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 investigate the safety of tocilizumab (TCZ) in the treatment of children with systemic juvenile idiopathic arthritis (sJIA).Methods:Data of children aged 2 to 18 years with the diagnosis of sJIA and treated with TCZ from June 1, 2017 to June 30, 2022 at our hospital were retrospectively collected. The clinical medication characteristics, incidence, severity and outcome of adverse drug reactions (ADR) were statistically analyzed. Univariate and multivariate analysis were used to analyze the risk factors of TCZ-induced ADR. Univariate comparison between groups were compared to the measured data followed by t test for normal distribution, and the counting data were paired with Chi-square test. Binary logistic regression analysis was used for multivariate analysis. Results:A total of 83 eligible children were enrolled. The age at TCZ initiation was (8.5±3.7) years old. Most of the children received oral glucocorticoid (86.8%) and/or methotrexate (72.3%) prior to TCZ treatment. The mean time of TCZ duration was (1.2±0.9) years, the total TCZ exposure was 92.70 patient years. Fifty-five (66.3%) children reported 123 ADR, with a rate of 132.69/100 patient years. Forty-two (50.6%) children reported 103 general ADR, with a rate of 111.11/100 patient years. Eighteen (21.7%) children reported 20 serious ADR, with a rate of 21.57/100 patient years. The results of univariate analysis showed that the dosage of glucocorticoid in ADR group was higher than that in non-ADR group [(0.76±0.50) mg·kg -1·d -1vs. (0.52±0.41) mg·kg -1·d -1, t=2.27, P=0.026], and the difference was statistically significant. However, there were no significant differences in gender [(male 23, female 32) cases vs. (male 9, female 19) cases, χ2=0.73, P=0.392], age at TCZ initiation [(8.5±3.8) years old vs. (9.0±3.1) years old, t=-0.65, P=0.516], duration of TCZ treatment [(1.24±1.00) years vs. (1.05±0.90) years, t=0.87, P=0.385], methotrexate doses weekly [(8.0±5.2) mg/m 2vs. (7.6±5.1) mg/m 2, t=0.39, P=0.696], and history of drug or food allergy (11 cases vs. 5 cases, χ2=0.06, P=0.815) between the two groups. The results of binary logistic regression analysis showed that the combined use of oral glucocorticoids was an independent risk factor for TCZ-induced ADR [ OR (95% CI) =3.05 (1.11, 8.36), P=0.030]. The risk of ADR was 3.05 times higher in the combined daily dose of glucocorticoids ≥0.76 mg/kg prednisone equivalent than that of < 0.76 mg/kg. Common general ADR to TCZ include infections (38.83/100 patient years) and abnormalities in laboratory parameters (37.76/100 patient years) such as elevated glutamic-pyrupiane transaminase (18.34/100 patient years), dyslipidemia (12.94/100 patient years), and hemocytopenia (5.39/100 patient years). The serious ADR included serious infection (9.71/100 patient years) and serious infusion reaction(7.55/100 patient years). All ADR were improved after drug withdrawal or symptomatic treatment, and no deaths occurred. Conclusion:TCZ has a good safety profile in the treatment of sJIA. Serious infections and severe infusion reactions often lead to discontinuation of the drug. The combination of glucocorticoids≥0.76 mg/kg prednisone equivalent is an independent risk factor for TCZ-induced ADR. Monitoring should be strengthened during the application of TCZ, and ADR should be detected and treated as early as possible to reduce the risk of medication related adverse reactions.

Objective:To investigate the efficacy of a proximal femoral locking plate (LPFP) versus a proximal femoral anti-rotation intramedullary nail (PFNA) in the treatment of femoral intertrochanteric fractures in older adult patients. Methods:A total of 130 older adult patients with femoral intertrochanteric fractures who received treatment in Linghu People's Hospital of Huzhou from May 2017 to June 2020 were included in this study. They were randomly assigned to undergo treatment with either a PFNA (observation group, n = 65) or an LPFP (control group, n = 65). Intraoperative blood loss, incision length, operative time, and time to fracture healing were determined in each group. At 1, 3, and 6 months after surgery, the Harris hip score was used to evaluate hip joint recovery. Coxa vara, incision infection, and internal fixation loosening were compared between the two groups. Results:Intraoperative blood loss in the observation group was less than that in the control group [(189.26 ± 48.15) mL vs. (96.47 ± 40.21) mL, t = -11.93, P < 0.001]. Incision length, operative time, and time to fracture healing in the observation group were significantly shorter than those in the control group [(4.03 ± 1.48) cm vs. (12.16 ± 1.55) cm, (72.13 ± 28.75) minutes vs. (120.34 ± 29.01) minutes, (9.89 ± 1.52) weeks vs. (13.63 ± 1.74) weeks, t = -30.59, -9.52, -13.05, all P < 0.001]. At 1 month after surgery, there was no significant difference in Harris hip score between the two groups ( t = 1.28, P > 0.05). At 3 and 6 months after surgery, the Harris hip score gradually increased in the control and observation groups ( F = 13.44, 8.26, both P < 0.001). At 3 and 6 months after surgery, Harris hip scores in the observation group were significantly higher than those in the control group [(85.17 ± 4.29) points vs. (79.50 ± 4.12) points, (95.30 ± 1.04) points vs. (87.69 ± 1.25) points, t = 7.69, 37.73, both P < 0.001]. The incidence of complications in the observation group was significantly lower than that in the control group [1.54% (1/65) vs. 10.77% (7/65), χ2 = 4.80, P = 0.029). Conclusion:Compared with LPFP, PFNA can effectively reduce intraoperative blood loss in older adult patients with femoral intertrochanteric fractures, accelerate the progress of fracture healing, promote the recovery of the hip joint, and has fewer complications. Therefore, PFNA is worthy of popularization.

Rheumatic diseases are inflammatory diseases characterized by severe immune dysregulation, which can affect tissues and joints.Neutrophils are the key factor contributing to immune disorders, and they play an important role in rheumatic diseases.Neutrophil extracellular traps (NETs) are network structures released by neutrophil granulocytes when stimulated.As a novel immune defense mechanism, NETs have attracted wide attention.In this paper, the formation, function and role of NETs in rheumatic diseases and other diseases were reviewed.

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.

Objective:To analyze the in-hospital mortality and influential factors of total hip arthroplasty (THR) in older adult patients.Methods:A total of 130 older adult patients subjected to THR in Department of Orthopedics, Huzhou Linghu People's Hospital between August 2019 and August 2021 were included in this study. In-hospital mortality was calculated. These patients were divided into death and survival groups according to whether they were dead or alive. Sex, age, smoking, drinking, disease type, complications, cardiovascular disease history, operation severity score, operative time, amount of intraoperative blood loss, postoperative osteoporosis treatment and postoperative rehabilitation training were compared between the two groups. The risk factors that influence in-hospital mortality were analyzed using logistic analysis method.Results:Among 130 patients, 9 patients died in Huzhou Linghu People's Hospital, with the mortality of 6.92%. Thus, there were 9 patients in the death group and 121 patients in the survival group. There were significant differences in age, smoking, complications, operation severity score and postoperative osteoporosis treatment between the two groups ( t = 1.70, χ2 = 5.48, χ2 = 4.09, t = 2.86, χ2 = 4.03, all P < 0.05). Multivariate logistic analysis showed that the age (≥ 85 years old), smoking (yes), complications (≥ 3), operation severity score (≥ 15 points) were the risk factors that influence in-hospital mortality. Postoperative osteoporosis treatment was the protective factor of THR. Conclusion:The in-hospital mortality of older adult patients after THR is high. Age ≥ 85 years old, smoking, complications ≥ 3, and operation severity score ≥ 15 are the risk factors of THR and may greatly affect the prognosis. More attention should be paid to older adult patients who have these risk factors.

Objective:Kawasaki disease shock syndrome(KDSS) is a serious complication of Kawasaki disease(KD). The main manifestations are low blood pressure and decreased blood perfusion at the acute stage of onset, which is life-threatening.The purpose of this study was to provide early intervention and reduce the complications and mortality of this disease by analyzing clinical features and expression level of interleukin-6(IL-6)of children with KDSS.Methods:In this study, a total of 25 children with KDSS in Nanjing Children′s Hospital were collected, and their clinical characteristics, relevant laboratory indicators and IL-6 expression levels were analyzed by retrospective case-control study.Results:Compared with ordinary KD children, KDSS children were more common in older children, with prolonged fever, severe skin rash, high inflammatory indicators, and more likely to be associated with coronary dilatation.The level of IL-6 in children with KDSS in the acute phase was significantly higher than that in children with KD[(28.5±39.2) ng/mL vs.(226.8±102.9) ng/mL, P<0.05], while the level of IL-6 in children with KDSS in the convalescent period was significantly lower than that in the acute phase of KDSS[(226.8±102.9) ng/mL vs.(5.6±1.7)ng/mL, P<0.05], and the difference was statistically significant. Conclusion:The systemic inflammatory response of KDSS is more obvious, IL-6 plays an important role in it, and the therapeutic effect of IL-6 blocker in KD and its complications needs further study.

Severe combined immunodeficiency(SCID)is a kind of severe primary immunodeficiency disease.Patients often develop symptoms after birth, which is characterized by recurrent and life-threatening infection, sometimes accompanied by varying degrees of dysplasia.According to the classification proposed by the PID expert Committee of the International Union of Immunological Societies, SCID is clinically classified by the number of lymphocytes.Early screening and intervention of SCID is significant to improve the prognosis.With the promotion of TREC and other early screening methods, the detection rate of SCID has significantly improved, but the accurate detection rate in different ethnic groups still needs further explored.Hematopoietic stem cell transplantation is a traditional therapy for radical cure of SCID.And the safety of gene therapy for SCID has been gradually guaranteed with continuous improvement during recent years.This article will review the classification, early screening methods and treatment progress of SCID.

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.

The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA)is the most common cause of periodic fever in childhood.The diagnosis of PFAPA is typically made on clinical grounds.The patients usually show normal growth and development.The current pharmacological treatment includes corticosteroids for the management of febrile episodes, colchicine for the prophylaxis of febrile episodes, and other medication with unproven efficacy.Tonsillectomy is an option for selected patients.Usually PFAPA syndrome resolves during adolescence, but there is increasing evidence that this condition may persist into adulthood.This article reviews the pathogenesis and treatment of PFAPA in order to raise pediatricians′ awareness of this disease.