ObjectiveWhile triple antiphospholipid antibody (aPL) positivity is associated with a higher risk of thrombosis, the clinical significance of single aPL positivity remains unclear. This study aimed to assess the prevalence, clinical characteristics, and cardiovascular disease (CVD) risk profile of persistently single aPL-positive primary antiphospholipid syndrome (PAPS) patients.MethodsWe conducted a retrospective analysis for APS patients from the APS ACTION registry with confirmed persistent single aPL positivity (after testing for lupus anticoagulant [LA], anticardiolipin [aCL] IgG/IgM, or anti-β2-glycoprotein I [aβ2GPI] IgG/IgM), defined by the Revised Sapporo APS Classification Criteria. Triple aPL-tested single aPL-positive patients with no APS classification were used as a control group. Demographics, clinical characteristics, aPL profiles, and adjusted Global APS Score (aGAPSS) were compared between two groups.ResultsAmong 233 PAPS patients tested, 84 (36%) had single aPL positivity: 67 (79%) LA positive, eight (10%) aCL IgG or IgM positive, and 9 (11%) aβ2GPI IgG or IgM positive. Among 97 aPL-positive patients with no APS classification, 22 (22.7%) had single aPL-positivity: nine LA positive, eight aCL IgG or IgM positive, and 5 aβ2GPI IgG or IgM positive. Single LA positivity was significantly more common in PAPS patients, whereas aCL IgM or aβ2GPI IgM positivity were significantly more common in those without APS classification. Historical thrombosis recurrence was observed in 26% of single aPL-positive PAPS patients, predominantly among those with LA positivity (86%).ConclusionsIn this international cohort of persistently aPL-positive individuals, 36% had single aPL positivity, mostly positive LA test. Although "single" aPL-positivity is traditionally considered as a lower-risk profile, findings highlight the importance of differentiating between single LA-positivity and single aCL/aβ2GPI positivity, and the need for individualized risk stratification.
ObjectivesTo explore pregnancy-related concerns among women with systemic lupus erythematosus (SLE) and impact of the disease on desired family size.MethodCross-sectional, single-center study, including women with SLE diagnosed before menopause. Data were collected via questionnaires and medical records. Participants were classified into three groups: (i) those who never conceived (Group 1); (ii) those who had conceived before SLE diagnosis (Group 2), and (iii) those who had conceived at least once after disease diagnosis (Group 3). Continuous variables were analyzed using Kruskal-Wallis with Mann-Whitney U post-hoc tests; categorical variables using Chi-square or Fisher's exact tests.Results152 patients were included and 75% had received information from their treating physician on reproductive health. Most common pregnancy-related concerns were the need to receive medication during pregnancy (56.6%), followed by concerns about pregnancy complications (53.9%). Women in Group 1 were younger and mainly attributed not having children to personal choice and not to SLE (56.4%). In contrast, women in Groups 2 and 3 frequently reported having fewer children than desired (35.3% and 44.6%, respectively), mostly due to SLE-related concerns (83.3% and 86.2%, respectively). In Group 3, deviation from desired family size was smaller among women who conceived in more recent periods (2015-2024, 32%), although attribution of deviations to SLE remained high.ConclusionsSLE affects women's reproductive decisions, underscoring the importance of tailored counseling.
BackgroundSystemic Lupus Erythematosus (SLE) is a heterogeneous autoimmune disease characterized by dysregulated type I interferon signaling. Epigenetic alterations, particularly DNA methylation changes in interferon-regulated genes, have emerged as promising biomarkers for disease diagnosis and stratification. Among these, IFI44L promoter hypomethylation has been repeatedly reported as one of the most consistent and disease specific in SLE.ObjectiveTo systematically evaluate the evidence on IFI44L promoter methylation in SLE and to quantitatively synthesize its epigenetic and diagnostic performance across diverse populations using meta-analytic approaches.MethodsA systematic search of published literature was conducted to identify studies reporting IFI44L promoter methylation in patients with SLE and controls. Study characteristics, ethnicity, cell type, direction of methylation, CpG hypo/hyper methylation counts were extracted. Descriptive analyses were performed across ethnicities and cell types. Diagnostic performance was summarized as the pooled area under the receiver operating characteristic (ROC) curve (AUC). Variance of AUC estimates was approximated using the Hanley-McNeil method, and a random-effects meta-analysis (DerSimonian-Laird) was applied to account for between-study heterogeneity.Results16 study datasets reporting hypomethylation of the IFI44L promoter were included. IFI44L promoter hypomethylation was consistently observed across ethnic groups and biological sample types. Descriptive subgroup analyses identified heterogeneity in the magnitude of IFI44L promoter hypomethylation across ethnic groups, study periods, and methylation profiling methodologies, whereas differences according to biological sample type were less pronounced and did not reach statistical significance. Hypomethylation predominated over hypermethylation among studies reporting both methylation directions. Diagnostic AUC values varied across studies but were largely independent of sample size and the magnitude of hypomethylation. The pooled area under the curve (AUC) was 0.74 (95% CI 0.64-0.84), indicating moderate overall diagnostic performance.ConclusionIFI44L promoter hypomethylation represents a significant and reproducible epigenetic signature of SLE across populations and study designs. Despite variability in methylation magnitude, its diagnostic performance remains consistent, supporting IFI44L methylation as a promising biomarker for SLE.
IntroductionSystemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease associated with impaired health-related quality of life (HRQoL). The LupusQoL is a disease-specific instrument designed to capture SLE-related HRQoL domains; however, a validated Arabic version remains unavailable.ObjectiveTo evaluate the reliability and validity of the Arabic version of LupusQoL (Ar-LupusQoL) in SLE Egyptian patients.MethodsIn this multicenter cross-sectional study, 156 patients with SLE completed the Arabic LupusQoL and Short Form-36 (SF-36). Disease activity and damage were assessed using Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI), respectively. Internal consistency was assessed using Cronbach's alpha, and test-retest reliability using intraclass correlation coefficients. Construct validity was examined through item-total correlations and correlations with corresponding SF-36 domains. Discriminant validity was also evaluated.ResultsThe mean age was 33.37 ± 10.71 years, and 98.1% were female. The cohort demonstrated a high level of disease activity (87.2% with SLEDAI >4; mean SLEDAI 19.52 ± 16.20). Internal consistency was high across domains (α = 0.78-0.98). Item-total correlations were strong (r > 0.30, p < 0.001). Significant correlations with corresponding SF-36 domains supported convergent validity (r = 0.668-0.829, p < 0.05). The Ar-LupusQoL effectively discriminated between active and inactive disease (p < 0.001). Ceiling effects were observed in selected domains, with no significant floor effects.ConclusionThe Arabic version of the LupusQoL is a reliable and valid instrument for assessing HRQoL in Egyptian patients with SLE. The high disease activity observed, likely reflecting recruitment from tertiary referral centers, may limit generalizability to patients with milder disease.
Nocardiosis is an uncommon infection that presents as a chronic, debilitating illness with radiographic manifestations simulating lung cancer or tuberculosis. Immunocompromised hosts may develop a fulminant disease resembling acute bacterial pneumonia or disseminated disease. We report a case of a juvenile lupus who developed disseminated Nocardia pseudobrasilienisis infection in form of necrotizing pneumonia, subcutaneous nodules and central nervous system (CNS) lesion in the right parietal lobe, following inadvertent hiking of steroids after a minor organ flare. This case was successfully treated with intravenous meropenem, amikacin, and oral cotrimoxazole therapy for 6 weeks which lead to resolution of pneumonia, and partial resolution of of the cutaneous and CNS lesion. As the patient was immunosuppressed, and had disseminated nocardiosis, we planned to put her on oral cotrimoxazole for the next 1 year.
ObjectivesSystemic lupus erythematosus (SLE) is a major autoimmune disease. Recent studies have found that changes in lipid metabolism and gut microbes are associated with the pathogenesis of SLE. However, the coordination of gut commensal bacteria and SLE lipid metabolism is not clear.MethodsGut microbiota profiling was performed using 16S rRNA gene sequencing of fecal samples, and functional prediction was conducted using PICRUSt. Plasma lipidomics was performed using LC-MS. Associations between differentially abundant microbial taxa and differential lipids were assessed using Spearman's rank correlation.ResultsWe found obvious abnormalities in lipid metabolism in SLE patients. Sphingolipid metabolism and glycerophospholipid metabolism were the most significantly dysregulated pathways in these patients. There is an obvious intestinal flora imbalance in SLE patients, the Becteroides and Lachnoclostridium is the most important genus to distinguish SLE case groups from control groups. Some differential metabolites in the plasma of SLE patients were correlated with some differential bacteria in the stool. Most of the bacteria associated with lipid changes belonged to Firmicutes, Bacteroidetes, Actinobacteriaceae, Peptostreptococcaceae, Bacteroidetes, Gordonibacter, Romboutsia, etc.ConclusionOur findings illustrate the disruption of the gut microbiome and lipid group in SLE patients, which may facilitate the development of new SLE interventions.
ObjectiveHematological involvement is a common manifestation of juvenile-onset systemic lupus erythematosus (jSLE). While nephrological and neurological involvement often guide treatment decisions in the early disease course, hematological findings may also impact morbidity and mortality. The aim of this study is to evaluate the clinical characteristics and treatment approaches of jSLE patients with hematological involvement.MethodThis retrospective, single-center cohort study was conducted on patients diagnosed with jSLE who were followed up at the pediatric rheumatology clinic between January 2015 and May 2025. Patients included in the study had been diagnosed with jSLE according to the 2012 Systemic Lupus International Collaborating Clinics classification criteria.ResultsThe study included 53 SLE patients, 48 of whom (90.6%) were female. The median age at diagnosis was 13 years (IQR: 11-15), and the median follow-up period was 26 months (IQR: 12-48). Hematological involvement was detected in 28 patients (52.8%). Anemia was observed in 18 (64.3%) of 28 patients, followed by lymphopenia in 16 (57.1%), thrombocytopenia in 14 (50.0%), and pancytopenia in 4 (14.3%). The median time to improvement of cytopenia following treatment was 32 days (IQR: 27-61). In 11 patients, treatment was based on isolated hematological findings, whereas in 17 patients with additional major organ involvement, treatment was mainly directed by the major organ manifestations. The frequency of constitutional symptoms, hypocomplementemia, and direct Coombs positivity were found to be significantly higher in patients with hematological involvement (p = 0.003, p = 0.034, p = 0.039, respectively). Intravenous ımmunoglobulin (IVIG) was also found to be administered more frequently in patients with hematological involvement (p = 0.010).ConclusionHematological involvement was detected in approximately half of jSLE patients. Hypocomplementemia and positive Coombs test were more frequently observed in patients with hematological involvement. The use of IVIG was also more common in patients with hematological involvement, and individualized treatment options remain important in the management of the disease.
IntroductionNewborns of mothers with autoimmune systemic connective tissue disease (CTD) have a higher incidence of major congenital heart defects (CHDs) compared to unexposed newborns. Less is known about the association between maternal CTD and less severe cardiac abnormalities in the newborn.MethodsWe analysed prospectively collected echocardiographic data from the Copenhagen Baby Heart Study (CBHS), comparing newborns exposed to maternal CTD with those who were not exposed. Maternal autoimmune CTD diagnoses were identified through the National Patient Register and validated by medical record review. Outcome measures included minor CHDs (atrial and ventricular septal defects, bicuspid aorta valve and patent ductus arteriosus), as well as cardiac dimensions and function.ResultsIn total 25,590 newborns underwent echocardiography in the CBHS, of whom 57 (0.22%) were born to mothers with CTD. When comparing newborns of mothers with overall CTD to non-exposed newborns, we found no differences in structural and functional cardiac parameters. Minor CHDs were more common in newborns born to mothers with Sjögren's disease (n = 3, 33.3%) than in unexposed newborns (n = 1,945, 7.6%, p = 0.03).ConclusionsIn this large population-based study, we did not observe consistent associations between overall maternal CTD and cardiac structure or function in the infant, although minor CHD were more common in newborns exposed to maternal Sjögren's disease. Findings should be interpreted with caution, considering the small sample size of exposed newborns and potential underrepresentation of major CHD due to standard clinical management practices.
IntroductionRemission is a key treatment goal associated with improved patient outcomes in systemic lupus erythematosus (SLE), often measured by Definition of Remission In SLE (DORIS) criteria. In a post hoc analysis of clinical trial data, belimumab, a B-cell modulator targeting the central immunopathogenic pathway in SLE, plus standard therapy, improved DORIS remission rates versus placebo plus standard therapy; however, US real-world remission rates remain limited. Measuring DORIS criteria in real-world clinical practice is challenging because required variables, particularly the SLE Disease Activity Index (SLEDAI) component, are often unavailable or incompletely recorded. Therefore, we evaluated an adapted DORIS definition for real-world datasets and examined predictors of remission among US patients initiating belimumab.MethodsThis retrospective observational cohort study analysed data for patients with SLE initiating belimumab from the OM1 PremiOM™ SLE dataset, comprising electronic health records/healthcare claims data (2013-2024) for patients with SLE. The primary outcome was probability of achieving adapted DORIS remission at 28, 48, and 52 weeks post-treatment initiation, defined as clinician-recorded SLEDAI (crSLEDAI) or estimated SLEDAI (eSLEDAI) = 0, Physician Global Assessment (PGA) <2 on a 0-10 numerical rating scale, and prednisone-equivalent dose ≤5 mg/day. Kaplan-Meier estimates provided remission probabilities and logistic regression identified predictors of remission.ResultsMost patients (N = 398) were White, female, from the Southern region of the USA, and had commercial insurance. The probability of achieving adapted DORIS remission reached 28.3% (95% confidence interval: 19.8-35.9) by 52 weeks post-belimumab initiation. Older patients (≥50 years), non-White individuals, and patients with cr/eSLEDAI ≤5 demonstrated slightly higher remission probabilities than their counterparts; however, estimates differed by censoring approach, and confidence bounds overlapped. Adjusted multivariable analysis confirmed increasing age, non-White race, and cr/eSLEDAI ≤5 as significant predictors of remission.ConclusionThis study demonstrates the application of an adapted DORIS definition to large real-world observational datasets. The probability of adapted DORIS remission in US patients initiating belimumab was similar to rates reported in observational studies outside the USA, supporting the real-world effectiveness of belimumab. The increased likelihood of remission in patients with cr/eSLEDAI ≤5 supports the benefits of initiating belimumab treatment early in the disease course.
BackgroundSystemic lupus erythematosus (SLE) is a complex autoimmune disease with 0.4 million new cases diagnosed annually. With its wide variety of visible and invisible manifestations, people living with SLE report being exposed to stigmatization, which impacts their personal and professional lives. However, the current literature is unclear on whether healthcare management teams assess this concern during follow-up. This study aims to synthesize existing evidence on the prevalence and determinants of stigma among people living with SLE.MethodsThis systematic review and meta-analysis gathered evidence from observational studies identified from three databases on 16 July 2025. Dual independent screening, data extraction, and risk-of-bias assessment (using the Newcastle-Ottawa Scale) were performed. Results were synthesized using descriptive statistics, narrative synthesis, and indicator-level meta-analyses.ResultsWithin the past two decades, 11 studies comprising 2254 people living with SLE reported and measured stigma- and discrimination-related events using various scales. Stigma was found to be prevalent across its three constructs: interpersonal, perceived, and intrapersonal stigma. This review demonstrated that people living with SLE reported a moderate overall burden of stigma (34.71 [95% CI 26.15, 43.27]), with average stigma scores indicating psychological impact. Additionally, nearly one in two persons (46% [95% CI 28-66%]) experienced at least one form of stigma or discrimination, most commonly social isolation and unfair treatment. Mental health associations were correlated with higher stigma burden.ConclusionThis review demonstrates that stigma and discrimination are not just social challenges but also critical determinants of health. With cautious interpretation, pooled evidence reveals a consistent high prevalence of stigma and discrimination, which act as "toxic" stressors, creating a vicious cycle with psychological stress and psychiatric manifestations and disease activity. There is an urgent clinical need to move beyond a mere biological approach to disease assessment and management and to begin screening for the "invisible" burden of invalidation and discrimination.
BackgroundIn patients with active lupus nephritis (LN), 8 different triple immunosuppressive therapies have been compared to the standard of care (SOC) of double immunosuppressive therapy in 12 induction randomized clinical trials (RCTs). The triple immunosuppressive therapies of SOC+Tacrolimus, SOC+Voclosporin, SOC+Belimumab, and SOC+Obinutuzumab compared to SOC alone demonstrated significantly higher remission of LN without excessive risk of serious adverse events (SAEs) and serious infectious events (SIEs) in RCTs. Direct or indirect comparisons among triple immunosuppressive therapies have not been studied. In this network meta-analysis (NMA), we systematically provide indirect comparisons of the relative efficacy and safety of triple immunosuppressive therapies, ranking them according to efficacy achieving primary renal remission (PRR) of LN and safety reducing the risk of SAEs and SIEs.ResultsThe NMA included predominantly women with LN. In direct pairwise comparisons, SOC+Tacrolimus (benefit ratio 1.91 [95% Credible Interval 1.45 to 2.56]), SOC+Voclosporin (1.69 [1.30 to 2.23]), SOC+Obinutuzumab (1.41 [1.09 to 1.84]), and SOC+Belimumab (1.32 [1.04 to 1.69]) had significantly higher PRR compared to SOC alone. In indirect pairwise comparisons, patients who received SOC+Tacrolimus compared to SOC+Rituximab (2.30 [1.27 to 4.20]), SOC+Anifrolumab (1.98 [1.07 to 3.52]), SOC+Ocrelizumab (1.81 [1.12 to 2.82]), and SOC+Abatacept (1.78 [1.07 to 2.91]) had significantly higher PRR. Patients who received SOC+Voclosporin compared to SOC+Rituximab (2.03 [1.14 to 2.68]) had significantly higher PRR. SOC+Belimumab, SOC+Obinutuzumab, SOC+Voclosporin and SOC+Tacrolimus ranked as effective therapies inducing PRR with SUCRA probability scores between 61.14% and 95.12%. Risk ratios of SAE and SIE were not significantly different among the immunosuppressive therapies.ConclusionIn patients with LN, induction therapies of SOC with calcineurin inhibitors had the highest relative benefit achieving remission of LN.
BackgroundLupus nephritis (LN) is an autoimmune disease that affects kidneys and is considered one of the most prevalent complications of systemic lupus erythematosus (SLE). We screened differentially expressed circRNAs through circRNA sequencing in peripheral blood molecular cells (PBMC) of LN patients and verified that circSMURF2 was downregulated and its correlation with laboratory indicators.Our study reveals for the first time that circSMURF2 was downregulated in LN patients and mediated cell apoptosis in HK2s by modulating DIABLO ubiquitination.MethodsThe expression and function of circSMURF2 were detected by qRT-PCR and FISH. The interactions between circSMURF2 and DIABLO were analyzed by RNA pull-down assay, Mass Spectrometry and RNA Immunoprecipitation. HK2s treated with lentivirus overexpression of circSMURF2 was applied for experiments.ResultFunctionally, circSMURF2 promoted proliferation and inhibited apoptosis in Human renal cortex proximal tubule epithelial cells (HK2). Mechanistically, circRNA pull-down analysis showed that circSMURF2 functions in HK2s by binding to DIABLO protein and negatively regulating its expression through accelerating its ubiquitination.
ObjectivesTo evaluate the diagnostic performance of the SLE Risk Probability Index (SLERPI) in a European cohort and explore its potential prognostic utility for early flare risk stratification.MethodsThis retrospective study included 280 patients with physician-diagnosed SLE and 156 non-SLE controls from a tertiary centre. Diagnostic performance (sensitivity, specificity, and area under the curve [AUC]) of the SLERPI and the 2019 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria were assessed against physician diagnosis as the reference standard. Severe flares within 3 years of diagnosis were defined using the SELENA-SLEDAI Flare Index. Multivariable logistic regression was used to identify predictors of severe flare.ResultsSLERPI demonstrated a higher AUC than the 2019 EULAR/ACR criteria (0.967, 95% CI 0.950-0.984 vs 0.932, 95% CI 0.908-0.957; DeLong p = 0.0004) and greater specificity (97.4% vs 94.9%), whereas sensitivity (85.7% vs 87.1%) and negative predictive value (79.2% vs 80.4%) were slightly lower. Analysis of discordant classifications revealed that the SLERPI captured cutaneous-predominant presentations more effectively, while the EULAR/ACR criteria showed greater sensitivity for serositis-driven disease. Patients who developed a severe flare within 3 years had significantly higher baseline SLERPI scores (p = 0.047). Youden's J statistic identified a threshold of ≥10.2 points as potentially informative for severe flare risk stratification. In multivariable analysis, a model incorporating SLERPI constituent items identified proteinuria and serositis at diagnosis as independent predictors of severe flare (AUC 0.708, 95% CI 0.641-0.775); bootstrap internal validation yielded a lower, optimism-corrected AUC of 0.635 (95% CI 0.574-0.695).ConclusionSLERPI demonstrates high diagnostic accuracy in a single-centre retrospective Central European cohort. Exploratory analyses suggest that higher baseline SLERPI scores may be associated with an increased risk of early severe flares; however, these findings require external validation before prognostic use can be recommended.
BackgroundSystemic lupus erythematosus is a chronic autoimmune disease associated with heightened cardiovascular risk. Data on the impact of systemic lupus erythematosus on outcomes following heart failure hospitalization remain limited. This study aimed to evaluate whether systemic lupus erythematosus is independently associated with 90-days readmission and other clinical outcomes among patients hospitalized with heart failure.MethodsWe conducted a retrospective cohort study using the 2016-2017 Nationwide Readmissions Database to evaluate the association of systemic lupus erythematosus with 90-days readmission after heart failure hospitalization. Adults ≥18 years with an index admission for heart failure were included. The primary outcome was 90-days all-cause readmission. Secondary outcomes included in-hospital mortality, median length of stay, and hospitalization costs. Multivariable Cox proportional hazards were used to identify independent predictors of outcomes.ResultsAmong 1,625,731 patients hospitalized with heart failure, 9096 had comorbid systemic lupus erythematosus. Compared with non-systemic lupus erythematosus patients, those with systemic lupus erythematosus were younger (mean age 61 vs 72 years), predominantly female, and more likely to have socioeconomic disadvantage and a higher comorbidity burden. The 90-days readmission rate was significantly higher in the systemic lupus erythematosus cohort (41%) versus the non-systemic lupus erythematosus cohort (34%) (HR: 1.07; 95% CI: 1.02-1.12; p = 0.010). In-hospital mortality did not differ significantly between groups; however, mortality during readmissions was nearly doubled compared with index admissions (5.4% vs 2.9%). SLE patients had a median length of stay of 4 days (vs 4 days in non-SLE) and incurred median hospitalization costs of USD 32,872 (13% higher than non-SLE patients). Independent predictors of readmission included Medicaid insurance, weekend admission, renal failure, myocardial infarction, and discharge to a non-home setting, whereas female sex, treatment at metropolitan teaching hospitals, and comorbid hypertension or diabetes were associated with a lower risk of readmission.ConclusionSystemic lupus erythematosus is independently associated with an increased risk of 90-days readmission following heart failure hospitalization, contributing to greater healthcare utilization and costs. These findings highlight the need for tailored strategies for transitional care, multidisciplinary follow-up, and socioeconomic support.
Objectives: To investigate the association of serum glutathione peroxidase 4 (GPX4) levels and GPX4 polymorphisms with systemic lupus erythematosus (SLE) and lupus nephritis (LN) in a Chinese population.Methods: This hospital-based case-control study included 28 patients with LN, 28 patients with SLE without renal involvement, and 28 healthy controls for measurement of serum GPX4 levels using enzyme-linked immunosorbent assay (ELISA). In addition, 104 patients with SLE (including 64 with LN and 40 without renal involvement) and 100 healthy controls underwent genotyping of target single-nucleotide polymorphisms (SNPs) by polymerase chain reaction-coupled Sanger sequencing. Multivariable binary logistic regression analyses were performed to adjust for potential confounding factors.Results: Serum GPX4 levels were significantly lower in both the LN and non-LN groups than in the healthy control group, and GPX4 showed moderate diagnostic accuracy for SLE detection. In univariate analyses, carriers of the CC genotype and C allele at rs713041, as well as carriers of the GA genotype and A allele at rs4807542, had a significantly increased risk of SLE compared with healthy controls. Multivariable binary logistic regression further showed that the rs713041 CC genotype and rs4807542 A-carrier status remained independently associated with SLE susceptibility after adjustment for age and sex. Furthermore, in comparisons between LN and non-LN patients, carriers of the CC genotype and C allele at rs713041 were significantly associated with an increased risk of LN in univariate analyses, and the rs713041 CC genotype remained independently associated with renal involvement after adjustment for age, sex, disease duration, and SLEDAI score.Conclusions: Reduced serum GPX4 levels in both LN and non-LN patients suggest that GPX4 may be involved in the pathogenesis of SLE-related renal injury. In addition, GPX4 polymorphisms rs713041 and rs4807542 were independently associated with SLE susceptibility, whereas only rs713041 was independently associated with renal involvement in patients with SLE. These findings suggest that rs713041 may serve as a potential genetic marker of LN susceptibility in the Chinese population.
Background: Giant cell myocarditis (GCM) is a rare, and often fatal subtype of myocarditis, with limited reports of association with systemic lupus erythematosus (SLE). Purpose: We are first to report a unique case of an 18-year-old patient presenting with biventricular heart failure and newly-onset SLE, ultimately diagnosed with GCM. Analysis/Results: The patient had a 6-month prodrome of fatigue, fever, and malaise, followed by acute decompensation. Echocardiography showed a severely reduced left ventricular ejection fraction (5%). Serology confirmed SLE, endomyocardial biopsy identified multinucleated giant cells, confirming GCM. Despite immediate initiation of extracorporeal membrane oxygenation (ECMO) and aggressive immunosuppressive therapy (methylprednisolone, antithymocyte globulin, cyclosporine), cardiac function did not recover. The patient received a biventricular assist device (BiVAD) as intermediate-term bridge-to-transplant. However, significant complications occurred in the form of multiorgan failure, thromboembolism, and hemodynamic deterioration, ultimately leading to death before transplantation was possible. Conclusions: This case underscores the importance of differentiating lupus myocarditis from GCM, given the dramatically different prognosis and treatment strategies. Early differential diagnosis via biopsy is essential, particularly in young patients with rapidly progressing cardiac decline and concurrent autoimmune serologies. Aggressive immunosuppression remains the cornerstone of treatment, attempting to reduce myocardial damage and the resulting need for transplantation. Clinicians should maintain suspicion of GCM in lupus patients with cardiac compromise to initiate timely interventions.
ObjectiveTo develop a standardized nursing protocol for pediatric hypoprothrombinemia-lupus anticoagulant syndrome (HLAS), a rare condition lacking unified clinical nursing guidelines worldwide, aiming to improve nurses' predictive, precise and whole-course care capabilities and optimize long-term clinical outcomes in affected children.MethodsEvidence-based nursing principles were integrated with clinical practice. After the admission and standardized management of a typical index case in March 2025, a multidisciplinary team composed of pediatric nephrology, rheumatology, laboratory medicine and nursing specialists conducted retrospective case analysis and collaborative protocol formulation, with its core innovation being the integration of thromboelastography (TEG) parameters to establish a quantitative bleeding risk stratification model. The systematic literature search covered all mainstream Chinese and English databases from database establishment to March 2025, consistent with the timeline of case admission and protocol development.ResultsApplication of this protocol in the index case enabled early identification of high bleeding risk, effectively avoiding severe bleeding events and nosocomial infections. The standardized health education and targeted psychological intervention significantly improved the disease cognition and family self-management ability of caregivers. It also transformed nurses from passive caregivers to proactive care managers.ConclusionThis protocol fills the gap in specialized nursing guidelines for pediatric HLAS, standardizes clinical nursing practices, and provides a replicable methodological reference for the development of standardized nursing protocols for other rare pediatric diseases.
BackgroundSystemic lupus erythematosus (SLE) features aberrant T-B cooperation and expansion of atypical memory B cells (aMBCs) characterized by the expression of CD11c and T-bet. We investigated the relationship between IL-21/IL-21R and the activation state of cTfh and Tph, with CD11c+T-bet + B cell subsets and clinical activity.MethodsA cross-sectional study was conducted involving 40 patients with systemic lupus erythematosus (SLE) and 15 healthy subjects (HS). A multiparameter flow cytometry was used to evaluate Tph (CD4+CXCR5-PD-1+), cTfh (CD4+CXCR5+PD-1+), and aMBCs (CD19+CXCR5-CD11c+) subsets and intracellular expression of IL-17A (iIL-17A), IL-21 (iIL-21), and T-bet. The disease activity was assessed using the SLEDAI-2K.ResultsWe found an increased frequency of cTfh PD-1vh, HLA-DR+, IL-21R+, and Tph PD-1vh, HLA-DR+, and iIL-21+ cells in SLE patients. The aNAV T-bet+ cells were expanded in SLE patients. Activated T-cell states (iIL-21+/IL-21R+/PD-1vh/HLA-DR+) correlated with T-bet+ B cells subsets. Finally, activated cTfh/Tph and aMBCs correlated with SLEDAI-2K.ConclusionsOur findings provide new insights into the cooperative expression of IL-21/IL-21R and T-bet and their potential relationships with extrafollicular B-cell responses in SLE. These results highlight the IL-21/T-bet axis, offering potential avenues for biomarker development and targeted therapeutic intervention in SLE.
