OBJECTIVES:Cardiac involvement in idiopathic inflammatory myopathies (IIM) is rare but potentially severe. Anti-mitochondrial M2 antibody (AMA-M2) has been implicated in cardiac involvement, but the association remains underexplored. This study aims to evaluate the clinical, pathological and prognostic features of AMA-M2 IIM. METHODS:This historic prospective cohort included IIM patients hospitalized at Peking Union Medical College Hospital between 2008 and 2020. Outcomes were prospectively collected through the Prospective Registry Of MyositIS (PROMIS) registry. Cox regression models were employed to identify risk factors of cardiac involvement and mortality. RESULTS:Among 987 IIM patients, 55 (6%) were AMA-M2 positive. These patients exhibited higher rates of PM (56% vs 23.5%, P < 0.001), and elevated baseline gamma-glutamyl transferase (78.0 vs 35.0, P < 0.001) and alkaline phosphatase (85.0 vs 64.0, P < 0.001). Throughout disease courses, AMA-M2-positive patients had significantly higher rates of cardiac involvement (60% vs 12.9%, P < 0.001), including arrhythmias (56%), heart failure (44%) and pulmonary hypertension (31%). Some of the muscle biopsies showed features consistent with immune-mediated necrotizing myopathy, cardiac biopsies demonstrating structural degeneration with minimal inflammation and liver biopsies confirming early-stage primary biliary cholangitis (PBC). Multivariate Cox analysis identified AMA-M2 positivity as an independent risk factor for cardiac involvement (hazard ratio 3.156, P < 0.001). Despite frequent cardiac manifestations, long-term survival did not differ between AMA-M2-positive and -negative patients (mean survival: 103.9 months vs 98.0 months, P = 0.86). CONCLUSION:AMA-M2 positivity defines an IIM subgroup with significant cardiac involvement and an immune-mediated inflammatory muscle histology, but not necessarily worse long-term survival. These findings highlight the need for early recognition and tailored management of AMA-M2 IIM.

The early angiopathy of systemic sclerosis (SSc) is a key hub connecting inflammation and fibrosis. The animal models that simulate the pathological characteristics of SSc are of great significance to study the etiology, pathogenesis and treatment of SSc with vascular involvement. This review of the characteristics of different animal models of SSc with vascular involvement provides a reference for relevant researchers to select appropriate models in the process of exploring the pathogenesis of SSc with vascular involvement.

Background: Patients with systemic autoimmune diseases (sAIDs) are susceptible to active tuberculosis (ATB), especially in high-burden countries and regions. However, a comprehensive overview of the ATB burden across the full spectrum of sAIDs remains lacking. Methods: In this nationwide retrospective cohort study, we linked individual-level data from the Chinese Rheumatism Data Center (CRDC), the Infectious Disease Reporting System (IDRS), and the Tuberculosis Information Management System (TBIMS). Patients of all ages registered in CRDC between 2016 and 2023, with a confirmed diagnosis of 1 of 10 sAIDs, including rheumatoid arthritis, systemic lupus erythematosus, spondyloarthritis, primary Sjögren's disease, idiopathic inflammatory myopathies, undifferentiated connective tissue disease, systemic sclerosis, Behçet's disease (BD), Takayasu arteritis (TAK), and ANCA-associated vasculitis (AAV), were included. We estimated incidence rates, point prevalence, cumulative incidence, and age- and gender-adjusted standardized incidence ratios (SIRs) for ATB, and examined associations between SIRs and region-level socioeconomic indicators using Cox proportional hazards models. Findings: Among 255,103 eligible patients, 2,775 incident ATB cases were confirmed across all 10 sAIDs. The overall ATB incidence rate in 2023 was 157.79 per 100,000 person-years. Point prevalence of ATB among patients with sAIDs rose from 393.83 per 100,000 people in 2016 to 904.65 per 100,000 people in 2023. Cumulative incidence exceeded 1% within 10 years of sAID diagnosis. Patients with TAK (7.60, 95% CI 4.92-11.22), AAV (6.96, 4.90-9.59), BD (6.30, 4.25-9.00) carried the highest relative risks of ATB, and male and female patients exhibited comparable ATB susceptibility compared with general population. At the area level, higher urbanization was associated with increased ATB risk in patients with sAIDs, whereas higher educational attainment was protective. Interpretations: Patients with sAIDs constitute a distinctly high-risk population for ATB, with a burden substantially exceeding that of the general population and marked heterogeneity across diseases and socioeconomic context. These findings support the systematic integration of targeted TB screening and preventive strategies into routine rheumatology care, particularly in TB-endemic settings.

Abstract Neuropsychiatric systemic lupus erythematosus (NPSLE) is a potentially severe complication of systemic lupus erythematosus (SLE), yet its pathogenesis remains largely elusive. By jointly probing the immune dynamics of subjects’ cerebrospinal fluid (CSF) and peripheral blood, we showed that both innate and adaptive immune responses jointly contribute to the pathogenesis of NPSLE. In particular, we found the remarkable enrichment of BAM-CCL3, a subtype of border-associated macrophages with strong recruitment capacity, implicating its potential role in central nervous system (CNS) inflammation. We also observed pronounced activation of memory B cells and CD4 + regulatory T cells in NPSLE CSF, along with the preferential blood-to-CSF migration and subsequent within-CSF clonal expansion of CD8 + effector memory T cells in NPSLE patients, suggesting a persistent CNS-localized adaptive immune dysregulation. Finally, we developed the single-cell CNS disease CSF–Blood Atlas (scCDCB), a comprehensive collection for CSF and peripheral blood of multiple CNS diseases, which is publicly available at ( https://sccdcb.gao-lab.org ) to serve as a reference for future research on CNS diseases.

Background:Myocardial involvement frequently occurs in patients with systemic sclerosis (SSc), leading to myocardial tissue changes and subclinical ventricular dysfunction. This form of primary cardiac involvement is one of the major determinants of mortality in SSc and may eventually progress to overt heart failure. To date, the pathophysiology and subsequent subclinical myocardial dysfunction in asymptomatic patients with SSc have not been extensively described. This study aimed to assess subclinical myocardial deformation and tissue abnormalities in asymptomatic patients with SSc using non-contrast cardiac magnetic resonance (CMR) feature tracking and native T1 mapping, and to explore the relationship between native T1 values and myocardial strain. Methods:The patients with SSc and age- and sex-matched healthy controls (HCs) underwent non-contrast CMR. Myocardial strain parameters and myocardial native T1 values were measured and compared between the HCs and patient subgroups based on skin involvement and disease duration. The association between the myocardial strain parameters and myocardial native T1 values was analyzed using Pearson's or Spearman's correlation and multivariate regression models. Results:A total of 44 patients with SSc and 22 HCs were enrolled in the study. The patients with SSc had significantly lower absolute longitudinal peak strain (-17.5%±3.0% vs. -18.9%±2.3%, P=0.030) and peak diastolic strain rate (0.9±0.2 vs. 1.1±0.3 1/s, P=0.025) than the HCs, after adjustment for age, sex, and body mass index. The patients with SSc had significantly higher myocardial native T1 values compared with the HCs (1,278.4±59.7 vs. 1,233.1±27.9 ms, P<0.001). The myocardial native T1 values demonstrated moderate correlations with the longitudinal strain parameters (|r|=0.40-0.56) in the patients with SSc, but this association did not persist after adjustment for confounders in multivariate regression models. No significant differences were found in the myocardial strain parameters and myocardial native T1 values between the patient subgroups (all P>0.05). Conclusions:Reduced absolute myocardial longitudinal strain and elevated myocardial native T1 values in patients with SSc indicate myocardial dysfunction and tissue impairment. These findings suggest that CMR may serve as a valuable tool for characterizing early alterations of the heart and optimizing the clinical management of patients with SSc.

To evaluate the comparative value of traditional clinical features and novel immune-inflammatory indices in assessing disease activity in primary Sjögren's Disease (SjD), and to construct a robust classification model for moderate-to-severe disease activity using a machine learning approach. This cross-sectional study included 18,738 SjD patients from the Chinese Rheumatism Data Center (CRDC) multicenter prospective cohort. Spearman rank correlation and local polynomial regression (LOESS) were used to explore the relationship between systemic inflammatory indices and the EULAR Sjögren's syndrome disease activity index (ESSDAI). To identify moderate-to-severe disease activity (ESSDAI ≥ 5), data were randomly split into training and validation sets (7:3). Classification models were developed using various machine learning techniques. Higher ESSDAI scores were significantly associated with elevated levels of both traditional markers (ESR, CRP, IgG) and novel composite indices (NLR, CAR, CLR, IBI) (all p < 0.001). However, these indices exhibited only weak-to-moderate linear correlations with ESSDAI and displayed non-linear saturation trends. In multivariable logistic regression, traditional markers remained independent risk factors, whereas all novel composite indices lost statistical significance, yielding a poor validation area under the curve (AUC) of 0.678. Conversely, the XGBoost model demonstrated modestly favorable overall performance Feature importance analysis revealed that patient-reported outcomes, hemoglobin, WBC, age, and IgG were the top contributors to the model, while novel inflammatory indices were completely excluded. Novel immune-inflammatory indices lack provide no independent discriminative value for moderate-to-severe SjD activity when adjusted for traditional parameters. The XGBoost machine learning model effectively handles non-linear variables and highlights the core diagnostic importance of patient-reported outcomes (ESSPRI) and traditional clinical features, providing a favorable evaluation tool.

Objective: To evaluate the efficacy and safety of Xeligekimab (GR1501), a fully human monoclonal antibody against interleukin-17A, in Chinese patients with active axial spondyloarthritis (axSpA) who did not respond or were intolerant to nonsteroidal anti-inflammatory drugs (NSAIDs). Methods: This phase II, randomized, double-blind, placebo-controlled, multicenter study enrolled 160 patients with active axSpA. Participants were randomized equally to receive placebo or Xeligekimab at 100 mg, 200 mg, or 300 mg subcutaneously every two weeks for 16 weeks, followed by an 8-week follow-up. The primary endpoint was the Assessment of SpondyloArthritis International Society 20 (ASAS20) response at week 16. Secondary endpoints included ASAS40, ASDAS-CRP, BASDAI, BASFI, and patient-reported outcomes. Safety and immunogenicity were evaluated throughout the study. Results: At week 16, ASAS20 response rates were 52.5% with placebo and 77.5%, 75.0%, and 72.5% with Xeligekimab 100 mg, 200 mg, and 300 mg, respectively. The 100 mg and 200 mg groups showed significant improvement versus placebo (P < 0.05). Benefits in ASAS40, ASDAS-CRP, and BASDAI were maintained through week 24. Xeligekimab was generally well tolerated, and the overall incidence of adverse events was comparable to placebo. No treatment-emergent anti-drug antibodies were detected. Conclusion: Xeligekimab at 100 mg and 200 mg achieved meaningful clinical improvement with good tolerability in patients with active axSpA, supporting further clinical evaluation of this therapy.

Background Patients with systemic lupus erythematosus (SLE) complicated by immune thrombocytopenia (SLE-ITP) exhibit an increased incidence of bleeding events and significantly reduced long-term survival, with poor responses to conventional therapies as well as biologic agents. Methods In an investigator-initiated, single-arm, dose-escalation trial, six patients with refractory SLE-ITP received anti-CD19 chimeric antigen receptor (CAR) T cells after lymphodepleting chemotherapy. The primary outcome was safety. Secondary outcomes included overall response rate at time points (complete remission [CR]: platelet count ≥ 100 × 109/L; partial remission [PR]: ≥30 × 109/L with 2-fold baseline increase). Findings All patients achieved clinical response (CR or PR)—with three attaining CR—and discontinued immunosuppressants, with a median follow-up of 12 months. Treatment-related adverse events were limited to grade 1 cytokine release syndrome in two patients, and no immune effector cell-associated neurotoxicity syndrome (ICANS) occurred. Continuously accumulating bone marrow plasma cells, overactivated bone marrow B cell signaling pathways, and weakened activity of the megakaryocyte maturation pathway, along with dysregulated transcription factors, were observed in three PR patients by exploratory single-cell multi-omics. Conclusion These results preliminarily support CD19 CAR T cell therapy as a promising and safe treatment for refractory SLE-ITP. Large-scale trials are needed to verify these conclusions (ClinicalTrials.gov: NCT05930314). Funding This study was supported by the Chinese National Key Technology R&D Program, Ministry of Science and Technology (2021YFC2501300); the CAMS Innovation Fund for Medical Sciences (CIFMS) (2021-I2M-1-005); and National High Level Hospital Clinical Research Funding (2025-PUMCH-D-001, 2022-PUMCH-B-013, D-009). This study was also funded and supported by Juventas Cell Therapy Ltd.

Background and Objectives: Neuropsychiatric (NP) manifestations are common, heterogeneous and severe in systemic lupus erythematosus (SLE) patients, with attribution to SLE remaining diagnostically challenging. Traditional classification focuses on clinical syndromes, overlooking neuropsychiatric systemic lupus erythematosus (NPSLE) immunological heterogeneity. To address the heterogeneity of NPSLE, this study aimed to delineate distinct disease subgroups by clustering patients based on their antibody profiles. These subgroups were then evaluated for diferences in clinical presentation and prognosis, to better characterize disease subsets and support individualized approaches to diagnosis and management. Methods: This retrospective single-center study included hospitalized SLE patients with NP manifestations, collecting demographic, clinical and laboratory data. Patients were classified as NPSLE or non-NPSLE by clinical judgment after excluding alternative causes. Hierarchical cluster analysis explored autoantibody-clinical feature associations. Results: Among the 167 patients analyzed, 152 had NP manifestations attributed to SLE. Central nervous system (CNS) involvement was predominant (89.1%), with seizures, cerebrovascular disease, acute confusional state (ACS), and demyelinating syndrome being most prevalent manifestations. Hierarchical clustering of 152 NPSLE patients identified two subgroups: Cluster 1 (23.7%) demonstrated cerebrovascular injury as the predominant manifestation, with higher positivity rates of antiphospholipid antibodies (APLs) (P < 0.01) and a higher incidence of cerebrovascular disease (P < 0.01). Cluster 2 (76.3%) showed immune-mediated inflammatory profile, with higher positivity of anti-SSA (P < 0.01), antidsDNA (P < 0.05), and anti-RiboP antibodies (P < 0.05). Neurological involvement predominantly manifesting as ACS (P < 0.05), accompanied by a higher frequency of fever and joint involvement. Conclusions: In this study, NPSLE exhibited distinct serological profiles and segregated into two immunologically defined clusters, reflecting its clinical and biological heterogeneity, and suggesting that immunological profiling may enhance precise classification and personalized management of affected patients.

OBJECTIVE:This multicenter study aimed to analytically compare the performance of automated quantitative assays-chemiluminescence immunoassay (CLIA) and multiplexed bead immunoassay (MBI)-for detecting anti-Ro/La antibodies in Chinese patients with primary Sjögren's syndrome (pSS), compared to line immunoassay (LIA) that is most commonly used in China. METHODS:Based on the Chinese Sjögren's Syndrome Collaborative Research Group, serum samples from 434 patients with pSS and 100 healthy controls were analyzed using LIA, CLIA, and MBI. Sensitivity, specificity, and qualitative agreement were assessed. Receiver operating characteristic (ROC) analysis was carried out to compare the analytical accuracy among assays in detecting anti-Ro/La antibodies, and the DeLong test was used to compare areas under curves (AUCs). RESULTS:High specificity (95% to 100%) was observed in these assays, and LIA demonstrated the highest sensitivity for anti-Ro60 (92.6%) and anti-Ro52 (89.2%). CLIA and MBI exhibited comparable sensitivity to LIA for anti-La (50.9% vs. 50.2% vs. 48.2%). Qualitative agreement among assays was good for anti-Ro60 (κ: 0.84-0.93) and moderate for anti-La (κ: 0.74-0.86). MBI achieved the highest AUC values for anti-Ro60 (0.980), anti-Ro52 (0.970), and anti-La (0.935), outperforming CLIA and LIA (p  < 0.05). CONCLUSION:Automated assays (CLIA and MBI) demonstrate high specificity and analytical accuracy for anti-Ro/La antibody detection in pSS. MBI achieved the highest accuracy, whereas LIA showed superior sensitivity for anti-Ro60/52 detection. CLIA provided reliable specificity but failed to detect anti-Ro52 in this study. Collectively, these findings indicate that CLIA and MBI may represent reliable alternatives to LIA, although validation in disease-controlled cohorts is required before clinical implementation.

Objective Renal Fanconi syndrome(FS)is a rare renal manifestation of primary Sjögren's syndrome(pSS).This study aims to analyze the clinical and prognostic characteristics of patients with pSS-associ-ated renal FS(pSS-FS)and provide insights for clinical management.Methods Patients diagnosed with pSS-FS via renal biopsy at Peking Union Medical College Hospital from 1993 to 2024 were enrolled.Data collected in-cluded age,sex,clinical symptoms(xerostomia,xerophthalmia,skin purpura,arthralgia,polyuria,and systemic symptoms),laboratory findings[serum immunoglobulin G(IgG)and IgM,complement(C3,C4),antinuclear antibody,anti-Sjögren's syndrome-associated antigen A antibody(SSA),anti-SSB antibody,24-hour urinary pro-tein quantification,tubular proteinuria,serum creatinine,serum electrolytes],treatment,and follow-up informa-tion.Systematic assessments included the EULAR Sjögren's Syndrome Disease Activity Index(ESSDAI)score,pulmonary involvement(including non-infectious interstitial pneumonia,pulmonary fibrosis,pulmonary hyperten-sion,etc.),hematological involvement(anemia,leukopenia,thrombocytopenia),etc.Efficacy evaluations en-compassed improvements in immunological parameters,renal function,and tubular function.Group comparisons were performed using chi-square/Fisher's exact tests,t-tests,or non-parametric tests.Changes over time were an-alyzed using paired t-tests,and repeated measures during follow-up were assessed using mixed linear models.Results A total of 38 patients with pSS-FS were included,with 37(97.4%)being female.The median age at pSS diagnosis was 43(37,57)years.Xerostomia(76.3%)and xerophthalmia(71.1%)were the predominant clinical symptoms.The most common renal tubular dysfunctions were generalized aminoaciduria(96.9%),tubular proteinuria(96.0%),and hypokalemia(94.7%).The median eGFR was 52.57(32.04,76.10)mL/(min·1.73 m2),with60.5%(23/38)of patients having an eGFR below60 mL/(min·1.73 m2).After six months of immunosuppressive therapy,including moderate-to-high-dose glucocorticoids,significant improvements were observed in immunological parameters(improvement rate:69.2%),renal tubular function(89.5%),and renal function(44.4%).Following immunosuppressive treatment,the median eGFR increased from 54.95(33.06,76.10)mL/(min·1.73 m2)to 65.56(56.24,83.58)mL/(min·1.73 m2).Compared to patients with normal or mildly impaired baseline eGFR[≥60 mL/(min·1.73 m2)],those with significantly de-creased baseline eGFR[＜60 mL/(min·1.73 m2)]were older(46 years vs.37 years),had higher ESSDAI scores(16 vs.13),higher 24-hour urinary protein levels(1.16 g/d vs.0.48 g/d),but lower positivity rates of serum anti-SSA antibody(36.4%vs.86.7%)and anti-SSB antibody(22.7%vs.73.3%).Moreover,this group showed more pronounced renal function improvement after 6 months(58.8%vs.20.0%)and 12 months(66.7%vs.25.0%)of immunosuppressive therapy.Conclusions This study reports the clinical characteristics of the lar-gest single-center cohort of pSS-FS patients internationally,characterized by varying degrees of proximal renal tu-bular dysfunction and renal impairment.Timely initiation of immunosuppressive therapy,including glucocorticoids,is crucial,particularly for patients with significantly reduced eGFR,who may experience more substantial renal function improvement.

Primary Sjögren's syndrome (pSS) patients with low disease activity remain at risk for disease progression, yet predictive factors are poorly understood. We aimed to identify risk factors for disease worsening in pSS patients with initially low disease activity. We conducted a registry-based cohort study using prospectively collected data from the Chinese Rheumatism Data Center (CRDC). Patients with pSS meeting either 2002 AECG or 2016 ACR/EULAR classification criteria and baseline ESSDAI < 5 were included. Disease worsening was defined as ESSDAI increase ≥ 3 points during follow-up. Cox proportional hazards regression with LASSO selection identified independent risk factors. Among 745 patients (median age 46 years, 97.4

To evaluate whether low-to-moderate dose glucocorticoids provide additional benefit in primary Sjögren’s syndrome patients treated with hydroxychloroquine and/or iguratimod. Using the Chinese Rheumatism Data Center database, we emulated a pragmatic trial including 395 patients with primary Sjögren’s syndrome treated between May 2016 and August 2025. All patients received hydroxychloroquine and/or iguratimod as background therapy. Patients were categorized by glucocorticoid use: HCQ/IGU plus glucocorticoid group (≤ 30 mg/day prednisone equivalent, n = 188) or HCQ/IGU group (n = 207). Primary outcome was ESSDAI score change at 1 month. Secondary outcomes included ESSPRI scores and laboratory parameters. Treatment effects were estimated using overlap weighting of propensity scores. Stratified analyses examined subgroups by baseline ESSDAI, IgG levels, and arthritis status. After propensity score weighting, baseline characteristics were well-balanced. At 1 month, glucocorticoids provided no additional benefit in ESSDAI change compared to hydroxychloroquine/iguratimod (Cohen’s d = − 0.12; 95

PV048 / #473 Poster Topic:AS05 - CNS Lupus Patients with Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) experience impaired quality of life, while the underlying causes remain unknown. Seizure, one of the common subtypes of NPSLE, is predictive of poor prognosis for SLE. In this study, we evaluated clinical features and the risk factors with prognostic outcome in a large dataset of NPSLE with seizure in China. NPSLE inpatients and outpatients with seizure (after diagnosed of SLE, or before but within 6 months) treated mainly at Peking Union Medical College Hospital and other multiple institutions around China between July 2011 and June 2024 were considered. SLE was diagnosed when the SLE classification criteria recommended by the American College of Rheumatology (ACR) in 1997 or those of the Systemic Lupus International Collaborating Clinics (SLICC) in 2012 and the diagnostic criteria for NPSLE in SLE proposed by the ACR in 1999 were fulfilled. Clinical data and laboratory examination results were retrospectively collected. The SLE disease activity index (SLEDAI) was used to evaluate lupus activity. SLEDAI ≥ 15 points indicates severe SLE activity. SPSS 27.0 software and GraphPad Prism 10.2.0 were used for statistical analyses. Data were collected from a total of 632 NPSLE patients with seizures; of these, 556 (88.0%) were female, with an average age of 34.1 years (range, 9-73). The median duration of SLE was 3.0 years (range, 0-42), and the median duration of NP was 2 months (range, 0-150). Among all patients, 301 (47.6%) had central nervous system (CNS) involvement aside from seizures, 20 (3.2%) had peripheral nervous system (PNS) involvement, and 13 (2.1%) had both. The most common NP subtypes were acute confusional state (ACS), cognitive impairment, cerebrovascular disease, headache, and psychosis (Table 1). In the prognostic analysis, 23 patients (3.6%) died, of whom 20 (87.0%) were women, and 17 (73.9%) were older than 25 years. Comparing the deceased and surviving groups, there were significant differences in the prevalence of serositis (p = 0.003), respiratory involvement (p = 0.002), cardiac involvement (p = 0.003), renal involvement (p = 0.013), proteinuria (p = 0.025), elevated serum creatinine (Scr) (p < 0.001), and SLEDAI score ≥ 15 (p = 0.021). These findings indicate that these factors are associated with a poor prognosis (Table 2). Table 1. Baseline characteristics of NPSLE patients with seizure. Table 2. Prognostic analysis of clinical characteristics of NPSLE patients with seizure. NPSLE patients with seizures, combined with serositis, respiratory, cardiac, or renal involvement, proteinuria, elevated serum creatinine, or high disease activity, may have a poorer prognosis.

Objectives Research on the specific role of immunoglobulin G (IgG) glycosylation in SLE development and progression is limited, especially regarding changes in IgG glycosylation profiles among different SLE subtypes. In this study, we aimed to characterise the glycosylation profile of serum IgG in patients with SLE.Methods Lectin microarrays with 56 lectins were used to analyse serum IgG glycosylation in 194 patients with SLE, 100 disease controls (40 primary Sjögren’s syndrome (pSS), 60 rheumatoid arthritis (RA)) and 100 healthy controls (HCs). Differences between SLE and control groups, as well as SLE subgroups, were validated by lectin blotting. Altered IgG glycosylation patterns were identified and further confirmed. Receiver operating characteristic (ROC) analysis evaluated the diagnostic value of these glycosylation changes in SLE and its subgroups, including neuropsychiatric SLE (NPSLE), lupus nephritis (LN), pulmonary arterial hypertension, immune thrombocytopaenia and SLE without major organ involvement (WMOI).Results Compared to DC and HC groups, the IgG glycan level of Galβ3GalNAc (binding Jacalin (11.3%) and Maclura pomifera lectin (14.4%)) was significantly increased, whereas most IgG glycan levels were significantly decreased, including core fucose, high mannose, GlcNAc, GalNAc and Galβ4GlcNAc in the SLE group (all p<0.05).The IgG glycan levels were elevated in GalNAc and galactose patterns in the NPSLE group compared to the WMOI group, as well as higher Galβ3GalNAc and galactose patterns in NPSLE and LN compared to HCs.Moreover, ROC curve analysis showed PNA levels might have moderate potential for discriminating SLE from pSS.Conclusions Patients with SLE show disease-specific alterations in serum IgG glycosylation, and aberrant Galβ3GalNAc, galactose and GalNAc glycosylation may have diagnostic value for SLE and NPSLE. Abnormal IgG glycans may provide new insights into their roles in SLE pathogenesis and progression.