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Langerhans cells infiltration in lymph nodes of patients with systemic lupus erythematosus. 系统性红斑狼疮患者淋巴结中的朗格汉斯细胞浸润。
IF 3.7 2区 医学
Lupus Science & Medicine Pub Date : 2025-04-14 DOI: 10.1136/lupus-2024-001474
Jinyi Qian, Lei Li, Jing Lv, Yingjie Jiang, Qianchen Ma, Haoyu Pan, Xiaohan Wei, Zhixia Yang, Shuyi Yu, Yuying Fan, Jialin Teng, Chengde Yang, Aifei Zhang, Yue Yang, Hui Shi
{"title":"Langerhans cells infiltration in lymph nodes of patients with systemic lupus erythematosus.","authors":"Jinyi Qian, Lei Li, Jing Lv, Yingjie Jiang, Qianchen Ma, Haoyu Pan, Xiaohan Wei, Zhixia Yang, Shuyi Yu, Yuying Fan, Jialin Teng, Chengde Yang, Aifei Zhang, Yue Yang, Hui Shi","doi":"10.1136/lupus-2024-001474","DOIUrl":"https://doi.org/10.1136/lupus-2024-001474","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to characterise the clinical features and treatment regimens of patients with lupus who have lymphadenopathy (LAP), as well as to investigate the presence and potential implications of Langerhans cells (LCs) infiltration in lymph nodes.</p><p><strong>Methods: </strong>A case-control study was conducted to identify the clinical characteristics of newly diagnosed, treatment-naïve patients with lupus who have LAP. Lymph node biopsies were performed, and LC infiltration was assessed using immunohistochemical staining for S100, CD1a and Langerin.</p><p><strong>Results: </strong>A total of 59 patients with SLE who have LAP (SLE-LAP) were enrolled, with 81 patients with SLE without LAP serving as controls. The SLE-LAP group exhibited significantly higher frequencies of fever (64.4% vs 35.8%, p<0.001), anaemia (71.2% vs 42.0%, p<0.001), serous effusion (27.1% vs 11.1%, p=0.015), myositis (10.2% vs 1.2%, p=0.045) and elevated CRP levels (44.1% vs 22.2%, p=0.006). Moreover, autoantibodies, including anti-Smith (37.3% vs 16.0%, p=0.004), anticardiolipin IgG (27.1% vs 11.1%, p=0.015), IgM (42.4% vs 9.9%, p<0.001) and IgA (8.5% vs 0.0%, p=0.027), were more frequently detected in the LAP group. LC infiltration was confirmed in 29 of the 59 lymph node biopsies (49.2%). Immunohistochemical analysis revealed a scattered (58.6%) or focal (41.4%) distribution of LCs. Patients with LC infiltration predominantly presented with fever (72.4%), anaemia (64.3%), skin rashes (62.1%) and arthritis (62.1%). However, no significant differences in clinical manifestations were observed between LC-positive and LC-negative patients.</p><p><strong>Conclusion: </strong>LC infiltration in the lymph nodes of patients with SLE is relatively common and should be carefully evaluated to prevent misdiagnosis. The role of LCs in the autoimmune response and pathogenesis of SLE warrants further investigation.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11997825/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144031334","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Modern therapy of patients with lupus erythematosus must include appropriate management of their heightened rates of atherosclerotic cardiovascular events: a literature update. 红斑狼疮患者的现代治疗必须包括适当的管理他们的动脉粥样硬化心血管事件的高发率:文献更新。
IF 3.7 2区 医学
Lupus Science & Medicine Pub Date : 2025-04-08 DOI: 10.1136/lupus-2024-001160
Lais Lopes Almeida Gomes, Daniella Forman Faden, Lillian Xie, Shae Chambers, Caroline Stone, Victoria P Werth, Kevin Jon Williams
{"title":"Modern therapy of patients with lupus erythematosus must include appropriate management of their heightened rates of atherosclerotic cardiovascular events: a literature update.","authors":"Lais Lopes Almeida Gomes, Daniella Forman Faden, Lillian Xie, Shae Chambers, Caroline Stone, Victoria P Werth, Kevin Jon Williams","doi":"10.1136/lupus-2024-001160","DOIUrl":"https://doi.org/10.1136/lupus-2024-001160","url":null,"abstract":"<p><p>Atherosclerotic cardiovascular disease (ASCVD) remains the biggest killer of patients with lupus erythematosus (LE) and the general non-autoimmune population. In this literature update on LE and ASCVD, we focused on published work since our earlier review article, meaning from 2021 to the present, with an emphasis on cutaneous LE. Several themes emerged. First, new work shows that patients with lupus still exhibit a high burden of conventional risk factors for ASCVD events. Second, recent studies continue to implicate possible effects of lupus disease activity to worsen rates of ASCVD events beyond predictions from conventional risk factors. Third, new work on estimating the risk of future ASCVD events in patients with lupus supports arterial-wall imaging, inclusion of lupus-specific factors, estimators of ASCVD event risk that take lupus status into account and considering lupus as a diabetes equivalent or even as a diabetes-plus-smoking equivalent in this context. Technologies for arterial-wall imaging continue to improve and will likely play an increasing role in ASCVD assessment and management. Fourth, purported cardiovascular benefits from certain disease-modifying antirheumatic drugs such as antimalarials have become less clear. Fifth, earlier treatment of atherosclerosis, which is a lifelong disease, can be accomplished with diet, exercise, smoking cessation and new classes of safe and effective medications for lipid-lowering and blood pressure control. Benefits on subclinical arterial disease by imaging and on ASCVD events have been reported, supporting the concept that ASCVD is eminently manageable in this autoimmune condition. Sixth, despite the heightened risk for ASCVD events in patients with lupus, available therapeutic approaches remain unused or underused and, accordingly, event rates remain high.Raising awareness among patients and healthcare providers about ASCVD assessment and management in patients with LE is essential. Greater vigilance is needed to prevent ASCVD events in patients with lupus by addressing dyslipidaemias, hypertension, smoking, obesity and physical inactivity.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11979607/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143989108","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Application of machine learning in assessing disease activity in SLE. 机器学习在SLE疾病活动性评估中的应用。
IF 3.7 2区 医学
Lupus Science & Medicine Pub Date : 2025-04-08 DOI: 10.1136/lupus-2024-001456
Yun Wang, Peihong Yuan, Wei Wei, Rujia Chen, Ting Wang, Renren Ouyang, Feng Wang, Hongyan Hou, Shiji Wu
{"title":"Application of machine learning in assessing disease activity in SLE.","authors":"Yun Wang, Peihong Yuan, Wei Wei, Rujia Chen, Ting Wang, Renren Ouyang, Feng Wang, Hongyan Hou, Shiji Wu","doi":"10.1136/lupus-2024-001456","DOIUrl":"https://doi.org/10.1136/lupus-2024-001456","url":null,"abstract":"<p><strong>Objective: </strong>SLE is a chronic autoimmune disease with immune complex deposition in various organs, causing inflammation. The Systemic Lupus Erythematosus Disease Activity Index 2000 assesses disease severity but is subjective. This study aimed to construct a machine learning model based on objective laboratory indicators to assess SLE disease activity.</p><p><strong>Methods: </strong>A retrospective study was conducted on 319 patients with SLE, collecting their clinical characteristics and laboratory indicators as model-building indicators. Multiple machine learning algorithms were employed to construct models for assessing SLE disease activity.</p><p><strong>Results: </strong>The patients were divided into two cohorts, cohort 1 used as the training set to build the machine learning models and cohort 2 for external validation. Six laboratory indicators, including anti-dsDNA (IFT), quantitative anti-dsDNA, neutrophils, globulin, proteinuria and NK cells, were selected to construct the SLE disease activity evaluation model. The XGBoost model demonstrated superior performance in distinguishing active SLE, with an area under the receiver operating characteristic curve of 0.934, accuracy of 0.925, sensitivity of 0.969, specificity of 0.750 and F1 score of 0.954.</p><p><strong>Conclusions: </strong>This pioneering machine learning model, using objective laboratory indicators, enhances clinical feasibility and provides a novel method for assessing SLE disease activity, that may enable timely evaluation of SLE activity, facilitating preparation for treatment and prognosis.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11979605/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144027515","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Comparison of TLR4, NF-κB and IRF3 expression in kidney tissue between lupus nephritis (LN) and systemic lupus erythematosus (SLE): a pristane-induced lupus mice model study. 狼疮性肾炎(LN)和系统性红斑狼疮(SLE)小鼠肾组织TLR4、NF-κB和IRF3表达的比较:前列腺素诱导狼疮小鼠模型研究
IF 3.7 2区 医学
Lupus Science & Medicine Pub Date : 2025-04-07 DOI: 10.1136/lupus-2024-001445
Yuswanto Setyawan, Hani Susianti, Nur Samsu, Loeki Enggar Fitri
{"title":"Comparison of TLR4, NF-κB and IRF3 expression in kidney tissue between lupus nephritis (LN) and systemic lupus erythematosus (SLE): a pristane-induced lupus mice model study.","authors":"Yuswanto Setyawan, Hani Susianti, Nur Samsu, Loeki Enggar Fitri","doi":"10.1136/lupus-2024-001445","DOIUrl":"10.1136/lupus-2024-001445","url":null,"abstract":"<p><strong>Introduction and purpose: </strong>Lupus nephritis (LN) is a major cause of morbidity and mortality in patients with SLE, a complex autoimmune disease characterised by loss of tolerance to self-nuclear antigens. Toll-like receptor 4 (TLR4), the first line of defence in the innate immune system, has been linked to the pathogenesis of autoimmune diseases and LN by activating nuclear factor-κB (NF-κB) or interferon regulatory transcription factor 3 (IRF3). Local expression of those biomarkers in pristane-induced lupus mice is still unknown. Therefore, this study aimed to prove the role of TLR4, NF-κB and IRF3 in pristane-induced lupus mice.</p><p><strong>Subjects and methods: </strong>The study subjects were female Balb/c pristane-induced lupus mice model, 8-12 weeks of age, n=30, divided into two groups, nephritis (LN group) and non-nephritis (SLE group). The control group were age-matched healthy female Balb/c mice, n=11. All mice were euthanised at weeks 16. Kidney tissue was taken for histopathology examination and TLR4, NF-κB, IRF3 immunofluorescence assay. The diagnosis of LN was based on proteinuria and histopathology examination according to the ISN/RPS 2004 classification of LN. Statistical analysis was performed using IBM SPSS Statistics V.25. P value <0.05 was considered statistically significant.</p><p><strong>Results: </strong>There were significant differences in the expressions of TLR4, NF-κB and IRF3 among the LN, SLE and healthy control groups (p=0.000), with the highest expression found in the LN group for all markers. The linear regression between TLR4 and NF-κB resulted in p value=0.000; R<sup>2</sup>=0.817; β=0.904. Linear regression between TLR4 and IRF3 showed p value=0.000; R<sup>2</sup>=0.896; β=0.947, which means TLR4 had an 81.7% effect on NF-κB and 89.6% on IRF3 expression.</p><p><strong>Conclusion: </strong>TLR4, NF-κB and IRF3 expression were increased in lupus, with the highest expression found in the LN group, suggesting that these biomarkers may be responsible for the development of nephritis in SLE, with TLR4 likely playing a dominant role in this pathway. Increased expression of these biomarkers in lupus without nephritis may indicate progression towards nephritis, which still needs to be proven with further research.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11977469/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143811743","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
IgG glycosylation profiling of systemic lupus erythematosus using lectin microarray. 利用凝集素芯片分析系统性红斑狼疮IgG糖基化特征。
IF 3.7 2区 医学
Lupus Science & Medicine Pub Date : 2025-04-05 DOI: 10.1136/lupus-2024-001413
Yang Wu, Minhui Wang, Chaojun Hu, Shangzhu Zhang, Jiuliang Zhao, Qian Wang, Dong Xu, Xinping Tian, Yan Zhao, Xiaofeng Zeng, Mengtao Li
{"title":"IgG glycosylation profiling of systemic lupus erythematosus using lectin microarray.","authors":"Yang Wu, Minhui Wang, Chaojun Hu, Shangzhu Zhang, Jiuliang Zhao, Qian Wang, Dong Xu, Xinping Tian, Yan Zhao, Xiaofeng Zeng, Mengtao Li","doi":"10.1136/lupus-2024-001413","DOIUrl":"10.1136/lupus-2024-001413","url":null,"abstract":"<p><strong>Objectives: </strong>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.</p><p><strong>Methods: </strong>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).</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11973783/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143788422","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Prevalence and correlates of restricted community mobility in a population-based cohort of adults with systemic lupus erythematosus. 在以人群为基础的系统性红斑狼疮成人队列中,流行率和社区活动受限的相关因素。
IF 3.7 2区 医学
Lupus Science & Medicine Pub Date : 2025-03-28 DOI: 10.1136/lupus-2024-001430
Leila Milanfar, Christopher Barrett Bowling, Courtney Hoge, Amanda Eudy, Patricia Katz, Jinoos Yazdany, Laura Plantinga
{"title":"Prevalence and correlates of restricted community mobility in a population-based cohort of adults with systemic lupus erythematosus.","authors":"Leila Milanfar, Christopher Barrett Bowling, Courtney Hoge, Amanda Eudy, Patricia Katz, Jinoos Yazdany, Laura Plantinga","doi":"10.1136/lupus-2024-001430","DOIUrl":"10.1136/lupus-2024-001430","url":null,"abstract":"<p><strong>Objective: </strong>Restrictions in community mobility, defined as the frequency of and help required to travel to 'life-spaces' (bedroom, home, yard, neighbourhood and town), are associated with poor outcomes among older adults. We aimed to describe and explore factors associated with community mobility among adults with SLE.</p><p><strong>Methods: </strong>We assessed community mobility cross-sectionally in a population-based SLE cohort (October 2019 to May 2022), using the University of Alabama Birmingham Study of Aging Life-Space Assessment (UAB LSA) (score range, 0-120; higher scores=greater community mobility). Community mobility was considered to be restricted if the individual reported not reaching the neighbourhood life-space or beyond at least weekly and without help. Estimated percentages (95% CIs) with restricted community mobility were assessed with multivariable logistic regression adjusting for demographics and disease activity and damage.</p><p><strong>Results: </strong>Among 447 participants (91.7% women; 82.6% Black; mean age 46.2; mean UAB LSA score 53.6), 41.6% had restricted community mobility. After adjustment, Black versus White race (43.4% (95% CI 38.5% to 48.2%) vs 24.4% (12.7% to 36.2%)), lowest versus highest educational attainment (51.1% (41.4% to 60.7%) vs 27.2% (20.7% to 33.6%)) and higher versus lower disease activity (55.2% (48.4% to 62.0%) vs 28.5% (22.9% to 34.3%)) were associated with a higher prevalence of restricted community mobility; there were no differences by age, sex or disease damage.</p><p><strong>Conclusion: </strong>Restricted community mobility was common among adults with SLE, and Black race, lower education and high disease activity were associated with more restricted community mobility. Further research to understand the association of community mobility with outcomes and implement strategies to improve community mobility in people with SLE is warranted.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11956307/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143743626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Using patient-reported measures to predict hospitalisation in a population-based lupus cohort. 使用患者报告的措施来预测基于人群的狼疮队列的住院情况。
IF 3.7 2区 医学
Lupus Science & Medicine Pub Date : 2025-03-23 DOI: 10.1136/lupus-2024-001406
Sung Sam Lim, Sandra Sze-Jung Wu, Ryan Ross, Gaobin Bao, Megan Richards, Liisa Palmer, Gary Bryant
{"title":"Using patient-reported measures to predict hospitalisation in a population-based lupus cohort.","authors":"Sung Sam Lim, Sandra Sze-Jung Wu, Ryan Ross, Gaobin Bao, Megan Richards, Liisa Palmer, Gary Bryant","doi":"10.1136/lupus-2024-001406","DOIUrl":"10.1136/lupus-2024-001406","url":null,"abstract":"<p><strong>Objective: </strong>SLE is a multisystem autoimmune disease where periods of disease activity, often difficult to predict, can cause irreversible disease damage. This study aimed to develop a patient-centric predictive model using real-world data that can identify patients with SLE at a higher risk of hospitalisation compared with the general SLE population.</p><p><strong>Methods: </strong>This observational, retrospective analysis used data from the Georgians Organized Against Lupus (GOAL) cohort from 2011 to 2013. The GOAL cohort is a population-based SLE cohort that collects yearly self-report surveys covering participants' sociodemographic characteristics, clinical characteristics and perceived SLE symptoms (using the Systemic Lupus Activity Questionnaire (SLAQ)). GOAL data were linked to the Georgia Hospital Discharge Database to collect participants' all-cause hospitalisation events in the 6 months following survey completion. A two-step approach was used to predict all-cause hospitalisations-logistic regressions selected a list of GOAL predictors that were subsequently included in the classification and regression tree (CART) models to generate patient subsets based on estimated hospitalisation rates.</p><p><strong>Results: </strong>There were 846 participants who completed 1486 surveys. Participants who were hospitalised within 6 months after survey completion were more likely to be younger, living in poverty and have more reported SLE symptoms than participants without a hospitalisation. CART modelling identified participants who reported any weight loss without trying, severe fatigue and Raynaud's symptoms as most likely to have an all-cause hospitalisation: one in three (34%) patients in this subset were hospitalised in the 6 months following survey completion, 2.6-fold the hospitalisation rates of the overall GOAL cohort (13%) and 6.8-fold the rate in the subset with the lowest hospitalisation rate (5%).</p><p><strong>Conclusions: </strong>This study suggests that patient-reported SLE symptoms and disease activity, specifically certain components of the SLAQ, may be of value in SLE risk management when considering hospitalisation reduction as a treatment goal.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931938/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143692519","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Ras-MAPK pathway in patients with lupus nephritis. 狼疮性肾炎患者的 Ras-MAPK 通路。
IF 3.7 2区 医学
Lupus Science & Medicine Pub Date : 2025-03-18 DOI: 10.1136/lupus-2024-001345
Changming Zhang, Xiaoman Jing, Yangyang Zhang, Ying Jin, Xingjian Gao, Jingxian Yu, Dandan Liang, Jiahui Zhang, Qing Zhong, Haitao Zhang, Zhihong Liu
{"title":"Ras-MAPK pathway in patients with lupus nephritis.","authors":"Changming Zhang, Xiaoman Jing, Yangyang Zhang, Ying Jin, Xingjian Gao, Jingxian Yu, Dandan Liang, Jiahui Zhang, Qing Zhong, Haitao Zhang, Zhihong Liu","doi":"10.1136/lupus-2024-001345","DOIUrl":"10.1136/lupus-2024-001345","url":null,"abstract":"<p><strong>Background: </strong>Pathogenic mutations in genes encoding components of the Ras/mitogen-activated protein kinase (Ras-MAPK) pathway cause RASopathy. Here, we describe five unrelated patients with SLE carrying mutations associated with RASopathy and investigate the activity of the Ras-MAPK pathway.</p><p><strong>Methods: </strong>Pathogenic variants were identified by whole-exome/whole-genome sequencing. The activity of the Ras-MAPK pathway in peripheral blood mononuclear cells (PBMC) and kidneys was evaluated using RNA sequencing and datasets from the nephroseq database, respectively.</p><p><strong>Results: </strong>Five (likely) pathogenic variants in four Ras-MAPK genes were identified, including NRAS: c.G38A: p.G13D; ARAF: c.C1435T: p.R479C; KRAS: c.T341C: p.V114A; PTPN11: c.G455A: p.R152H and NRAS: c.G34A: p.G12S. Kidney injury is the main feature, presenting with nephrotic syndrome (2/5), proteinuria and haematuria (2/5). Acute kidney injury and rapidly progressive nephritic syndrome were noted in one patient each. Other clinical features included mucocutaneous lesions (5/5), cardiac involvement (4/5) and arthralgia (3/5). Laboratory abnormalities included hypocomplementaemia (5/5), presence of antiphospholipid antibodies (4/5), decreased regulatory T cells (3/3), pancytopenia (3/5) and persistent monocytosis (2/5). Kidney biopsy revealed lupus nephritis. Most patients responded well to standard therapy, with the exception of the patient with the NRAS p.G13D mutation who died. The Ras-MAPK pathway was activated in both PBMC and kidney of patients with LN as indicated by increased expression of NRAS, KRAS, RIT1, MRAS, PPP1CB, SHOC2, SOS2 and MAP2K1, as well as decreased expression of negative regulators of the Ras-MAPK pathway, CBL, LZTR1 and NF1.</p><p><strong>Conclusion: </strong>Kidney involvement may be the main feature of the clinical spectrum of RASopathy. Genetic screening should be considered for patients with early onset lupus.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11934627/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143657655","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Levels of physical activity in a large international cohort of patients with systemic lupus erythematosus. 系统性红斑狼疮患者大型国际队列的身体活动水平。
IF 3.7 2区 医学
Lupus Science & Medicine Pub Date : 2025-03-15 DOI: 10.1136/lupus-2024-001443
Timothée Mischler, Lou Kawka, Juan C Sarmiento-Monroy, Philippe Mertz, Luc Pijnenburg, Marina Rinagel, Manuel Francisco Ugarte-Gil, Sophie Geneton, Julien Blaess, Matteo Piga, Christelle Sordet, Laurent Arnaud
{"title":"Levels of physical activity in a large international cohort of patients with systemic lupus erythematosus.","authors":"Timothée Mischler, Lou Kawka, Juan C Sarmiento-Monroy, Philippe Mertz, Luc Pijnenburg, Marina Rinagel, Manuel Francisco Ugarte-Gil, Sophie Geneton, Julien Blaess, Matteo Piga, Christelle Sordet, Laurent Arnaud","doi":"10.1136/lupus-2024-001443","DOIUrl":"10.1136/lupus-2024-001443","url":null,"abstract":"<p><strong>Introduction: </strong>Physical activity (PA) holds a pivotal role in the improvement of mental health or depressive symptoms, as well as in the prevention of cardiovascular diseases (CVDs). Patients with SLE are exposed to an increased risk of CVDs and suffer from deteriorated quality of life compared with the general population. The aim of this study was to assess PA level and characteristics in a large international cohort of patients with SLE.</p><p><strong>Methods: </strong>PA was assessed in metabolic equivalent of tasks (METs) using the International Physical Activity Questionnaire (IPAQ) and classified into three levels: low, moderate and high PA. Other data such as fatigue, disease activity, pain, insomnia, anxiety, depression, stress and fibromyalgia were collected using validated patient-reported instruments, using the Lupus Expert system for the Assessment of Fatigue (LEAF) digital tool.</p><p><strong>Results: </strong>1029 LEAF participants with SLE (986 (95.8%) women) with a median age of 43 years were analysed. The median physical expenditure was 936 METs/week (IQR: 297-2622). 456 (44.3%) participants were classified as having low PA levels. Increased fatigue according to the Functional Assessment of Chronic Illness Therapy-Fatigue Scale (p<0.0001), the Multidimensional Fatigue Inventory (p<0.0001), Visual Analogue Scale for fatigue (p=0.02), pain (p=0.009), depression (p=0.02) and stress (p<0.0001) were significantly more prevalent in less active patients, in IPAQ classification.</p><p><strong>Conclusion: </strong>In this large international study, more than 40% of patients with SLE were not active enough. We found an inverse association between PA levels and fatigue, pain, stress or depression. This points out the necessity to better assess PA in patients with SLE, as well as the aforementioned comorbidities to improve quality of life and reduce cardiovascular risk.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11911810/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143639678","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Metabolomic analysis suggests thiamine monophosphate as a potential marker for mesenchymal stem cell transplantation outcomes in patients with SLE. 代谢组学分析表明,单磷酸硫胺素是SLE患者间充质干细胞移植结果的潜在标志物。
IF 3.7 2区 医学
Lupus Science & Medicine Pub Date : 2025-03-12 DOI: 10.1136/lupus-2024-001197
Xiaoman Jiang, Zhuoyang Jia, Bin Yang, Xiaojun Tang, Xuebing Feng, Lingyun Sun
{"title":"Metabolomic analysis suggests thiamine monophosphate as a potential marker for mesenchymal stem cell transplantation outcomes in patients with SLE.","authors":"Xiaoman Jiang, Zhuoyang Jia, Bin Yang, Xiaojun Tang, Xuebing Feng, Lingyun Sun","doi":"10.1136/lupus-2024-001197","DOIUrl":"10.1136/lupus-2024-001197","url":null,"abstract":"<p><strong>Objective: </strong>The objective of this research is to identify metabolic markers associated with successful treatment by evaluating the effect of mesenchymal stem cell transplantation (MSCT) on the metabolic profiles of patients with SLE.</p><p><strong>Methods: </strong>Plasma samples were collected from 20 patients with SLE before and after MSCT. Principal component analysis (PCA) was used to distinguish pretreatment and post-treatment groups and pathway analysis for identifying involved metabolic pathways. Clinical variables were monitored with a median follow-up time of 180 days. Pearson correlation and receiver operating characteristics (ROC) analysis were employed to associate metabolite changes with clinical outcomes and to predict treatment success.</p><p><strong>Results: </strong>We detected 18 121 metabolites, with 1152 showing significant changes post-treatment, which could be clearly distinguished between pretreatment and post-treatment groups through PCA. Pathway analysis indicated involvement in riboflavin and thiamine metabolism. Clinical improvements were observed at a median follow-up time of 180 days after MSCT, including decreased SLE Disease Activity Index scores, urine protein/creatinine ratios, and erythrocyte sedimentation rates, along with increased levels of complement C3 and C4, haemoglobin, and platelets. Pearson correlation indicated that specific metabolite changes were associated with clinical improvements, particularly increases in thiamine monophosphate (TMP) and asiaticoside levels. ROC analysis identified TMP level changes as the most predictive of treatment success, with a 35% increase indicating a good response to MSCT.</p><p><strong>Conclusion: </strong>This study concludes that TMP is a potential biomarker that can predict the efficacy of MSCT in treating SLE, providing valuable insights for clinical practice and further research.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11907080/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143615823","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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