Lupus Science & Medicine最新文献

{"title":"Anti-CD20 alone as maintenance immunosuppressive therapy in severe lupus nephritis: a promising experience that needs to be evaluated.","authors":"Sébastien Boutinet, Ludivine Lebourg, Stanislas Faguer, Christophe Richez, Noémie Jourde-Chiche, Eric Daugas","doi":"10.1136/lupus-2025-001509","DOIUrl":"https://doi.org/10.1136/lupus-2025-001509","url":null,"abstract":"","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12067818/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144016026","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}

{"title":"Domains for inclusion in a novel Treatment Response Measure for Systemic Lupus Erythematosus (TRM-SLE): results of a modified Delphi study.","authors":"Kathryn Connelly, Rachel Koelmeyer, Darshini Ayton, John May, Kate Gregory, Laura E Eades, Raychel Barallon, Rangi Kandane-Rathnayake, Vera Golder, Afia Anzum, Maisarah Mydin, Munni Akther, Alan Friedman, Anca D Askanase, Cynthia Aranow, Edward Vital, Guillermo Pons-Estel, Hermine Brunner, Kenneth Kalunian, Khadija Dantata, Laurent Arnaud, Laurie Burke, Lee S Simon, Qing Zuraw, Sandra Garces, Victoria P Werth, Ying B Sun, Yoshiya Tanaka, Youmna Lahoud, Alain Cornet, Alessandro Sorrentino, Anisur Rahman, Anna Stevens, Catherine Barbey, Ida Dzifa Dey, Elaine Karis, Eloisa Bonfá, Erika Noss, Eve M D Smith, George Stojan, Jeanette Andersen, Joseph F Merola, Jorge A Ross Terres, Joy Buie, Justine Maller, Marta Mosca, Maja Hojnik, Maria Dall'Era, Richard A Furie, Ronald F van Vollenhoven, Subhashis Banerjee, Eric Morand","doi":"10.1136/lupus-2024-001484","DOIUrl":"10.1136/lupus-2024-001484","url":null,"abstract":"<p><strong>Objectives: </strong>To achieve consensus on domains of active disease for inclusion in a novel outcome measure for SLE randomised controlled trials (RCTs), the Treatment Response Measure for SLE (TRM-SLE).</p><p><strong>Methods: </strong>Domains nominated by TRM-SLE Taskforce members were rated in a two-stage modified Delphi study. Each stage comprised two online survey rounds separated by a structured discussion meeting. In Stage 1, expert lupus clinicians and patient representatives rated domain 'importance' (impact on symptoms, function or survival). In Stage 2, clinicians rated 'important' domains on three characteristics relevant to RCT utility: 'appropriateness' for evaluating change in disease activity, 'representation' in patients with active SLE and 'measurability' in an RCT context. Consensus for domain inclusion was prespecified as all four characteristics achieving a rating ≥7 on a 1-9 scale by ≥70% of participants.</p><p><strong>Results: </strong>Domain nominations from 36/59 (61%) TRM-SLE Taskforce members yielded 34 potential domains which were rated in the modified Delphi study. At least one Delphi round was completed by 87 clinicians and 13 patient representatives. In Stage 1, 14 domains met consensus on 'importance' in both clinician and patient groups, and 11 domains met consensus among patients only. After Stage 2, eight of these domains also reached consensus on 'appropriateness', 'representation' and 'measurability': alopecia, arthritis, haemolytic anaemia, nephritis, mucosal ulcers, rash, serositis and thrombocytopenia.</p><p><strong>Conclusions: </strong>Considering patient and clinician perspectives, we reached consensus to include eight disease activity domains for future development into the novel TRM-SLE clinical trial outcome measure, aiming to improve trial interpretability and success.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.5,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12067843/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143990381","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}

{"title":"Adverse pregnancy outcomes across SLE subgroups: significance of cardiovascular events.","authors":"Rashmi Dhital, Rebecca J Baer, Kenneth Kalunian, Christina Chambers","doi":"10.1136/lupus-2025-001507","DOIUrl":"https://doi.org/10.1136/lupus-2025-001507","url":null,"abstract":"<p><strong>Objective: </strong>SLE is associated with increased risks of maternal cardiovascular events (CVEs) as well as adverse pregnancy outcomes. The influence of maternal CVEs on pregnancy complications in lupus is not clearly known. Our primary aim was to assess the risks of adverse pregnancy outcomes in individuals with SLE, specifically examining the influence of CVEs.</p><p><strong>Methods: </strong>Using a California population-based birth cohort from 2005 to 2020, pregnant individuals with SLE were identified via International Classification of Diseases codes on maternal discharge records and further subdivided based on whether they had lupus nephritis (LN) or antiphospholipid syndrome (APS). We analysed adjusted relative risks (aRRs) of adverse pregnancy outcomes in SLE subgroups, comparing those with and without CVEs, to the reference group of pregnant individuals without autoimmune rheumatic diseases or APS and CVEs. CVEs were broadly defined to encompass thromboembolic and cardiovascular conditions associated with SLE.</p><p><strong>Results: </strong>CVEs complicated 17 130/7004 334 (0.2%) of pregnancies in individuals without autoimmune rheumatic diseases or APS, and 176/8422 (2.1%) with SLE, including 52/903 (5.8%) with LN and 40/513 (7.8%) with APS. Compared with the reference group, the aRRs for maternal complications were higher in SLE subgroups: non-cardiac severe maternal morbidity (3.2-fold to 31.5-fold), intensive care admission (2.0-fold to 12.2-fold), 1 year re-admission (2.4-fold to 6.0-fold) and death (7.0-fold to 7.9-fold). Similarly, adverse infant outcomes were higher: preterm birth (2.3-fold to 6.8-fold), small-for-gestational-age infant (1.8-fold to 3.4-fold), neonatal intensive care admission (2.1-fold to 7.9-fold) and infant death (1.6-fold to 3.7-fold), with highest risk estimates for SLE with LN or APS, particularly when complicated by CVEs.</p><p><strong>Conclusions: </strong>LN and APS in SLE contributed to incremental risks for adverse outcomes, with the combination of LN or APS with CVEs yielding the highest point estimates. This underscores the importance of disease severity but also the impact of CVEs, helping to individualise the risks of pregnancy complications for various SLE subpopulations.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12049864/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144000718","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}

{"title":"Validation cohort of a tool to predict proliferative histological class in lupus nephritis based on clinical and laboratory data: LUCAS Study (Lupus Nephritis Class Assessment System).","authors":"Antonio Silaide Araujo, Emilia Inoue Sato, Alexandre Wagner Silva de Souza, Fábio Jennings, Gianna Mastroianni Kirsztajn, Ricardo Sesso, Edgard Torres Dos Reis-Neto","doi":"10.1136/lupus-2025-001538","DOIUrl":"https://doi.org/10.1136/lupus-2025-001538","url":null,"abstract":"<p><strong>Objectives: </strong>Primary: To validate a previously developed lupus nephritis (LN) histologic class predictor tool (III or IV±(V) vs V). In this instrument, urinary sediment, anti-dsDNA antibody and serum creatinine were predictor variables. Secondary: To evaluate its accuracy in different cut levels of urinary red cell count and LN patient's profile that may be useful to guide diagnosis and treatment, especially when kidney biopsy is not available.</p><p><strong>Methods: </strong>A retrospective, cross-sectional study of 196 patients with SLE who underwent kidney biopsy, analysing sensitivity, specificity, positive and negative predictive values, accuracy, and positive and negative likelihood ratios.</p><p><strong>Results: </strong>81.6% of the patients were female, 60.2% were non-Caucasian and the mean age at the time of the biopsy was 31.2±10.4 years. 30 patients presented class III, 104 class IV, 36 class V and 26 mixed classes (7 class III+V and 19 class IV+V). In the validation cohort, sensitivity was 90.6%, specifically was 66.7%, positive predictive value was 92.4% and accuracy was 86.2%, in predicting proliferative classes (class III or IV(±V) vs class V). There was no difference when analysing urinary red cell counts >5 x 10³/mL, >10 x 10³/mL or >20 x 10³/mL, as well as when the tool was applied in the first or recurrent LN and in proliferative LN without class V.</p><p><strong>Conclusions: </strong>The validation of a tool to predict proliferative histologic class showed good performance, like that found in the development cohort, without difference in accuracy with different cut-off points for urinary red cell count or number of flares of LN. It is easily applied and can be accessed via the internet (https://nefritelupica.medicalcore.com.br).</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12039031/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143989136","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}

{"title":"Rural-urban disparities in hospitalisation for myocardial infarction in systemic lupus erythematosus in the USA.","authors":"Jasvinder A Singh, Sumanth Chandrupatla","doi":"10.1136/lupus-2025-001516","DOIUrl":"https://doi.org/10.1136/lupus-2025-001516","url":null,"abstract":"<p><strong>Objective: </strong>To assess whether rural-urban disparities exist in people with SLE for hospitalisation with myocardial infarction (MI).</p><p><strong>Methods: </strong>We used the 2016-2019 US National Inpatient Sample data that contain all hospitalisation data. In people with a diagnosis of SLE, we assessed the multivariable adjusted ORs (aORs) to examine the association of rural patient residence with MI hospitalisation, while adjusting for demographics, payer, income, hospital characteristics and the Deyo-Charlson Comorbidity Index.</p><p><strong>Results: </strong>We found that the crude rates of patients hospitalised with MI per 100 000 area specific SLE hospitalisations were higher in rural versus urban residents with SLE, 2265 versus 1435 (p value<0.001). In the multivariable-adjusted model that accounted for demographics, insurance payer, household income, comorbidities and hospital characteristics including geographical location, we found that rural residence was associated with an aOR of 1.98 (95% CI, 1.71 to 2.29; reference category, urban residence) of MI hospitalisations in people with SLE. Other factors significantly associated with the risk of MI were male sex, Medicaid or private insurance, urban not teaching or urban teaching hospital, Midwest region and a private hospital control, either for profit or not for profit.</p><p><strong>Conclusion: </strong>Rural residence doubled the risk of MI hospitalisation in people with SLE independent of demographics, payer status, social determinants of health and hospital characteristics. Our study highlights the disproportionate effect of rurality on health outcomes in people with SLE within the USA and a clear rural-urban gap disparity. Interventions to reduce this disparity are needed.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12039025/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144027540","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}

{"title":"Prevalence of sarcopenia and its association with clinical features and health-related quality of life in Brazilian women with systemic lupus erythematosus.","authors":"Emerson Pena, Leonardo Peterson Dos Santos, Rafaela Cavalheiro do Espírito Santo, Lucas Denardi Dória, Stephanie Pilotti, André Luiz Silveira Mallmann, Daniel Nóbrega de Moraes, Clarice Moura Mata Machado, Cristina Costa Duarte Lanna, Olivio Brito Malheiro, Emanoel Luis da Silveira, Rosa Weiss Telles, Fabiana de Miranda Moura, Andrese Aline Gasparin, Vanessa Hax, Poli Mara Spritzer, Tayane Muniz Fighera, Rafael Mendonça da Silva Chakr, Ricardo Machado Xavier, Odirlei André Monticielo","doi":"10.1136/lupus-2024-001447","DOIUrl":"10.1136/lupus-2024-001447","url":null,"abstract":"<p><strong>Objectives: </strong>To assess the prevalence of sarcopenia and examine its association with clinical features, health-related quality of life (HRQoL), muscle-specific strength and body composition in patients with systemic lupus erythematosus (SLE).</p><p><strong>Methods: </strong>In this cross-sectional multicentre study, women with SLE (18-50 years old) were included. Data collected included clinical features and HRQoL. Muscle strength was assessed using the handgrip test (kg), appendicular skeletal muscle mass index (ASMI, kg/m²) was measured using dual-energy X-ray absorptiometry. Physical performance was assessed using the timed-up-and-go test (TUG, seconds). Sarcopenia was defined by the European Working Group on Sarcopenia in Older People-2 criteria. The muscle-specific strength was evaluated by dividing their arm strength by their lean arm mass. Pearson's or Spearman's correlation coefficients were performed (accepted at p<0.05).</p><p><strong>Results: </strong>Seventy-three SLE women were included, with median (IQR) age and disease duration of 37 (30-44) years old and 10.0 (4.0-16.8) years, respectively. Most of the patients (83.5%) had inactive or low disease activity and 31.0% presented a disease damage index score ≥1. Mean (±SD) handgrip strength, ASMI and muscle-specific strength was 25.58±8.31 kg, 6.62±0.97 kg/m² and 6.6±2.3, respectively. Median TUG was 6.9 (6.1-8.2) s. The prevalence of probable sarcopenia was 11.1%, and sarcopenia was 2.7%. Lower muscle strength, lower muscle-specific strength and lower physical performance, as well as sarcopenia, were correlated with worse HRQoL (p<0.05).</p><p><strong>Conclusion: </strong>In Brazilian patients with SLE with inactive or low disease activity, the prevalence of sarcopenia was low. However, low muscle strength, low muscle-specific strength and low physical performance were correlated with worse HRQoL, emphasising the need for muscle strength assessments in SLE management.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12039037/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143979005","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}

{"title":"Can conventional brain MRI support the attribution process in neuropsychiatric SLE? A multicentre retrospective study.","authors":"Ettore Silvagni, Antonio Marangoni, Carlo Garaffoni, Simone Appenzeller, George Bertsias, Antonis Fanouriakis, Matteo Piga, Enrico Fainardi, Greta Carrara, Carlo Alberto Scirè, Marcello Govoni, Alessandra Bortoluzzi","doi":"10.1136/lupus-2024-001490","DOIUrl":"10.1136/lupus-2024-001490","url":null,"abstract":"<p><strong>Objectives: </strong>We aimed to investigate which elementary lesions, identified through conventional brain MRI, correlated with the attribution of neuropsychiatric (NP) manifestations of SLE as determined by clinical judgement (CJ) and a validated attribution algorithm (AA).</p><p><strong>Methods: </strong>We conducted a multicentre, retrospective cohort study of patients with SLE (1999-2018) from four tertiary SLE centres. Patients were assessed using American College of Rheumatology nomenclature and underwent MRI at their first NP event. NP manifestations were attributed to SLE using CJ and the AA. Elementary lesions were classified as follows: large infarcts, parenchymal haemorrhages, subarachnoid haemorrhages, inflammatory-type lesions, myelopathy, T2/fluid-attenuating inversion recovery (FLAIR) hyperintense lesions, lacunes, cerebral atrophy and microbleeds. Statistical analyses were performed using χ² and Fisher's exact tests. Univariable and multivariable logistic regression models were performed. A sensitivity analysis was performed using a revised AA, which excluded the item 'presence of abnormal MRI' from the list of favouring factors.</p><p><strong>Results: </strong>Among 154 patients, 88 (57%) had NP events attributed to SLE by CJ and 85 (55%) by AA. MRI was normal in 57/154 (37%) cases, while T2/FLAIR hyperintense lesions were the most frequent findings (71/154, 46%). A normal MRI was more common in non-attributed NP events per CJ and AA (OR 0.42, 95% CI 0.21 to 0.82 and 0.27, 95% CI 0.13 to 0.52, respectively). Cerebral atrophy was more frequent in non-attributed events per CJ (adjusted OR 0.06, 95% CI 0.01 to 0.35), while inflammatory-type lesions were more prevalent in SLE-attributed events according to AA (OR 3.91, 95% CI 1.15 to 18.1), with no significant change in sensitivity analyses.</p><p><strong>Conclusions: </strong>Our study elucidates the role of conventional MRI findings in the attribution process in NPSLE. The presence of selected elementary lesions or, instead, their absence could have a relevant weight in assessing NP events. These findings may assist clinicians in achieving a more accurate attribution of NP manifestations.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12039020/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144027516","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}

{"title":"Proteomics uncovers ICAM2 (CD102) as a novel serum biomarker of proliferative lupus nephritis.","authors":"Zhengyong Li, Yifang Sun, Yixue Wang, Fengxun Liu, Shaokang Pan, Songwei Li, Zuishuang Guo, Dan Gao, Jinghua Yang, Zhangsuo Liu, Dongwei Liu","doi":"10.1136/lupus-2024-001446","DOIUrl":"https://doi.org/10.1136/lupus-2024-001446","url":null,"abstract":"<p><strong>Objectives: </strong>This study aimed to identify novel, non-invasive biomarkers for lupus nephritis (LN) through serum proteomics.</p><p><strong>Methods: </strong>Serum proteins were detected in patients with LN and healthy control (HC) groups through liquid chromatography-tandem mass spectrometry. The key networks associated with LN were screened out using Cytoscape software, followed by pathway enrichment analysis. The best candidate biomarkers were selected by machine learning models, further validated in a larger independent cohort. Finally, the expression of these candidate markers was verified in kidney tissue samples, and the mechanism was explored by knocking down the expression of intercellular adhesion molecule 2 (ICAM2) through in vitro cell transfection with siRNA.</p><p><strong>Results: </strong>Following the serum proteomic screening of LN, a key network of 20 proteins was identified. Machine learning models were used to select ICAM2 (CD102), metalloproteinase inhibitor 1 (TIMP1) and thrombospondin 1 (THSB1) for validation in independent cohorts. ICAM2 exhibited the highest area under the curve (AUC) value in distinguishing LN from HC (AUC=0.92) and was significantly correlated with activity index, proteinuria, albumin and anti-dsDNA antibody levels. Particularly, ICAM2 was significantly elevated in proliferative LN and was associated with specific pathological attributes, outperforming conventional parameters in distinguishing proliferative LN from non-proliferative LN. ICAM2 expression was also elevated in renal tissue samples from patients with proliferative LN. In vitro, knockdown of ICAM2 expression can inhibit the activation of the PI3K/Akt pathway and alleviate the injury of glomerular endothelial cells.</p><p><strong>Conclusion: </strong>ICAM2 (CD102) may serve as a potential serum biomarker for proliferative LN that reflects renal pathology activity, potentially contributing to the progression of LN through the PI3K/Akt pathway.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12020755/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144017967","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}

{"title":"Correction: Hydroxychloroquine in patients with systemic lupus erythematosus: how much is enough?","authors":"","doi":"10.1136/lupus-2024-001254corr1","DOIUrl":"https://doi.org/10.1136/lupus-2024-001254corr1","url":null,"abstract":"","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-04-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12010306/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143971518","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}

{"title":"Clinical and immunological differences between primary and autoimmune-associated neuromyelitis optica spectrum disorders: a retrospective study.","authors":"Hung-Cheng Tsai, Yi-Syuan Sun, Wei-Sheng Chen, Wan-Hao Tsai, De-Feng Huang, Ying-Ying Yang, Hsien-Tzung Liao, Chang-Youh Tsai","doi":"10.1136/lupus-2024-001491","DOIUrl":"https://doi.org/10.1136/lupus-2024-001491","url":null,"abstract":"<p><strong>Introduction: </strong>Neuromyelitis optica spectrum disorder (NMOSD) is a rare immune-mediated disease affecting the spinal cord and optic nerves. While NMOSD has been widely studied, limited data exist on the subset associated with autoimmune diseases (AD-NMOSD), particularly in Taiwanese patients. Additionally, relapse and prognostic factors in AD-NMOSD remain unclear.</p><p><strong>Methods: </strong>We retrospectively analysed 71 NMOSD cases diagnosed between 2008 and 2023 at Taipei Veterans General Hospital. Clinical features, laboratory findings, autoimmune comorbidities, imaging and treatments were examined. Patients were stratified by relapse status and the presence of severe sequelae.</p><p><strong>Results: </strong>Among 71 NMOSD cases, 26 (37%) patients had AD-NMOSD. While no significant differences were observed in the number or severity of relapses and sequelae between AD-NMOSD and primary (p)-NMOSD, patients with AD-NMOSD exhibited lower white blood cell counts, haemoglobin, platelet counts, immunoglobulin G and C reactive protein levels. Specific risk factors for relapse in AD-NMOSD included onset age under 50 years, concurrent SLE and a longer duration of SLE before NMOSD presentation. In both AD-NMOSD and p-NMOSD, more relapses were associated with severe neurological sequelae. Although relapse-free survival did not differ significantly between the two groups, patients with AD-NMOSD tended to have a longer period without severe sequelae.</p><p><strong>Discussion: </strong>Taiwanese patients with AD-NMOSD show distinct laboratory characteristics compared with those without autoimmune diseases. Younger age and longer disease duration are key risk factors for relapses, which are linked to more severe neurological sequelae. Despite various treatments, no significant differences were found in relapse rates or sequelae severity, highlighting the need for personalised management strategies.</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/PMC11997819/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144064167","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}