Treatment of adult-onset Still's disease: a review

Adult-onset Still's disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. The former classification, based on the disease course, seems to be quite dated. Indeed, there is accumulating evidence that AOSD can be divided into two distinct phenotypes based on cytokine profile, clinical presentation, and outcome, ie, a "systemic" pattern and an "articular" pattern. The first part of this review deals with the treatments that are currently available for AOSD. We then present the different strategies based on the characteristics of the disease according to clinical presentation. To do so, we focus on the two subsets of the disease. Finally, we discuss the management of life-threatening complications of AOSD, along with the therapeutic options during pregnancy

Single-arm, open-label pilot intervention study to investigate an effect of oral 5-aminolevulinic acid plus sodium ferrous citrate on glucocorticoid reduction in patients with adult-onset Still disease

BACKGROUND: Glucocorticoids are an important class of medication for patients with adult-onset Still disease (AOSD), however, relapse following glucocorticoid reduction and adverse events due to long-term effects of glucocorticoid are still problematic. It is of course essential to minimize the risk of treatment. Immunosuppressive therapies such as methotrexate and biologics including tocilizumab are used in glucocorticoid-dependent patients with AOSD, but no second-line treatments for patients with glucocorticoid dependence have been established yet. Given that these drugs also have the potential to cause adverse events, alternative treatments are sought. Recently, elevated heme oxygenase-1 (HO-1) has been reported in the serum of patients with AOSD, suggesting that HO-1 activity contributes to AOSD pathogenesis and may represent a new therapeutic target for the treatment of AOSD. The amino acid 5-aminolevulinic acid (5-ALA) is a non-proteinogenic δ amino acid in human body. An addition of ferrous iron to 5-ALA enhances heme biosynthesis. The increase in heme in vivo induces HO-1 production, a heme-degrading enzyme. Elevated HO-1 has been suggested to contribute to the pathogenesis of AOSD, and administration of 5-ALA and ferrous iron may be a potential treatment for AOSD. METHODS/DESIGN: This study is a single-arm, open-label pilot intervention study using clinical endpoints to investigate the effects of oral 5-ALA with sodium ferrous citrate on glucocorticoid reduction in patients with AOSD receiving glucocorticoid therapy. DISCUSSION: This pilot intervention study will provide evidence regarding the effectiveness and safety of 5-ALA/sodium ferrous citrate as a potential new therapeutic agent for glucocorticoid-dependent patients with AOSD. TRIAL REGISTRATION: This study was registered in the Japan Registry of Clinical Trials (https://jrct.niph.go.jp) on January 14, 2020 as jRCTs071190042

Hypersensitivity pneumonitis: antigen diversity and disease implications

Article in PressHypersensitivity pneumonitis (HP) is an immune-mediated syndrome triggered by inhalation of a wide variety of allergens, to which an individual has previously been sensitized. More than 200 agents responsible for the disease have already been identified; however, HP occurs only in a small number of individuals exposed to causal antigens. The present report provides an overview of the role of antigen role in HP, highlighting its diversity, research methods, and prevention strategies, as well as the impact on disease prognosis following elimination of antigen. HP is an underdiagnosed disease and, therefore, it is difficult to accurately estimate its incidence. Triggering antigens can be divided into six broad categories: bacteria, fungi, mycobacteria, animal and plant proteins, chemicals, and metals, represented by disease prototypes. The identification of causal antigen is a major challenge; it is impossible to obtain in about 30-60% of cases. The acute form of HP, with early detection and immediate eviction of causal antigen, tends to have an excellent prognosis. In the chronic form, partial recovery of disease is still possible; however, some cases tend to progress to fibrosis, even after removal from exposure. In conclusion, HP diagnosis should be based on a proactive search for potential antigen sources, although their identification is hampered by the lack of standardized methods of demonstrating the specific antigen sensitization. Antigen avoidance is a critical determinant in disease prognosis.(undefined)info:eu-repo/semantics/acceptedVersio

Genetic Basis of Myocarditis: Myth or Reality?

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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