Lupus最新文献

{"title":"\"Long COVID in patients with systemic lupus erythematosus: A case‒control study\"-a methodological consideration.","authors":"Mustafa Enes Özden, Mithat Temizer","doi":"10.1177/09612033251352722","DOIUrl":"10.1177/09612033251352722","url":null,"abstract":"","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"982-983"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144310128","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Macrophage activation syndrome associated with neonatal lupus erythematosus.","authors":"Yuko Hayashi, Maho Hatano, Shuya Kaneko, Asami Shimbo, Hitoshi Irabu, Masaaki Mori, Masaki Shimizu","doi":"10.1177/09612033251346372","DOIUrl":"10.1177/09612033251346372","url":null,"abstract":"<p><p>Neonatal lupus erythematosus (NLE) is a multi-organ systemic inflammatory disease caused by passively transmitted maternal autoantibodies including anti-SSA/Ro antibody. NLE is clinically characterized by skin symptoms, atrioventricular block, liver dysfunction, and cytopenia. In addition, NLE can cause fever and is a rare cause for fever of unknown origin in the neonatal period. Furthermore, although rare, macrophage activation syndrome (MAS) can complicate NLE. However, the pathogenesis of MAS associated with NLE is still unknown. We report a case of NLE-associated MAS along with the results of serum cytokine profile analysis. A feature of the serum cytokine profile at the onset of disease in our patient was significant increase of serum IL-6 and sTNF-RII levels. Furthermore, in MAS phase, serum sTNF-RII level was massively increased and serum IFN-α level was persistently elevated. MAS complicating NLE might have similar \"pathophysiology to MAS secondary to systemic lupus erythematosus.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"978-981"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144128115","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Esculentoside A alleviates lupus nephritis by modulating MAIT cells in MRL/lpr mice.","authors":"Xing Wang, Xiaoxue Weng, Xianghui Zhang, Ting Zhao, Xianggui Zhang, Jieyin Tang","doi":"10.1177/09612033251348151","DOIUrl":"10.1177/09612033251348151","url":null,"abstract":"<p><p>IntroductionThis study aims to investigate the protective effect of esculentoside A (EsA) on lupus nephritis (LN) by modulating the mucosal-associated invariant T (MAIT) cells and associated cytokines in the renal tissue of MRL/lpr mice.MethodsEighteen 16-week-old female MRL/lpr mice were randomly assigned into the model and EsA groups (9 mice each), and 9 age-matched female Balb/c mice were included as a normal group. Mice were administered 0.2 mL of EsA solution (2.5 mg/mL) or RPMI 1640 medium (for control groups) via intraperitoneal injection once daily for 4 weeks. Urine protein/creatinine ratio (URCR) and blood creatinine (Cr) concentration were measured, Hematoxylin-eosin and Masson staining of renal tissues were performed, and the \"Austin\" acute index (AI) system for LN was determined and the protein expression of TNF-α, IFN-γ, IL-2, and IL-17 were assessed by Western blot. The proportion of MAIT cells in kidney tissues was analyzed using flow cytometry. Pearson correlation analysis was performed between renal MAIT cells and the expression of TNF-α, IFN-γ, IL-2, and IL-17 proteins in kidney tissues. Data were analyzed with SPSS 26.0 software.ResultsCompared with the normal group, the model group exhibited significantly elevated levels of Cr, UCPR, renal expression of TNF-α, IFN-γ, and IL-17, as well as an increased proportion of MAIT cells, along with the most severe renal histopathological damage and the highest AI score. In contrast, the EsA group showed a notable reduction in these parameters, although they remained elevated compared to the normal group. Kidney IL-2 expression was significantly lower in the model group than in the normal group, while EsA treatment resulted in an increase in IL-2 levels. Renal MAIT cells were positively correlated with TNF-α, IFN-γ, and IL-17 expression but negatively correlated with IL-2.DiscussionEsA confers protection against LN in MRL/lpr mice by downregulating the proportion of MAIT cells in kidney tissue, reducing the expression of pro-inflammatory cytokines (TNF-α, IFN-γ, IL-17), and increasing IL-2 expression.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"912-921"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144225853","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sirtuin-1 in systemic lupus erythematosus: Frequency of keratoconjunctivitis sicca and relation to disease activity.","authors":"Eman Ali Abdallah, Khaled Mohamed Amin Elzorkany, Dina Salem, Asmaa Elshemy, Manar Dawoud, Noha Khirat, Azza Gomaa, Dina Alsharaky","doi":"10.1177/09612033251349458","DOIUrl":"10.1177/09612033251349458","url":null,"abstract":"<p><p>BackgroundSystemic lupus erythematosus (SLE) is a complex autoimmune disorder characterized by the production of various autoantibodies and involvement of multiple organ systems, including the eyes. Growing evidence suggests that dysregulation of proteins from the sirtuin family, particularly sirtuin-1 (SIRT1), may play a crucial role in the pathophysiology of SLE, despite its incomplete understanding.ObjectiveTo evaluate SIRT1 levels and study potential association with keratoconjunctivitis sicca (KCS) in SLE patients in relation to disease activity.MethodsA cross-sectional study was conducted on 42 SLE adult patients and 42 healthy controls. Serum SIRT1 levels were measured using enzyme-linked immunosorbent assay. Disease activity was assessed using the SLE Disease Activity Index (SLEDAI) score. Correlations between SIRT1 levels, KCS and disease activity, were analyzed.ResultsPatients with SLE had significantly higher SIRT1 levels than healthy controls (<i>p</i> <  .001). SIRT1 was strongly correlated with disease activity (r = 0.958, <i>p</i> < .001), and albuminuria (r = 0.719, <i>p</i> < .001). Negative correlations were observed between SIRT1 levels and C3 (r = -0.843, <i>p</i> < .001), C4 (r = -0.861, <i>p</i> < .001), white blood cell count (r = -0.616, <i>p</i> < .001) and platelet count (r = -0.534, <i>p</i> < .001). Additionally, Patients with SLE who presented with KCS had significantly higher SIRT1 levels than those without (<i>p</i> < .001). SIRT1 exhibited exceptional diagnostic efficacy in differentiating between Patients with SLE and control subjects, with an AUC of 1 (<i>p</i> < .001) and in identifying KCS in SLE patients, with an AUC of 0.989 (<i>p</i> < .001). The multivariable regression model indicated that a lower platelets count (<i>p</i> = .034; OR -0.014; 95% CI -0.026 to -0.001) and high SIRT1 levels (<i>p</i> < .001; OR 0.838; 95% CI 0.678-0.998) were independent predictors of disease activity.ConclusionsSerum SIRT1 levels were significantly elevated in patients with SLE and correlated with the presence of KCS and disease activity. SIRT1 may serve as a potential biomarker of eye involvement in SLE, the disease pathogenesis and severity, and its modulation could offer a therapeutic target for the disease.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"932-942"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144326185","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Application of Bayesian statistics to support approval of intravenous belimumab in children with systemic lupus erythematosus in the United States.","authors":"Ginto Pottackal, James Travis, Lei Nie, Rebecca Chiu, Rosemarie Neuner, Gregory Levin, Jing Niu, Anshu Marathe, Lily Yeruk Mulugeta, Nikolay P Nikolov","doi":"10.1177/09612033251349930","DOIUrl":"10.1177/09612033251349930","url":null,"abstract":"<p><p>ObjectiveDue to the rarity of childhood-onset systemic lupus erythematosus (cSLE), the pediatric study (NCT01649765) conducted to support approval of intravenous (IV) belimumab in that population could not be designed with adequate power for efficacy to make statistical inferences based solely on the pediatric data. Bayesian analyses have been proposed to implement more flexible pediatric extrapolation approaches in similar situations. Here we discuss a post-hoc Bayesian analysis the FDA employed which borrowed information from the phase 3 IV belimumab studies in adults to supplement the efficacy information collected in the pediatric population.MethodsWe used a Bayesian method to estimate the treatment effect in SLE Responder Index (SRI) response in pediatric patients, with a robust mixture prior that was a weighted combination of a skeptical prior that assumed no treatment effect and a meta-analysis of the treatment effects seen in adults. The weighting was based on the degree of belief in the similarity of the disease and treatment effect between pediatric and adult subjects.ResultsThe trial design and conduct, endpoints, key demographic and disease characteristics as well as pharmacokinetics (PK) were similar between the pediatric and adult study populations, which justified borrowing information from across studies. A prior weight of 55% or larger on the adult data provided 95% credible intervals that excluded an odds ratio of one, providing supportive evidence that belimumab is an effective treatment in pediatric patients. These results were further supported by the secondary efficacy endpoints, PK and safety.ConclusionThis approach contributed to FDA's decision to approve belimumab as the first treatment for cSLE. If pre-specified, such an approach may help expedite future clinical development in pediatric rheumatic diseases, and address some of the challenges with conducting trials for rare diseases.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"943-952"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144512118","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A study on the evaluation of left atrial function in lupus nephritis patients using left atrial automated functional imaging technology.","authors":"Jing Zhang, Yanyan Ye, Xu Huang, Yan Ding, Xia Li, Chunquan Zhang","doi":"10.1177/09612033251346342","DOIUrl":"10.1177/09612033251346342","url":null,"abstract":"<p><p>ObjectivesEmploying advanced left atrial automated functional myocardial imaging technology to investigate whether patients with lupus nephritis (LN) experience more severe cardiac impairment compared to extra-renal systemic lupus erythematosus (extra-renal SLE) patients, while also identifying the primary risk factors affecting left atrial reservoir strain (LASR) in LN patients.MethodsRoutine echocardiographic evaluations were performed on patients with lupus nephritis (LN), Extra-renal systemic lupus erythematosus (SLE), and healthy controls. The left atrial automated functional imaging (AFILA) technique was employed to measure LASR, left atrial conduit strain (LASCD), and left atrial contraction strain (LASCT) for all study participants. Demographic characteristics of all participants were collected, along with clinical and laboratory features for the case group.ResultsWe included 42 healthy controls, 91 patients with extra-renal SLE, and 79 patients with LN. LASR, LASCD, and LASCT in the LN group were all significantly lower than those in the extra-renal SLE group and the control group, with the extra-renal SLE group also exhibiting lower values than the control group (LASR: 22.00% vs 27.00% vs 36.00%, <i>p</i> < .001; LASCD: -14.00% vs -17.00% vs -24.00%, <i>p</i> < .001; LASCT: -9.00% vs -10.00% vs -14.00%, <i>p</i> < .001). A multiple regression analysis of LASR in the LN group indicated that triglycerides (TG) were the sole laboratory indicator independently associated with it (β[SE]: -1.025 [0.370], <i>p</i> = .007).ConclusionsThe degree of LA involvement in patients with LN was significantly greater than in those with extra-renal SLE, with TG potentially serving as the only laboratory indicator capable of effectively predicting LASR. Furthermore, AFILA technology demonstrates considerable potential for the early identification of subclinical myocardial damage.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"888-898"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144258384","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association between antiphospholipid syndrome and postural orthostatic tachycardia syndrome (POTS).","authors":"Aashish Batheja, Nehal R Shah","doi":"10.1177/09612033251353082","DOIUrl":"10.1177/09612033251353082","url":null,"abstract":"","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"984-986"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144317327","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epigenetic mechanisms of B cells: Recent advances in targeted therapies for SLE.","authors":"Kunyu Cao, Xiaoping Du, Miao Tu, Yujiao Wei, Liu Wan, Jiyu Ju","doi":"10.1177/09612033251348154","DOIUrl":"10.1177/09612033251348154","url":null,"abstract":"<p><p>Systemic lupus erythematosus (SLE) is a complex autoimmune disease, and abnormal B-cell function plays a key role in its pathogenesis. Multiple epigenetic mechanisms, such as DNA methylation, histone modification, and non-coding RNAs, can regulate B cell development, differentiation, and function. This review outlines the relationship between the epigenetic regulation of B cells and the pathogenesis of SLE and prospectively discusses possible therapeutic approaches.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"879-887"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144225852","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A study of calcineurin inhibitors in comparison with intravenous cyclophosphamide for initial induction treatment of lupus nephritis class Ⅲ, Ⅳ, V in Chinese children.","authors":"Yanxinli Han, Hongyu Sha, Lanqi Zhou, Yuan Yang, Yi Wang, Zhuowei Yu, Liru Qiu, Yu Zhang, Jianhua Zhou","doi":"10.1177/09612033251351338","DOIUrl":"10.1177/09612033251351338","url":null,"abstract":"<p><p>BackgroundLupus nephritis (LN) is one of the most common secondary glomerulonephritis in children, and may progress to end-stage kidney disease if remission induction is not successful. Currently, the recommended induction treatment for lupus nephritis class Ⅲ/Ⅳ/V are corticosteroids combined with cyclophosphamide (CYC). Although tacrolimus has demonstrated comparable efficacy to CYC in adult patients with LN, its effectiveness and safety in the treatment of childhood lupus nephritis (cLN) have not been extensively investigated, particularly in comparison to cyclophosphamide (CYC). The objective of this study is to retrospectively assess the effect of calcineurin inhibitors (CNIs) compared to CYC as the initial induction therapy for class Ⅲ/Ⅳ/Ⅴ cLN.MethodscLN of class Ⅲ/Ⅳ/V treated initially with intravenous CYC or CNIs from January 2009 to January 2022 were included. The collected clinical, pathological, and treatment data were analyzed to assess and compare the efficacy and safety of CNIs versus CYC.ResultsA total of 75 LN patients were eligible. 46 patients received corticosteroids combined with intravenous CYC for induction remission, while 29 patients with oral CNIs, mainly tacrolimus. The CNI group had a significantly higher complete remission (CR) rate (55.2%) compared to the CYC group (17.4%) after 3 months of treatment, and this difference remained at 6 months (62.1% vs 26.1%). The reduction of proteinuria was also more significant in the CNI group compared to the CYC group at 3-month induction. The overall incidence of adverse effects was significantly lower in CNI than in CYC (17% vs 63%, <i>p</i> = 0.032). Multiple logistic regression analysis identified edema, anti-dsDNA, and CYC as risk factors for non-remission after 6-month induction therapy.ConclusionsThis study demonstrates that CNIs are safer and more effective than CYC for quickly reducing proteinuria and achieving higher CR rate in initial induction treatment for lupus nephritis class III/IV/V.Clinical Trial RegistrationChiCTR2300075587.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"961-971"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144326183","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association of truncal involvement in lupus erythematosus panniculitis patients with calcinosis cutis: A retrospective cohort study.","authors":"Jay Garza, Benjamin F Chong","doi":"10.1177/09612033251349451","DOIUrl":"10.1177/09612033251349451","url":null,"abstract":"<p><p>BackgroundLupus erythematosus panniculitis (LEP) is a rare variant of cutaneous lupus erythematosus that typically presents as indurated nodules or plaques. Calcinosis cutis (CC) is a potential complication of this disease with limited treatment modalities and significant quality of life complications. The rate and risk factors of CC in LEP are not well understood. Thus, we conducted a retrospective cohort study on patients diagnosed with LEP.ObjectiveTo quantify the rate of CC and to identify the risk factors associated with its development in LEP.MethodsThis retrospective cohort study analyzed data from 27 LEP patients recruited in outpatient dermatology clinics at University of Texas Southwestern Medical Center and Parkland Health from January 2009 to December 2024. The primary outcome measure was CC development based on clinical diagnosis from a dermatologist, biopsy, or radiographic imaging. Data collected included demographics, smoking history, disease duration, medications, and lesion location. Predictor variables associated with CC development were analyzed either by Mann-Whitney U or Fisher's exact tests.Results10/27 (37%) LEP patients had CC during the evaluation period. LEP patients with CC had a higher rate of truncal involvement (9/10 (90%) versus 7/17 (41.2%); <i>p</i> = .02) and a lower rate of head & neck involvement (3/10 (30%) versus 13/17 (76.5%); <i>p</i> = .04) of their LEP lesions compared to those without CC.LimitationsThis study is limited by its single-center design, retrospective nature, and small sample size.ConclusionsThis cohort of LEP patients had over a third developing CC. LEP lesion location significantly differed in those who developed CC compared with those who did not. CC is a common complication of LEP that requires close monitoring by clinicians. Prospective multicenter studies are needed to confirm these findings and better understand the predictive factors for the development of CC in LEP patients.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"972-977"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12227800/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144248539","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}