorable with cessation of occupational exposure to flours and transient therapy with prednisone and immunosuppressive agents. To our knowledge, this report is the first of a well-documented case of hypersensitivity pneumonitis due to sensitization to fungi- and mite-contaminated flours. Hypersensitivity pneumonitis--and not only asthma and allergic rhinitis--should be suspected in bakers with respiratory symptoms

Evaluation of Glycosylated Ferritin in Adult-Onset Still’s Disease and Differential Diagnoses

Glycosylated ferritin (GF) has been reported as a good diagnostic biomarker for adult-onset Still’s disease (AOSD), but only a few studies have validated its performance. We performed a retrospective study of all adult patients with at least one GF measurement over a 2-year period in one hospital laboratory. The diagnosis of AOSD was based on the expert opinion of the treating physician and validated by two independent investigators. Patients’ characteristics, disease activity, and outcome were recorded and compared. Twenty-eight AOSD and 203 controls were identified. Compared to controls, the mean GF was significantly lower (22.3% vs. 39.3, p < 0.001) in AOSD patients. GF had a high diagnostic accuracy for AOSD, independent of disease activity or total serum ferritin (AUC: 0.674 to 0.915). The GF optimal cut-off value for AOSD diagnosis was 16%, yielding a specificity of 89% and a sensitivity of 63%. We propose a modified diagnostic score for AOSD, based on Fautrel’s criteria but with a GF threshold of 16% that provides greater specificity and increases the positive predictive value by nearly 5 points. GF is useful for ruling out differential diagnoses and as an appropriate classification criterion for use in AOSD clinical trials