Belimumab is the only biologic approved for childhood-onset systemic lupus erythematosus (cSLE), yet evidence on the optimal timing of initiation and duration of therapy remains limited. This study aimed to evaluate the impact of belimumab treatment duration and timing of initiation on clinical outcomes in cSLE. This retrospective study included 182 patients with cSLE treated with belimumab at Beijing Children’s Hospital between September 2015 and September 2025. Patients were stratified by timing of initiation (≤ 6 months vs. > 6 months from diagnosis) and treatment duration. Primary outcomes included childhood Lupus Low Disease Activity State (cLLDAS) and childhood Clinical Remission (cCR). In a subgroup of 95 patients with ≥ 2 years of follow-up, outcomes were compared by treatment duration (< 1 year, 1–2 years, ≥ 2 years) and by timing of initiation. Multivariable logistic regression was performed to identify independent predictors. Among 182 patients (81.9

OBJECTIVES:Enthesitis-related arthritis (ERA) is a form of chronic inflammatory arthritis. Even when administered with a combination of non-steroidal anti-inflammatory drugs (NSAIDs), disease-modifying anti-rheumatic drugs (DMARDs), and biologics, some patients remain in an active disease state. Antecedent studies have revealed that interleukin-17 (IL-17) plays an important role in the pathogenesis of ERA and have demonstrated the efficacy of IL-17 inhibitors. This real-world, retrospective study aimed to assess the efficacy and safety of secukinumab in patients with ERA. METHODS:This was a retrospective, single-center cohort study of 31 Chinese patients who had been diagnosed with ERA and treated with secukinumab for at least 3 months. The primary outcomes were the proportion of patients achieving JIA ACR 30/50/70/90/100 response criteria and ACR inactive disease, as well as the change in Juvenile Spondyloarthritis Disease Activity (JSpADA) Index from baseline. Secondary outcomes included changes in the number of joints with active arthritis and enthesitis, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR). Outcomes were assessed every 3 months after secukinumab initiation. The Wilcoxon signed-rank test and paired t-test were used to analyze the data. RESULTS:Male predominance (24/31, 77.4%), late disease onset, HLA-B27 positivity (13/31, 41.9%), and enthesitis (10/31, 32.3%) were the key characteristics of our cohort. The median follow-up duration was 0.9 years (ranging from 0.4 to 2.7 years). At the last follow-up, ACR 30/50/70/90/100 response rates were 87% (27/31), 68% (21/31), 65% (20/31), 32.3% (10/31) and 12.9% (4/31); 25.8% (8/31) of patients achieved ACR inactive disease. JSpADA scores decreased from baseline (median 5.0, IQR: 3.5-5.0) to month 3 (median 3.5, IQR: 2.5-4.0; median difference = 1.25, Z = -4.26, p < 0.001, 95% CI: 0.8 to 2.0), to month 6 to 3.0 (IQR: 2.0-4.0; median difference = 1.5, Z = -3.85, p < 0.001, 95% CI: 1.0 to 2.3), month 9 to 3.0 (IQR: 1.0-3.0; median difference = 2.3, Z = -3.09, p = 0.002, 95% CI: 1.5 to -3.0) and month 12 to 2.5 (IQR: 1.0-3.0; median difference = 2.25, Z = -2.95, p = 0.003, 95% CI: 1.5 to 3.3). The mean active joint count decreased from 9.3 ± 0.7 at baseline to 3.3 ± 0.5 at 12 months. Normalization of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) was observed. CONCLUSION:In this retrospective, single-center study, secukinumab appears to be associated with improvement in arthritis and disease activity in Chinese children with ERA. Further studies are needed to investigate the correlation between IL-17 and ERA.

Double-negative T cells (DNTs) are significantly elevated in autoimmune diseases and are thought to play an important role in inflammation. The purpose of this study was to explore their role in childhood-onset systemic lupus erythematosus (cSLE). DNTs, as well as T and B cell subsets in peripheral blood, were detected by flow cytometry in 78 patients, including 34 cSLE. Clinical and laboratory data of cSLE patients were collected to analyze the correlation between DNTs and these indices, including demographics: proportion of female patients and mean age (± SD); Organ involvement: presence of lupus nephritis, neuropsychiatric manifestations, and pulmonary involvement; Hematologic parameters: leukopenia, anemia, and thrombocytopenia (WBC, Hb, and PLT counts); Autoantibody profiles: ANA, anti-dsDNA, and anti-Sm antibodies. The changes in DNT levels after glucocorticoid treatment were observed, and the effects of different doses of glucocorticoids on DNTs were analyzed. DNT levels were significantly increased in the peripheral blood of cSLE patients. DNTs were correlated with SLE disease activity, organ involvement, the production of autoantibodies, naive B cells, and plasmablasts. DNT levels increased after low-dose glucocorticoid treatment (9.12 ± 1.43 vs 14.24 ± 1.36, p < 0.01) but gradually decreased with increasing glucocorticoid doses (14.24 ± 1.36 vs 13.45 ± 1.51 vs 7.45 ± 1.01 vs 4.72 ± 1.20, p < 0.05). DNT levels significantly decreased from the fourth day of glucocorticoid pulse therapy. DNT levels were positively correlated with disease activity in cSLE patients, and the effect of glucocorticoid dose on DNT levels varied.

Systemic juvenile idiopathic arthritis (sJIA) is an autoinflammatory disorder that impacts children aged ≤ 16 years. This study evaluated the efficacy and safety of firsekibart, a fully human anti-IL-1β monoclonal antibody, in children with active sJIA. This multicenter, randomized, open-label, active-controlled phase 2 study (NCT05925452) enrolled participants aged 2– < 18 years with active sJIA across 14 sites in China. Participants were randomized (1:1:1) to firsekibart 3.0 mg/kg or 4.0 mg/kg subcutaneously every 4 weeks, or tocilizumab intravenously every 2 weeks, for 24 weeks. The primary endpoint was modified JIA American College of Rheumatology Pediatric criteria (ACR Pedi) 30 response at day 28. Secondary endpoints included ACR Pedi 30/50/70/90 responses, corticosteroid tapering, immunogenicity, and safety. Fifty participants were randomized (firsekibart 3.0 mg/kg: n = 17; 4.0 mg/kg: n = 16; tocilizumab: n = 17). At day 28, ACR Pedi 30 response rates were 94.1

OBJECTIVE:To investigate clinicopathological correlations, treatment responses, and predictors of outcomes in childhood lupus nephritis (cLN). METHODS:Retrospective cohort study of 71 biopsy-proven cLN patients at Beijing Children's Hospital (2005-2024). Renal biopsies were classified per 2018 ISN/RPS criteria with activity index (AI) and chronicity index (CI) assessment. RESULTS:Over a median 40-month follow-up, 69.1% were female (mean onset age 10.0 ± 2.3 years). Proliferative LN (Class III/IV ± V) accounted for 76.0%. AI correlated with acute injury markers (creatinine, BUN, proteinuria); CI showed stronger associations with chronic tubular biomarkers but not eGFR. Complete renal remission (CRR) was achieved in 92.8% (mean 13.4 months); 26.6% experienced relapse. Failure to achieve CRR was associated with higher AI (OR = 1.738, p = 0.045), elevated SLEDAI-2K (OR = 1.339, p = 0.023), and lower C3 (OR = 0.002, p = 0.035). Higher CI predicted lower likelihood of achieving LLDAS (OR = 1.425, p = 0.042). Belimumab reduced relapse risk (OR = 0.220, p = 0.043), decreased glucocorticoid doses (8.42 vs. 16.54 mg/day, p = 0.034), and improved LLDAS (68.4% vs. 30.0%, p = 0.006) and clinical remission rates (52.6% vs. 20.0%, p = 0.016). CKD developed in 4.3%; mortality was 1.4%. CONCLUSION:Modern treatment yields favorable outcomes for cLN. Baseline disease activity, hypocomplementemia, and histological AI predict treatment response. AI and CI demonstrate distinct associations with acute and chronic injury. Belimumab offers significant advantages in relapse prevention and glucocorticoid reduction.

This study assessed pharmacokinetics and safety of subcutaneous (SC) belimumab in Chinese paediatric patients with systemic lupus erythematosus (cSLE) who previously received intravenous (IV) belimumab; only the IV form is approved for cSLE in China. This single-arm, open-label, 12-week bridging study (GSK Study 217091) characterised belimumab 200 mg SC exposure in Chinese paediatric patients (5–17 years) with cSLE who completed the 48-week IV belimumab Phase 4 trial (GSK Study 213560). Safety and tolerability of SC belimumab on-treatment and in a 16-week follow-up period were also assessed. Patients were categorised based on weight; data were summarised using descriptive statistics. Overall, 16 patients were enrolled (≥ 15– < 30 kg, n = 1; ≥ 30– < 50 kg, n = 5; ≥ 50 kg, n = 10) with a mean age of 12.9 years. The geometric mean approximate Cmax (3 days post-administration) after the first and last dose were 92.44, 63.41 and 68.87 μg/mL, and 28.01, 85.11 and 88.61 μg/mL for the three weight groups, respectively. The geometric mean Ctrough at steady state were, 20.44, 71.57 and 85.55 μg/mL for the ≥ 15– < 30 kg, ≥ 30– < 50 kg and ≥ 50 kg weight groups, respectively. Thirteen patients (81

OBJECTIVE:Establishing a predictive model using clinical indicators for the early identification of JIA-U. METHOD:A cross-sectional study was conducted with 255 patients admitted at Beijing Children's Hospital between 2018 and 2023. The model was fitted using stepwise logistic regression as well as least absolute shrinkage and selection operator (LASSO) regression. Calibration and decision curve analysis were used for validation. RESULTS:The final predictive model included four clinical variables (patient's gender, age at onset, arthritis subtype, and ANA status). A nomogram for risk prediction was developed, which demonstrated good discrimination in both the training cohort (AUC = 0.8417; 95% CI = 0.775-0.9085) and the testing cohort (AUC = 0.782; 95% CI = 0.6752-0.8884). Calibration curves showed that, through bootstrap resampling, the nomogram performed well in predicting the occurrence of uveitis in JIA. CONCLUSION:This study established a predictive model using routine clinical indicators to assess the risk of uveitis in JIA patients.

Objective:To characterize the clinical features, risk factors, and outcomes of juvenile idiopathic arthritis-associated uveitis (JIA-U), aiming to improve early detection and management strategies. Methods:This study conducted a retrospective cohort analysis of JIA patients diagnosed and treated at the Department of Rheumatology at Beijing Children's Hospital (2016-2023), with subgroup evaluation of JIA-U cases. Results:Among 1494 JIA patients, 72 (4.82%) developed uveitis. The oligoarticular subtype (OJIA, 47.2%) and enthesitis-related arthritis (ERA, 27.8%) predominated. Uveitis onset occurred at a median of 10 months post-arthritis diagnosis (range: 0-86 months), with 93% manifesting within 4 years. Chronic anterior uveitis was the most frequent phenotype. ANA positivity and HLA-B27 were significantly associated uveitis. First-line acute management involved topical corticosteroids, with methotrexate escalation for severe cases and TNF-α inhibitors (adalimumab preferred) for refractory disease. Ocular complications arose in 25.9% during follow-up. Conclusion:Uveitis, often bilateral and insidious, is a common extra-articular manifestation of JIA. Absent arthritis signs may delay diagnosis, highlighting the need for regular screening and close rheumatology-ophthalmology collaboration to optimize outcomes.

ObjectiveJuvenile dermatomyositis (JDM) is a rare but chronic autoimmune disease with systemic nonsuppurative inflammation. Many studies have focused on the clinical characteristics and therapy of JDM. Whereas a few studies have reported the epidemiological characteristics and social burden of patients with JDM internationally, no study has been performed in China to date.MethodsThis study was based on the Futang Updating Medical Records (FUTURE) database. Data were extracted from registry information in inpatient medical records. The epidemiological characteristics and economic burden of Chinese patients with JDM were analyzed.ResultsA total of 1164 patients with JDM from 24 hospitals were enrolled from January 2016 to December 2021. The ratio of boys to girls was 1:1.23, and half were between 6 and 12 years old. Over half (n = 629) were admitted to the hospital at least twice for intensive treatment. In total, 20.02% of patients with JDM had lung involvement and 2.23% experienced subcutaneous calcification. The median number days of hospitalization was 10 (IQR 6-14), whereas the median hospitalization expense was US $2370.50 (IQR 1373.70- 3541.90). Lung involvement was the most frequent complication, causing high inpatient burden, length of stay, and expense. Nearly 17% of patients with JDM were admitted to the hospital as emergencies, suggesting a severe disease activity stage requiring urgent treatment. No deaths occurred in our study.ConclusionIn our study, we analyzed the epidemiological characteristics and social burden of patients with JDM in China, contributing to the enhanced comprehension and effective management of JDM in the country.

ObjectivesThis study aims to investigate CD4+ central memory T cells (CD4+ TCM) levels in childhood-onset systemic lupus erythematosus (cSLE) and their association with disease activity, clinical features, and treatment responses.MethodsA total of 202 children with newly diagnosed, untreated rheumatic diseases were recruited, comprising 64 cases of cSLE, 71 cases of juvenile idiopathic arthritis, 31 cases of juvenile dermatomyositis, 36 cases of autoinflammatory diseases, and 22 healthy controls. Lymphocyte subsets were analyzed using multi-color flow cytometry, and clinical data and laboratory test results were collected. The correlation between CD4+ TCM levels and SLEDAI scores, clinical manifestations, autoantibodies, and kidney injury markers was examined. Subsequently, 21 cSLE patients underwent follow-up assessments and retesting post-treatment.ResultsThe proportion of CD4+ TCM (44.3 ± 11.5%) in cSLE was significantly higher compared to those with other pediatric rheumatic diseases (p < .05). A negative correlation was observed between the level of CD4+ TCM and the SLEDAI-2000 score (r = -0.255, p = .021), indicating that higher disease activity was associated with lower CD4+ TCM levels. Furthermore, CD4+ TCM levels were negatively correlated with oral ulcers (r = -0.285, p = .011) and positively correlated with leukopenia (r = 0.302, p = .008). In terms of laboratory indicators, CD4+ TCM showed negative correlations with anti-dsDNA antibodies (r = -0.294, p = .009) and anti-histone antibodies (r = -0.232, p = .033), while exhibiting a positive correlation with anti-Sm antibodies (r = 0.245, p = .025). Additionally, CD4+ TCM demonstrated significant negative correlations with early renal injury markers, urinary transferrin (r = -0.315, p = .008), and urinary microalbumin (r = -0.284, p = .015). CD4+ TCM was strongly negatively correlated with CD4+ Naive cells (r = -0.831, p < .001), positively correlated with other memory cell subsets, and negatively correlated with IFN-α levels (r = -0.364, p = .031). Longitudinal analysis revealed a time-dependent biphasic pattern in CD4+ TCM levels. Cyclophosphamide-treated patients showed significantly increased CD4+ TCM levels compared to non-cyclophosphamide groups (p = .034).ConclusionsCD4+ TCM likely plays a central immune regulatory role in cSLE, with its levels closely associated with disease activity, specific autoantibody production, and early organ damage. Post-treatment changes in CD4+ TCM levels may indicate therapeutic efficacy and suggest their potential as biomarkers, offering a fresh perspective on immune memory regulation in cSLE and exploring novel treatment approaches.

Blau syndrome (BS) is a rare autoinflammatory disease characterized by a clinical triad of uveitis, dermatitis and arthritis. The aim of our study was to summarize organ involvement, predict disease prognosis and evaluate treatment response. Clinical data of 47 Chinese children who were diagnosed with Blau syndrome in Beijing Children’s hospital, Capital Medical University was retrospectively analyzed. Direct sequencing of NOD2 gene was performed by sanger sequencing. Data were analyzed through SPSS 21.0. A Bayesian network was constructed to integrate prediction algorithms of genetic mutations and clinical manifestations, exploring the complex relationship between genotype and phenotype through R (Version 4.4.1, R Core Development Team). P value < 0.05 was significant. The 47 patients included 26 males and 21 females. Median age of disease onset was 13.64 months, ranging from 1 to 51 months. At baseline, incidence of fever, arthritis, rash, dermatitis and uveitis were 34

Abstract Background Circular RNA (circRNA) plays an important role in the pathogenesis of many diseases and can be used as a biomarker for diagnosis or disease monitoring. However, reports on circRNA in childhood‐onset systemic lupus erythematosus (cSLE) are limited. Therefore, this study aimed to investigate circEPSTI1 expression in cSLE and evaluate its potential as a biomarker for diagnosing cSLE. Methods This study included 70 children diagnosed with cSLE, 20 diagnosed with juvenile idiopathic arthritis (JIA), 20 diagnosed with juvenile dermatomyositis (JDM), and 50 healthy children at the Rheumatology Department of Beijing Children's Hospital from January 2019 to December 2019. Quantitative polymerase chain reaction was used to determine circEPSTI1 expression in the children. Correlations between circEPSTI1 and clinical features were assessed using Spearman's correlation test. Additionally, we calculated the receiver operating characteristic curve to assess the diagnostic efficacy. Results We found that circEPSTI1 expression was higher in children with cSLE (4.62 ± 3.55) than that in healthy children (1.00 ± 0.45), those with JDM (1.06 ± 0.76), and those with JIA (0.96 ± 0.48). The area of the curve of circEPSTI1 was 0.892 (95% confidence interval [CI]: 0.832–0.952, p < 0.001) to discriminate children with SLE from healthy children, with a specificity of 0.814 and a sensitivity of 0.922. Children with lupus nephritis showed a higher circEPSTI1 expression than healthy children, those with JDM, and those with JIA. In addition, circEPSTI1 expression in children with SLE showed significant correlations with the SLE Disease Activity Index (p < 0.0001) and C3 concentrations (p = 0.001). Conclusion Our study suggests that circEPSTI1 is a promising biomarker for the diagnosis and monitoring of cSLE.

Regulatory T cells (Tregs) play a critical role in maintaining immune homeostasis and preventing autoimmune diseases. Recent advances in immunometabolism have revealed the pivotal role of mitochondrial dynamics and metabolism in shaping Treg functionality. Tregs depend on oxidative phosphorylation (OXPHOS) and fatty acid oxidation (FAO) to support their suppressive functions and long-term survival. Mitochondrial processes such as fusion and fission significantly influence Treg activity, with mitochondrial fusion enhancing bioenergetic efficiency and reducing reactive oxygen species (ROS) production, thereby promoting Treg stability. In contrast, excessive mitochondrial fission disrupts ATP synthesis and elevates ROS levels, impairing Treg suppressive capacity. Furthermore, mitochondrial ROS act as critical signaling molecules in Treg regulation, where controlled levels stabilize FoxP3 expression, but excessive ROS leads to mitochondrial dysfunction and immune dysregulation. Mitophagy, as part of mitochondrial quality control, also plays an essential role in preserving Treg function. Understanding the intricate interplay between mitochondrial dynamics and Treg metabolism provides valuable insights for developing novel therapeutic strategies to treat autoimmune disorders and enhance immunotherapy in cancer.

Objective: Double-negative T cells (DNTs) were significantly elevated in autoimmune diseases and were thought to play an important role in inflammation. The purpose of this study was to explore its important role in children with systemic lupus erythematosus (cSLE). Methods: The DNTs, T and B cell subsets of peripheral blood were detected by flow cytometry in 78 patients, and clinical and laboratory data of cSLE patients were collected to analyze the correlation between DNTs and the above indexes. The changes of DNTs after glucocorticoids were detected, and the effects of different doses of glucocorticoids on DNTs were analyzed. Results: DNTs was significantly increased in peripheral blood of cSLE patients. DNTs were associated with SLE disease severity and organ involvement, as well as with the production of autoantibodies, Naive B cell and plasmablast cells. The level of DNTs increased after low-dose glucocorticoid treatment, but the proportion of DNTs gradually decreased with the increase of glucocorticoid dose. Conclusion: DNTs was positively correlated with disease severity in cSLE patients, and the effect of glucocorticoid dose on DNTs was different.

OBJECTIVES:In recent years, the distinct clinical presentations and elevated mortality rates of various subtypes of juvenile idiopathic arthritis (JIA) with pulmonary involvement have garnered significant attention. This study aimed to elucidate the clinical characteristics of pulmonary involvement in patients with JIA to improve clinicians' knowledge. METHODS:This single-centre retrospective study analysed the baseline data, treatment options, follow-up of sixty patients of JIA with pulmonary involvement in China. Patients with interstitial lung disease (ILD) were further classified in accordance with the 2013 American Thoracic Society/European Respiratory Society International multidisciplinary consensus on idiopathic interstitial pneumonia. RESULTS:Sixty patients (5.03%) with JIA were complicated with pulmonary involvement. The highest subtype was systemic JIA (sJIA, 63.3%), followed by rheumatoid factor (RF)-positive polyarthritis (pJIA, 25.0%). The incidence of macrophage activation syndrome (MAS) was 21.6%. The most common diagnosis was ILD (90%). Respiratory symptoms/signs were initially experienced by 61.7% of the patients, and respiratory support was required by 21.7%. High-resolution CT classification of sJIA revealed non-specific interstitial pneumonia (NSIP) and organising pneumonia. High-resolution CT classification of pJIA was NSIP and usually interstitial pneumonia (UIP). Patients were treated with NSAIDs, along with glucocorticoids, DMARDs, and biological agents. The survival rates after 1 and 5 years were approximately 93.3% and 90.0%, respectively. CONCLUSIONS:Patients with JIA with pulmonary involvement present with early onset, high mortality rate. JIA patients should undergo physical examination thoroughly and high-resolution CT scans, lung function tests for evaluating and monitoring the occurrence and development of pulmonary involvement in early stages to improve prognosis.

This study aims to assess current diagnostic and management for systemic Juvenile Idiopathic Arthritis (sJIA) among physicians, evaluate the challenges encountered in diagnosis and treatment, and identify the educational needs and professional development engagements of physicians managing sJIA. A nationwide survey was conducted from November 2023 to March 2024 across tertiary and secondary pediatric and general hospitals in China. The survey targeted physicians with at least three years of specialty experience, resulting in 310 valid responses from 25 provinces, autonomous regions, and municipalities. The survey collected data on diagnostic practices, treatment approaches, and professional development related to sJIA. Data collection was facilitated through WeChat, and statistical analysis was performed using descriptive statistics. Ethical approval was obtained from the Ethics Committee of Beijing Children’s Hospital, with informed consent provided electronically by participants. The survey indicated that all physicians encountered suspected or confirmed cases of sJIA, highlighting its prevalence and the diagnostic challenges associated. Regarding diagnostic standards, 53.9

ABSTRACT Importance Systemic lupus erythematosus (SLE) is a diffuse connective tissue disease with complex clinical manifestations and prolonged course. The early diagnosis and condition monitoring of SLE are crucial to disease prognosis. Objective To assess the diagnostic value of long noncoding RNA (lncRNA) nuclear enriched abundant transcript 1 (NEAT1) in childhood‐onset SLE (cSLE). Methods Fifty‐seven children diagnosed with SLE, 40 children diagnosed with juvenile idiopathic arthritis (JIA), and 40 healthy children were included. Peripheral blood samples from each patient were collected. A quantitative polymerase chain reaction was used to confirm the expression of lncNEAT1_1 and lncNEAT1_2 in peripheral blood. Associations among parameters were analyzed using the Mann‐Whitney U test or independent sample t ‐test. Results The expression of both lncNEAT1_1 and lncNEAT1_2 in patients with cSLE were significantly higher than that of healthy control and patients with JIA. Receiver operating characteristic curves revealed an area under the curve (AUC) of 0.633 (95% confidence interval [CI], 0.524–0.742; P = 0.024) for lncNEAT1_1. The AUC of lncNEAT1_2 was 0.812 (95% CI, 0.727–0.897; P < 0.0001) to discriminate individuals with cSLE from health control and children with JIA with a sensitivity of 0.622 and a specificity of 0.925. Moreover, lncNEAT1_2 expression was higher in patients with cSLE presenting with fever, lupus nephritis, elevated erythrocyte sedimentation rate, active disease activity, and decreased C3 level, compared with those without these conditions. However, no similar correlation was observed for lncNEAT1_1. Interpretation The expression of lncNEAT1_2 was significantly elevated in children with SLE, especially those with fever, renal involvement, and low C3 levels. These findings suggest that lncNEAT1_2 may represent a potential biomarker for cSLE.

Background Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by extensive immune cell dysregulation. The use of Imiquimod (IMQ), a topical immune response modifier, in animal models generates lupus-like symptoms, providing a valuable platform for probing the disease's mechanisms. Methods This study utilized single-cell RNA sequencing (scRNA-seq) to characterize the splenic cells from both IMQ-induced lupus model mice and control mice. Over 33,000 cells were analyzed and categorized into various immune cell subtypes based on gene expression markers. Results Our analysis of over 33,000 splenic cells from IMQ-induced lupus model and control mice revealed significant increases in the proportions of plasma cells, macrophages, and neutrophils in the lupus model. Further, B cell heterogeneity was dissected, revealing novel B cell subtypes and significant pathway enrichment related to B cell receptor signaling and cellular stress responses. In T cells, distinct subtype dynamics and pathway enrichments, including those associated with T cell activation and differentiation, were identified. Analysis of dendritic and neutrophil subtypes revealed specific transcriptional changes and pathway activations related to immune system processes. Lastly, enhanced cellular interactions and regulatory network analyses uncovered altered signaling pathways and key transcription factors like Foxp3, Lef1, and Cebpa, which are implicated in governing immune responses in lupus. Conclusion The application of scRNA-seq has unveiled the intricate immune landscape in lupus, demonstrating that IMQ-induced models effectively replicate key aspects of human lupus. The study not only enhances our understanding of lupus pathogenesis but also highlights potential targets for therapeutic intervention based on altered cell proportions, gene expression, and cell-cell interactions.

Given the limited tocilizumab (TCZ) treatment data for systemic juvenile idiopathic arthritis (sJIA) in China, we evaluated the long-term efficacy and safety of TCZ in Chinese patients with sJIA. In this multicentre, interventional Phase IV study, patients with sJIA and inadequate clinical response to non-steroidal anti-inflammatory drugs/corticosteroids received TCZ infusions every 2 weeks based on body weight (< 30 kg, 12 mg/kg; ≥ 30 kg, 8 mg/kg), over a 52-week open-label period and an 8-week safety follow-up period. The primary endpoint was the proportion of patients with a JIA American College of Rheumatology (ACR) 30 response and absence of fever at Week 12. Sixty-two patients were enrolled and treated (12-mg/kg group, 34; 8-mg/kg group, 28). At Week 12, 87.1