Objective To explore the diagnosis and treatment of five children with tuberculosis with arthritis as the initial manifestation. Methods The clinical features, laboratory tests and imaging manifestation of 5 children with joint tuberculosis were retrospectively analyzed. Results The course of disease was different. All the five patients were males (mean age 8.5 ±2.9 years old) and suffered from articular symptoms as initial feature. Four of them were diagnosed and treated as rheumatoid arthritis by other hospitals for up to three years, two patients have tuberculosis contact history, and another two patients were found with bone destruction, and one patient has pathologic fracture. Conclusions Tuberculosis is easily misdiagnosed as juvenile idiopathic arthritis , which deserves attention from a pediatric rheumatology physician.

Objective To analyze the clinical features and laboratory data of 10 patients with macrophage activation syndrome (MAS) complicating systemic onset juvenile idiopathic arthritis (soJIA),which were characterized by acute severe liver injury.Methods Data of 10 patients with soJIA/MAS from Nanjing Children's Hospital were collected retrospectively.The clinical features,laboratory findings,treatment,outcomes and prognosis were analyzed.Results In the total 10 patients,female (6/10) outnumbered male.Their age ranged from 1.5 to 9.5 years old (average 5.2±2.6).The most remarkable clinical manifestations were severe liver injury without systemic features,representing as hepatomegaly (10/10),splenomegaly (2/10) and strikingly increased transaminase (10/10,median:ALT 1 445 U/L,AST 885 U/L).Central nervous system dysfunction and hemorrhages were recorded in 20％ of the patients.Two patients had pulmonary infection.Laboratory data showed that platelet count was less than normal or precaution value (10/10,≤262×10g/L).Hyperferritinaemia (10/10,median:17 329 mg/ml) and soluble CD25 elevation (median:3 140 U/ml) were common in the soJIA/MAS patients.Evidence of macrophage hemophagocytosis was found in 90％ of the patients (9/10) who underwent bone marrow aspiration.Pathological findings of liver biopsy from 1 patient revealed massive infiltration of mononuclear cells in the portal tracts.Nearly all patients (9/10) received intravenous pulse methylprednisolone therapy,combined with cyclosporine A and high-dose intravenous immunoglobulin.Eight patients had good outcome.Only 2 patients were complicated with severe interstitial lung disease during 12-months follow-up.Conclusion MAS should be considered when patients with soJIA represents acute severeliver injury without systemic features combined with other laboratory data.Intravenous pulse methylprednisolone and cyclosporine A therapy may improve the prognosis of soJIA/MAS.

Objective:To investigate the different effects of different treatment regimens in resistant Kawasaki disease (KD) and to provide evidence for clinical treatment.Methods:Forty-nine inpatient children with resistant KD from July 2017 to June 2019 in Children's Hospital of Nanjing Medical University were enrolled into this study. Treatment and follow-up were still in progress. Rank sum test and χ2/Fisher test were used for statisic. Results:The incidence of resistance in infliximab group was significantly lower than that of intravenous immunoglobulin (IVIG) retreated group ( P<0.05). Sixteen cases were treated with 5 mg/kg infliximab (IFX), and 33 cases received methylprednisolone and an additional dose of IVIG. Nine cases who were resistant to IVIG and methylprednisolone were treated with IFX, 6 patients responded to IFX, 3 of them were treated with cyclosporine. Coronary artery changes were followed up. Coronary artery lesions (CALs) were improved in the IFX group, CALs occurred in 12(36%) patients received IVIG and methylprednisolone, 4 of them were improved( χ2=0.633 , P=0.426). Patients were followed up for 3-24 months, the incidence of CALs persistence was statistically significantly different between the two groups (0 vs 24%, P=0.021]. Conclusion:IFX might be an effective and tolerable treatment for resistant KD.

Enthesitis related arthritis（ERA）is one of the subtypes of juvenile idiopathic arthritis（JIA）. It is a group of diseases characterized by arthritis，inflammation，axial lesions，and human leucocyte antigen-B27 positive. In the previous classification criteria，ERA does not include all of the subsets of juvenile spondyloarthritis（JSpA）. Based on an evidence-based approach，the Pediatric Rheumatology International trials Organization developed the latest classification criteria in 2019，and proposed the classification criteria of ERA/SpA，to distinguish it from other subtypes of JIA. The new definition added the term spondylitis，included imaging criteria，and adopted a new definition of back pain. The pathogenesis，evolution of classification criteria and prognosis of spondyloarthritis are described in this paper，in order to improve the level of diagnosis and treatment of patients with axial joint involvement，so as to improve the prognosis.

Objective: To evaluate the clinical effect of pereutaneous vertbroplasty (PVP) combined with implantation of iodine-125 (125 I)radioactive particle in the treatment of vertebral metastasis,and to provide basis for the treatment of vertebral metastasis.Methods:A total of 69 patients with vertebral metastasis were divided into test group (n=32)and control group (n=37);the patients in test group were treated with PVP comined with implantation 125 I radioactive particle and the patients in control group were treated with PVP only.The heights of anterior and posterior vertebral bodies of the patients before and after treatment were detected by X-ray.The numerical rating scale (NRS)scores,pain relief rate and the incidence of surgical complications of the patients were recorded before operation and 1 d,1 week,1 month,3 months,and 6 months after operation.Results:The operation was successfully performed in all the patients without local bleeding;there were no movement dysfunction and nerve compression phenomenon.There was no leakage of bone cement.All the 125 I radioactive particles located well and there was no particle obscission.The heights of vertebral bodies of the patients in two groups after operation were increased compared with before operation (P <0.05).The NRS scores of the patients in two groups s at 1 d,1 week,1 month,3 months,6 months after operation were significantly decreased compared with before operation (P <0.05);compared with control group,the NRS scores of the patients in test group at 1 d,1 week, 1 month,3 months,6 months after operation were decreased (P <0.05).The incidence of pulmonary embolism or radiation myelitis complications was about 4.3% in 69 patients.Compared with control group,the difference in the incidence of complications of the patients in test group was not significant (P < 0.05 ).Conclusion:PVP combined with 125 I radioactive particle implantation is a safe and effective method in the treatment of vertebral metastasis,which can relieve the pain of the patients obviously compared with PVP.

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.