ObjectiveTo understand the difference in characteristics between juvenile dermatomyositis (JDM) and adult dermatomyositis (ADM).Methods Sixty-one cases of JDM were retrospectively analyzed and compared with 30 cases of ADM. Results The rashes were presented as the initial symptom in all expect one JDM patients. Gottron’s papules were presented in 90% JDM patients and 67% ADM patients. Calcium deposition was presented in 7% JDM patients and none of the ADM patients. The cardiovascular system was involved in 7 % JDM patients and 23% ADM patients. Cancer occurred in none of JDM patients and 13% ADM patients. In JDM and ADM patients, the ratio of elevated muscle enzymes from highest to lowest was LDH, hy-droxybutyric acid enzyme, CK-MB, AST, and CK. The positive ratio of magnetic resonance (MRI) all exceeded 80% in JDM and ADM groups. Two cases died in each group.Conclusions The clinical presentation of JDM is basically the same as that of ADM. The most common initial symptoms in JDM are skin rashes and Gottron's papules. Cardiovascular disease and cancer are less in JDM than in ADM. MRI is valuable in the diagnosis of DM.

ObjectiveTo evaluate the clinical and radiological efficacy of TNFR Ⅱ -Fc combined with methotrexate( MTX ) in treatment of patients with moderate and severe rheumatoid arthritis.MethodsThree hundred and ninty-six RA patients were randomized into the combined treatment group,the TNFR Ⅱ -Fc only group and MTX only group.All patients were treated for 24 weeks.ACR-N,ACR20,ACR50,ACR70,DAS28-ESR and Sharp score of both hands were measured for efficacy,and the side-effects were analyzed by one-way ANOVA.Results After 24-week therapy,the ACR-N of the combined treatment group [( 12.79±9.24)％-year] was significantly improved than that of the TNFR Ⅱ-Fc only group [(9.56±11.16)％-year,P＜0.05] and that of the MTX only group[(5.08±11.10)％-year,P＜0.05],and the TNFR Ⅱ-Fc group was significantly improved than that of the MTX group(P＜0.05).The ACR20 response rate of the combined group(80.4％) was significantly higher than that of the TNFR Ⅱ -Fc group(71.1％,P＜0.05) and the MTX group(56.7％,P＜0.01 ).The ACRS0 response rate of the combined group(53.6％) was significantly higher than that of the MTX group(30.8％,P＜0.01 ).The ACR70 response rate of the combined group was 27.7％,which was significantly different from that of the TNFR Ⅱ -Fc group (15.8％) and MTX group (7.7％,P＜0.05or P＜0.01 ).DAS28-ESR in the combination group was significantly reduced than those of the TNFR Ⅱ -Fc group and MTX group,and the DAS28-ESR of the TNFR Ⅱ -Fc group was significantly reduced than MTX group.The average total Sharp score of both hands,which demonstrated the radiographic changes,was significantly reduced in the combination group than the MTX group(P=0.03).The total adverse events in the combined group(40.9％) was significantly high than that of the MTX group(28.8％,P＜0.05).Conclusion TNFR Ⅱ -Fc combined with MTX can effectively control the activity of RA and radiological progress.

Purpose: Fibroblast growth factor receptor 4 (FGFR4) plays a critical role in cancer progression involving in tumor proliferation, invasion, and metastasis. This study clarified the role of FGFR4-Arg388 variant in gastric cancer (GC), and more importantly highlighted the possibility of this single nucleotide polymorphism (SNP) as potential therapeutic targets. Materials and Methods: FGFR4 polymorphism was characterized in advanced GC patients to perform statistical analysis. FGFR4-dependent signal pathways involving cell proliferation, invasion, migration, and resistance to oxaliplatin (OXA) in accordance with the SNP were also assessed in transfected GC cell lines. Results: Among 102 GC patients, the FGFR4-Arg388 patients showed significantly higher tumor stage (p=0.047) and worse overall survival (p=0.033) than the Gly388 patients. Immunohistochemical results showed that FGFR4-Arg388 patients were more likely to have higher vimentin (p=0.025) and p-STAT3 (p=0.009) expression compared with FGFR4-Gly388 patients. In transfected GC cells, the overexpression of FGFR4-Arg388 variant increased proliferation and invasion of GC cells, increasing resistance of GC cells to OXA compared with cells overexpressing the Gly388 allele. Conclusion The exploration mechanism may be through FGFR4-Arg388/STAT3/epithelial to mesenchymal transition axis regulating pivotal oncogenic properties of GC cells. The FGFR4-Arg388 variant may be a biomarker and a candidate target for adjuvant treatment of GC.

Objective: To explore the feasibility of high-throughput texture analysis in the distinction of single brain metastases (SBM) from high-grade gliomas (HGG) and validate the established model. Methods: A total of 86 patients who were histologically diagnosed with SBM or HGG were retrospectively collected, including 43 patients with SBM and 43 with HGG. All of patients were performed preoperative conventional head magnetic resonance imaging (MRI) scans. A total of 236 fluid-attenuated inversion recovery (FLALR) images containing the information of tumors were selected from the MRI images and each image was considered as an object. The training set had 200 images, including 106 from SBM group and 94 from HGG group, whereas the validation set had 36 images, including 19 from SBM group and 17 from HGG. After images preprocessing, images segmentation, features extraction, and features selection, a radiomic diagnostic model was finally established using the training set. The diagnostic performance of the diagnostic model was evaluated using a receiver operating characteristic (ROC) curve. Hierarchical clustering analysis was used to evaluate the quality of the extracted feature data and the classification effect of the model. The model was further validated using the independent validation set. Results: A total of 629 features were extracted and quantified from each sample, and 41 features were selected to establish feature subsets and the diagnostic model. The classification decision function of the model is f(x)=sign and the kernel function of the model is K(x, x_i)=exp. In the training set, the diagnostic accuracy, sensitivity, specificity, positive predictive value and negative predictive value were 0.845, 0.849, 0.840, 0.857 and 0.832, respectively. The area under the ROC curve reached to 0.939. Similar results were obtained in the validation set. Conclusion The high-throughput texture analysis shows high accuracy in differentiating SBM from HGG.

BACKGROUND: Rheumatoid arthritis (RA), a chronic systemic autoimmune disease, is characterized by synovitis and progressive damage to the bone and cartilage of the joints, leading to disability and reduced quality of life. This study was a randomized clinical trial comparing the outcomes between withdrawal and dose reduction of tofacitinib in patients with RA who achieved sustained disease control. METHODS: The study was designed as a multicenter, open-label, randomized controlled trial. Eligible patients who were taking tofacitinib (5 mg twice daily) and had achieved sustained RA remission or low disease activity (disease activity score in 28 joints [DAS28] ≤3.2) for at least 3 months were enrolled at six centers in Shanghai, China. Patients were randomly assigned (1:1:1) to one of three treatment groups: continuation of tofacitinib (5 mg twice daily); reduction in tofacitinib dose (5 mg daily); and withdrawal of tofacitinib. Efficacy and safety were assessed up to 6 months. RESULTS: Overall, 122 eligible patients were enrolled, with 41 in the continuation group, 42 in the dose-reduction group, and 39 in the withdrawal group. After 6 months, the percentage of patients with a DAS28-erythrocyte sedimentation rate (ESR) of <3.2 was significantly lower in the withdrawal group than that in the reduction and continuation groups (20.5%, 64.3%, and 95.1%, respectively; P  < 0.0001 for both comparisons). The average flare-free time was 5.8 months for the continuation group, 4.7 months for the dose reduction group, and 2.4 months for the withdrawal group. CONCLUSION: Withdrawal of tofacitinib in patients with RA with stable disease control resulted in a rapid and significant loss of efficacy, while standard or reduced doses of tofacitinib maintained a favorable state. TRIAL REGISTRATION Chictr.org, ChiCTR2000039799.
Humans ; Quality of Life ; China ; Arthritis, Rheumatoid/drug therapy* ; Piperidines/therapeutic use* ; Treatment Outcome ; Antirheumatic Agents/therapeutic use* ; Pyrroles/therapeutic use*

ATP-Binding Cassette Transporters ; genetics ; DNA Copy Number Variations ; genetics ; Genetic Predisposition to Disease ; Gout ; genetics ; Humans ; Receptors, IgG ; genetics ; Receptors, Interleukin-17 ; genetics