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Objectives Blau syndrome (BS), a rare autoinflammatory granulomatous disease, is a progressive disorder. Usually the maintenance dose of glucocorticoid may not be tapered below 15mg daily while immunosuppressives being used. There has been some experience with biologic agents in refractory BS patients. The objective of this study is to describe a case of BS benefit from Tocilizumab, a humanized monoclonal antibody against interleukin 6 receptor. Methods We report the first Chinese patient with BS who was resistant to currently available therapies, but had rapid quiescence after using Tocilizumab. We also conducted a systematic literature review about the current treatments of BS. Results A 13-year-old Chinese boy with BS, whose uveitis got worsened when treated with Infliximab, was well-controlled after the administration of Tocilizumab and prednisone was tapered off to a dose of 8mg/day. We identified 29 manuscripts providing 45 BS cases. Among these patients, 24 underwent biological treatments and 22 of them achieved remission. In the 29 manuscripts, the biological agents used to treat refractory BS included Etanercept, Infliximab, Adalimumab, Canakinumab and Anakinra. Conclusions Case reports of the use of biological agents have yielded mixed results. The diversity of the symptoms may be due to functional differences in NOD2 mutations. For BS patients with fever, lymphadenopathy and hepatosplenomegaly, Tocilizumab may be a better choice.

Objective Fas is a positive regulator of Th17 cells differentiation in experimental autoimmune encephalomyelitis (EAE). However, its upstream regulators are still not fully determined. This study was designed to explore the upstream regulators of Fas in regulating Th17 cells differentiation in EAE. Methods The mouse model of EAE was established by myelin oligodendrocyte glycoprotein injection. Th17 cells differentiation was induced by IL-23, IL-6 and TGF-β. Results Down-regulated Hsp70 and miR-374c and up-regulated Fas were observed in the spleen and brain of EAE mice. Hsp70 overexpression evidently reduced Fas protein level, but not mRNA level. The luciferase reporter assay indicated that miR-374c targets Fas. Overexpression of miR-374c down-regulated the mRNA and protein level of Fas. The concentration of IL-17A in CD4+ T-cells was reduced by miR-374c or Hsp70 overexpression, and Fas overexpression altered this trend. Hsp70 did not regulate the expression of miR-374c, and likewise, miR-374c did not regulate the expression of Hsp70. Further results suggested that Hsp70 and miR-374c regulated Fas expression through different ways to affect Th17 cells differentiation in EAE. Conclusions This study suggested that down-regulated miR-374c and Hsp70 promote Th17 cell differentiation by inducing Fas expression in EAE.

ORY-1001, an inhibitor of covalent lysine (K)-specific demethylase 1A (KDM1A), has been used as a therapy for the treatment of acute leukemia. However, the underlying mechanisms of anticancer are still not fully elucidated. Here, we report that KDM1A is highly expressed in lung cancers, where it appears to drive aggressive growth. Furthermore, lung cancer patients with higher KDM1A levels have worse survival outcomes than patients with lower KDM1A levels. Interestingly, ORY-1001significantly inhibited the cell proliferation, colony formation, cell cycle, and induced apoptosis, by regulating the Warburg effect through controlling Hexokinases 2 (HK2) expression. In summary, these results indicate that ORY-1001 could inhibit the growth of lung cancer cells via regulating the Warburg effect by controlling HK2.

Background IM is a group of disease characterised by chronic symmetrical myasthenia, muscle fatigue and monocyte infiltrating skeletal muscle. This disease usually associates with the muscle of trunk and proximal limbs, skin lesions companied or not. Dermatomyositis (DM) and polymyositis (PM) account for the major part of IM clinically. The diagnose of these diseases mainly rely on clinical manifestation, biopsy, ECG and serum creatase. Whole-body magnetic resonance imaging (WBMRI) has been reported succeeding in diagnosing PM/DM through short tau inversion recovery (STIR) sequence, but the ability to estimate disease activity hasn’t been reported. Objectives To evaluate the value in WBMRI through STIR sequence in evaluating the disease activity of polymyositis (PM) and dermatomyositis (DM) and screening interstitial lung disease (ILD) and osteonecrosis. Methods PM/DM patients meeting the Bohan and Peter diagnostic criteria from the First Affiliated Hospital of Zhengzhou University were admitted between 2015 August to 2017 December. A retrospective analysis of the WBMRI imaging data, serum creatine kinase, electromyography and muscle biopsy were performed. The WBMRI images were estimated by semiquantitative score and the disease activity was estimated by MDAAT. SPSS 20.0 was utilised to analyse the statistical significance. Results This study includes 61 patients, including 41 DM cases and 20 PM cases, all of these shows typical pathological feature and inflammatory muscle in WBMRI. Significant statistical correlation was found between the grade of muscle oedema estimated by WBMRI and the clinical assessment through MDAAT for muscle disease activity (DM r=0.57, p=0.006; PM r=0.84, p=0.001). The positive rate of serum creatine kinase test was 63% (39/61). Besides muscular changes, we also detected 35 cases in interstitial lung disease, 2 cases in osteonecrosis, 1 case in bone marrow oedema. Conclusions WBMRI is a sensitive, noninvasive method to evaluate the disease activity, which is better than serum creatine kinase. And it is of great value to screen the complication or concomitant diseases such as ILD and osteonecrosis. References [1] Elessawy SS, et al. Whole-body MRI for full assessment and characterization of diffuse inflammatory myopathy. Acta Radiol Open2016;5(9):2058460116668216.doi: 10.1111/j.1468–1331.2010.03012.x [2] Amato AA, Greenberg SA, Inflammatory myopathies. Continuum (Minneap Minn)2013;19(6 Muscle Disease): 1615–33. doi:10.1212/01.CON.0000440662.26427.bd [3] Tomasova SJ, et al. The role of MRI in the assessment of polymyositis and dermatomyositis. Rheumatology (Oxford)2007;46(7):1174–1179. doi:10.1093/rheumatology/kem088 [4] Andersson H, et al. Comparative analyses of muscle MRI and muscular function in anti-synthetase syndrome patients and matched controls: a cross-sectional study. Arthritis Res Ther2017;19(1):17. doi:10.1186/s13075-017-1219-y [5] Olsen NJ, Park JH. Inflammatory myopathies: issues in diagnosis and management. Arthritis Care Res1997;10(3):200–207. Disclosure of Interest None declared