Clinical characteristics and risk factors of juvenile dermatomyositis with relapses / 中华风湿病学杂志

Objective:To analyze the clinical characteristics and risk factors of juvenile dermatomyositis (JDM) with relapses by comparing clinical features, treatment and disease course among JDM patients with and without relapses.Methods:A retrospective analysis of 102 JDM patients from Children's Hospital of Nanjing Medical University between March 2017 and March 2021 was carried out. Patients were divided into two groups based on whether a JDM relapse had occurred or not. Initial clinical features, laboratory tests and treatment were compared between the two groups. T-test or Mann-Whitney U test was used for measurement data, chi-square test or fisher exact probability was used for count data. The features associated with risk of relapses were analyzed by multivariate logistic regression. Results:Among 102 children with JDM, twenty patients (19.6%) relapsed during drug reduction or after drug withdrawal. The mean duration to the first relapse was 3.24 years (range: 9 months to 7 years). Myositis specific antibodies (MSA) were positive for 8 (40.0%) patients with relapses. With 5 cases were anti-nuclear matrix protein 2 positive, 2 cases were anti-transcription interme-diary factor 1 gamma positive, 1 case was anti-signal recognition particle (SRP) positive, the other 12 cases were MSA negative. By binary logistic regression analysis, we found that peripheral calcinosis [ OR(95% CI)=17.54(1.55, 198.64), P=0.021], and interstitial lung disease [ OR(95% CI)=3.83(1.27, 11.59), P=0.017] were independently related to JDM with relapses. Fifty-three patients (51.9%) received methylpre-dnisolone pulse therapy for initial treatment and 13 (65.0%) patients with relapses received methylprednisolone pulse for initial treatment. There was no significant difference between the two groups ( χ2=1.70 , P=0.193). Tumor necrosis factor alpha antagonist combined with methotrexate (MTX) had achieved good results in clinical treatment in children with relapses. Conclusion:The risk of relapses is high in children with JDM. Calcinosis and interstitial lung disease at disease onset can predict a relapsing disease course. Aggressive treatment is urgently demanded for patients with JDM, especially those with relapses.

CD4 + T cell differentiation in rheumatoid arthritis / 国际儿科学杂志

The dysfunction of T cells, especially the interaction of antigen presenting cells and CD4 + T cells, leads to abnormal activation of CD4 + T cells and the initiation of adaptive immune response.Dysfunction of the immune system plays an important role in the pathogenesis of rheumatoid arthritis.It is always considered as the central link of synovial continuous inflammation, articular cartilage and bone destruction in rheumatoid arthritis patients.Exploring CD4 + T cell differentiation and cell subsets provides a new perspective for understanding of arthritis and helps to identify new drug targets.

Successful treatment of refractory systemic'onset juvenile idiopathic arthritis with tocilizumab: a retrospective analysis of 25 cases / 中华风湿病学杂志

Objective To investigate the efficacy and safety of tocilizumab inpatients with refractory systemic'onset juvenile idiopathic arthritis (SoJIA),and to provide a new option for the treatment of this severe disease.Methods We retrospectively studied 25 cases of hospitalized patients with refractory SoJIA treated withtocilizumab,of whom 22 had data that fit for analysis,from May 2005 to February 2016.Data of 22 cases were collected retrospectively from physicians in charge of the patients.Children with SoJIA were treated with nonsteroidal antiinflammatory drugs (NSAIDs),Glucocorticoid (GC),methotrexate,cyclosporin A,etanerceptetc before,but still in high disease activity due to inadequate response were involved.Weretrospective analyzedthe laboratory test results like C'reactive protein (CRP),Erythrocyte sedimentation rate (ESR),Ferritin and other inflammatory index.Improvement of pain,fever,rash,hepatosplenomegaly and lymphadenectasis of active SoJIA (disease course ≥6 months,and inadequate response to NSAIDs and GC) after tocilizumab treatment (Body weight ≥30 kg,8 mg/kg;Body weight＜30 kg,12 mg/kg,per 4 weeks) were analyzed.Safety data of 22 cases were collected throughout the treatment period including neutropenia,infections,anaphylaxis and elevated liver enzymes etc.We also retrospectively analyzedthe dose change of GC and the long'term effect.Dichtomous paramenters were compared teween groups using thex2 test.Continuous parameters were compared using the analysis of uariance.Results In comparison to the indices before the treatment,the level of CRP [(8.7±2.2) mg/L vs (111.6±74.4) mg/L,F=5.192,P=0.002],ESR [(6.4±6.3) mm/1 h) vs (65.6±24.3) mm/1 h,F=50.393,P=0.000],white blood cell (WBC) [(8.4±2.5)×109/L vs (17.6±8.6)×109/L,F=9.321,P=0.000],Neutrophil count [(4.9±2.4)×109/L vs.(14.4±8.7)×109/L,F=10.541,P=0.000],blood platelet (PLT) [(269.5±79.2)×109/L vs (405.4± 145.3)×109/L,F=5.704,P=0.000] and globulin [(19.2±4.1) g/L vs (30.1±3.8) g/L,F=22.896,P=0.000] decreased rapidly and hemoglobin [(118.3±9.0) g/L vs (108.5±9.8) g/L,F=4.693,P=0.002] increased significantly at 24 weeks after Tocilizumab (TCZ) treatment.Clinical manifestationssuch as fever,rash,hepatosplenomegaly,joint swelling and pain were significantly improved.GC dose [(1.25±3.8) mg·kg-1·d-1 vs (16.2±12.8) mg·kg-1·d-1,F=8.21,P=0.000] were significantly reduced after TCZ treatment (P＜0.05);American College of Rheumatology (ACR) Pedi 30/50/70/90 was improved after TCZ treatment.Adverse events occurred in 3 cases of 25 children,who were not included in the statistical analysis group.Conclusion This retrospective case series has demonstrated the efficacy of tocilizumab in SoJIA,low incidence of adverse reactions.Further studies are needed to be developed because this case series haslimited sample size.

Clinical features and long - term follow - up of neonatal lupus erythematosus / 中华实用儿科临床杂志

Objective To investigate the clinical features and long - term prognosis of neonatal lupus erythe-matosus(NLE)and to improve the understanding of NLE. Methods The clinical manifestations and related serologi-cal tests of NLE children diagnosed from June 2010 to January 2014 were analyzed. Regular follow - up was carried out to detect the general condition,rash,blood routine,urine routine,liver and kidney function,complement,red blood cell sedimentation rate(ESR),auto antibodies,electrocardiogram,and ultrasound cardiogram. Results Among the 11 NLE cases,there were 6 male and 5 female patients. All had lesions on skin,3 cases had hematologic changes,7 cases were had liver damage,and 4 cases had heart impairment. The antinuclear antibody and anti - sjogren sydrome A/ Ro antigen (SSA/ Ro)were positive in all the patients. The anti - sjogren sydrome B antigen was positive in 5 patients. The anti -double stands deoxyribonucleic acid antibody was positive in 4 patients. Antibody against U1 - ribonudeoprotein was positive in 3 patients,and the level of ESR was higher in 5 patients. The antinuclear antibody and anti - SSA/ Ro anti-body were positive in all mothers. Only 1 mother had no symptom before pregnancy,7 patients had SLE,3 patients had sjogren syndrome. Seven patients received protect liver enzyme treatment,3 cases of glucocorticoid therapy,and 1 case had combined intravenous treatment with gamma globulin. Among the 11 cases,10 cases were followed up for 10 months to 4 years,while 1 case died from complete bundle branch block after 5 weeks of birth. At 1 year old,10 cases of cuta-neous lupus damage had liver damage were resorted to normal,and the rheumatic autoimmune related autoantibodies of 9 cases turned to be negative,but 1 case was diagnosed as Kawasaki disease when she was 1 year old. Conclusions One of the most common clinical manifestations of NLE was damage of skin,had the liver and blood system abnormity were common but usually not serious. Heart disease especially complete atrioventricular block was less. The long - term follow - up for children with NLE is necessary,and the majority of the prognosis is good,as only a few have the possibi-lity of developing other autoimmune diseases.

THE ACROMIOCLAVICULAR DISLOCATION AND THE FRACTURE OF THE DISTAL END OF CLAVICLE TREATED WITH CLAVICLE-HOOK PLATE / 现代医院

Objective To explore into the superiority of the treatment of clavicle-hook plate for acromioclavicular dislocation and the fracture of the distal end of clavicle.Methods Select 35 clinical cases with the acromioclavicular dislocation and the fracture of the distal end of clavicle, and then evaluate the function of the joint and the union of fracture.Results All the fractures got fully union, no joint re-dislocation, and got 100% excellent and good result.Conclusion Clavicle-hook plate is the perfect fixation in the treatment of the acromioclavicular dislocation and the fracture of the distal end of clavicle.