Lupus Science & Medicine最新文献

{"title":"Evaluation of MRI-based brain oxygen extraction fraction mapping in patients with systemic lupus erythematosus.","authors":"Shuoqi Zhang, Jiayi Ma, Shaolong Wu, Ziwei Hu, Su Yan, Junghun Cho, Yi Wang, Lingli Dong, Shun Zhang, Wenzhen Zhu","doi":"10.1136/lupus-2025-001522","DOIUrl":"https://doi.org/10.1136/lupus-2025-001522","url":null,"abstract":"<p><strong>Objective: </strong>We aim to assess the cerebral oxygen extraction fraction (OEF) changes in patients with systemic lupus erythematosus (SLE) and neuropsychiatric SLE (NPSLE) by using an MRI-based technique and examine the relationship between OEF and cognition.</p><p><strong>Methods: </strong>43 SLE patients (18 NPSLE and 25 non-NPSLE) and 26 healthy controls (HC) were recruited. Cognitive function was assessed via the Montreal Cognitive Assessment (MoCA). OEF was calculated by quantitative susceptibility mapping plus quantitative blood oxygen level-dependent model (QQ). Whole-brain voxel-wise analysis of OEF was performed. In subcortical grey matter structures, regional OEF values were measured, and their relationship with MoCA scores was explored.</p><p><strong>Results: </strong>Whole-brain voxel-wise analysis revealed significant changes of OEF primarily in the limbic system, including the orbitofrontal cortex and bilateral insular lobes, among HC, non-NPSLE and NPSLE groups. Regional analysis indicated reduced OEF values in subregions of the amygdala, hippocampus and caudate nucleus in non-NPSLE compared with HC, with decreasing trends observed in all selected regions of subcortical grey matter structures. In the right hippocampus, OEF values were increased in NPSLE patients compared with non-NPSLE patients. Considering all subjects in the study, OEF values in the bilateral medial amygdalae, right lateral amygdala, left rostral hippocampus and right dorsal caudate nucleus were positively correlated with MoCA scores.</p><p><strong>Conclusion: </strong>Cerebral OEF mapping in patients with SLE is readily available using the MRI-based QQ method, which has the potential to serve as an adjunctive tool for diagnosing NPSLE and monitoring cognitive impairment in SLE.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144317348","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort.","authors":"Emiliano Marasco, Christina Düsing, Stefanie Keymel, Alessandra Bortoluzzi, Claudia Bracaglia, Matthieu Canuet, Ilaria Cavazzana, Gamal Chehab, Veronica Codullo, Rebecca Fischer, Franco Franceschini, Micaela Fredi, Stefano Ghio, Lisa Keller, Alain Meyer, Carlomaurizio Montecucco, Jutta Richter, Marianne Riou, Sezgin Sahin, Oliver Sander, Andreas Schwarting, Carlo Alberto Scirè, Ettore Silvagni, Konstantinos Triantafyllias, Giovanni Zanframundo, Lorenzo Cavagna, Matthias Schneider","doi":"10.1136/lupus-2024-001471","DOIUrl":"https://doi.org/10.1136/lupus-2024-001471","url":null,"abstract":"<p><strong>Objectives: </strong>The aim of our work was to identify specific patterns in clinical features and nailfold capillary changes that may help in screening for pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).</p><p><strong>Methods: </strong>We identified patients with SLE and type I PAH (n=20) without other connective tissue diseases and collected demographic, clinical and laboratory features. We selected as controls patients with SLE who underwent cardiopulmonary screening to exclude PAH (n=87): we collected demographic, clinical and laboratory features and performed nailfold videocapillaroscopy (NVC).</p><p><strong>Results: </strong>All patients with SLE-PAH were women; age and disease duration were not different from patients with SLE without PAH. Lupus anticoagulant (LAC)+and anti-ribonucleoprotein (RNP)+were more prevalent in patients with SLE-PAH (respectively, PAH 45.0% vs no-PAH 20.5%, p=0.042; PAH 45.0% vs no-PAH 19.5%, p=0.035). No differences were observed for anti-Sm, anti-Ro, anti-La and anti-cardiolipin and anti-beta2GPI antibodies. Among clinical features, mucocutaneous and central nervous system involvement were more prevalent in patients with SLE-PAH than in SLE controls (respectively, PAH 65.0% vs no-PAH 34.5%, p=0.024; PAH 25.0% vs no-PAH 8.0%, p=0.046). Raynaud's phenomenon (RP) was more prevalent in patients with SLE-PAH than in SLE controls (PAH 60.0% vs no-PAH 13.8%, p<0.001). RP was a predictor of PAH in patients with SLE (OR 3.8 (0.9-14.8)). We performed NVC on nine patients with PAH and on controls: we observed a significantly higher prevalence of scleroderma pattern at NVC in SLE-PAH than controls (PAH 66.7% vs no-PAH 9.2%, p<0.001). Patients with SLE-PAH showed a lower number of capillary density and a higher frequency of giant capillaries.</p><p><strong>Conclusions: </strong>Our data showed that LAC+, RNP+, RP and a scleroderma pattern at NVC was indicative for patients with SLE-PAH. Our results pointed to generalised microvascular involvement and a hypercoagulation state in patients with SLE-PAH. The variables we identified could be used to implement a screening algorithm to identify patients with SLE at risk of developing PAH.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12164622/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144293995","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":"Genetic relationships between systemic lupus erythematosus and a positive antinuclear antibody test in the absence of autoimmune disease.","authors":"Atlas Khan, Gul Karakoc, Ge Liu, Jacy Zanussi, Nancy J Olsen, Mingjian Shi, Nancy J Cox, Jonathan Mosley, Charles Michael Stein, Krysztof Kiryluk, Wei-Qi Wei, Frank Mentch, Scott Hebbring, James Linneman, Vivian Kawai","doi":"10.1136/lupus-2024-001476","DOIUrl":"10.1136/lupus-2024-001476","url":null,"abstract":"<p><strong>Objective: </strong>We defined the genetic factors associated with a positive ANA test (ANA+) in the absence of autoimmune disease and tested the association with SLE.</p><p><strong>Methods: </strong>Using a case-control design, we performed a genome-wide association study (GWAS) in individuals of European ancestry without an autoimmune disease who had ANA tested as part of clinical care from DNA biobanks linked to de-identified electronic medical records: BioVU and Electronic Medical Records and Genomics. GWAS results were meta-analysed and single nucleotide polymorphism (SNP) heritability was calculated. A polygenic risk score (PRS) for ANA+ and for SLE was constructed and compared in patients with SLE, ANA+ and ANA negative (ANA-) individuals without autoimmune disease and general controls who never had ANA testing performed.</p><p><strong>Results: </strong>A total of 7287 individuals of European ancestry were included in the meta-analyses (2169 ANA+ and 5118 ANA-); an SNP upstream of the <i>TSBP1</i> in the HLA locus (rs1967688) was associated with ANA+ (p=4.84×10^-8). SNP heritability for ANA+ was low (h² _SNP= 0.04), and the PRS for ANA+ was not significantly different in ANA+ and ANA- individuals. In contrast, the PRS for SLE was significantly higher in SLE compared with ANA+ individuals (p<2.2×10^-16) but did not differ among ANA+, ANA- and general control groups (p=0.17).</p><p><strong>Conclusions: </strong>ANA+ occurring in the absence of autoimmune disease has a genetic association with the <i>HLA</i> region, but overall heritability is low. In addition, few SLE-associated SNPs were associated with ANA+, and the PRS for SLE was not associated with ANA+, indicating limited genetic overlap.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12164615/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144293983","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":"Next generation sequencing analysis reveals complex genetic architecture of childhood-onset systemic lupus erythematosus.","authors":"Laura Lewandowski, Linda Hiraki, Christiaan Scott, Ana Barrera-Vargas, Jorge Romo Tena, Diana Gómez-Martín, Michael J Ombrello, Ivona Aksentijevich, Zuoming Deng, Anthony M Musolf, Subrata Paul, Shajia Lu, Massimo Gadina, Daniel Hupalo, Clifton L Dalgard, Sarfaraz Hasni, Earl D Silverman, Mariana J Kaplan","doi":"10.1136/lupus-2024-001475","DOIUrl":"10.1136/lupus-2024-001475","url":null,"abstract":"<p><strong>Objectives: </strong>Our current understanding of the genetic architecture of childhood-onset SLE (cSLE) is limited by a dearth of comprehensive genomic studies in cSLE. We have quantified the number of known rare and common SLE risk variants in a diverse cSLE cohort. We characterised type I interferon (IFN) gene expression scores along with genomic data.</p><p><strong>Methods: </strong>We performed whole genome sequencing on 83 patients with cSLE and 109 unaffected parents and analysed sequences for known common and rare SLE-associated risk variants. Type I IFN gene expression was quantified on a subset of patients. We investigated the relationship between clinical phenotype, genomic profile and type I IFN signatures in this cohort.</p><p><strong>Results: </strong>Patients with cSLE were enriched for common SLE risk variants compared with unaffected parents and controls. We identified rare SLE risk variants in 11% of individuals with cSLE; those with rare variants had earlier disease onset (<12 years) than those without variants. Patients with cSLE had elevated type I IFN gene expression compared with unaffected parents and controls, even though most patients were treated with immunosuppressive therapy.</p><p><strong>Conclusions: </strong>Patients with cSLE from this ancestrally and geographically diverse cohort are enriched for common cSLE risk variants compared with controls, and 11% carry a rare variant in known monogenic SLE risk genes. The relationship between rare and common risk variant burden is more complex than previously hypothesised. Our findings indicate that studying patients with cSLE is important for understanding genetic contributions to SLE pathogenesis.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161385/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275299","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":"Lupus nephritis serum induces changes in gene expression in human glomerular endothelial cells, which is modulated by L-sepiapterin: implications for redox-mediated endothelial dysfunction.","authors":"Dayvia A Russell, Justin P Van Beusecum, Margaret Markiewicz, Sandra M Sanchez, Jeremy L Barth, Jim C Oates","doi":"10.1136/lupus-2025-001568","DOIUrl":"10.1136/lupus-2025-001568","url":null,"abstract":"<p><strong>Objective: </strong>Lupus nephritis (LN) is characterised by renal endothelial dysfunction, which contributes to progressive kidney injury. Endothelial nitric oxide synthase (eNOS) plays a modulating role in LN, as genetic ablation of the eNOS enzyme worsens disease. Serum from patients with active LN induces uncoupling of eNOS homodimers, leading to superoxide (SO) rather than nitric oxide (NO) production by eNOS. This uncoupling is reversed with L-sepiapterin (L-Sep). This study was designed to further examine changes in gene expression in glomerular endothelial cells induced by LN serum and whether treatment with L-Sep can ameliorate these changes.</p><p><strong>Methods: </strong>Primary human renal glomerular endothelial cells (HRGECs) were cultured with serum from healthy controls (HCs), patients with LN during remission (LN rem) or patients with LN during flare (LN flare) with and without L-Sep. Bulk RNA sequencing was performed on RNA isolated from cultured cells. Differential gene expression was determined, and pathway and gene enrichment analyses were performed on differentially expressed genes.</p><p><strong>Results: </strong>L-Sep treatment induced differential gene expression after culture in HRGECs cultured with LN flare serum. Addition of L-Sep induced genes involved in promoting endothelial function and enriched for pathways of NO biosynthetic and metabolic processes, fatty acid and lipid biosynthesis, neurotransmitter biosynthesis, reactive oxygen biosynthesis, vascular endothelial growth factor production and regulation of smooth muscle contraction.</p><p><strong>Conclusions: </strong>These results indicate that glomerular endothelial cells can mount an active inflammatory response in an LN serum environment. More importantly, L-Sep modulates gene expression in a fashion consistent with reduction of oxidative stress and increased NO production. These findings provide the rationale to target endothelial dysfunction to modulate LN with L-Sep as a therapeutic.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161315/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144248597","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":"Comorbid psoriasis in systemic lupus erythematosus: a cohort study from a tertiary referral centre and the National Patient Register in Sweden.","authors":"Tomas Walhelm, Ioannis Parodis, Charlotta Enerbäck, Elizabeth Arkema, Christopher Sjöwall","doi":"10.1136/lupus-2025-001504","DOIUrl":"10.1136/lupus-2025-001504","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the prevalence of psoriasis in SLE using a Swedish regional cohort and a nationwide cohort from the National Patient Register (NPR). Furthermore, we compared clinical features between patients with and without comorbid psoriasis.</p><p><strong>Methods: </strong>In total, 351 patients diagnosed with SLE based on the 1982 American College of Rheumatology and/or the 2012 Systemic Lupus International Collaborating Clinics criteria from Linköping University Hospital were evaluated. We obtained patient-reported and relevant clinical data extracted in 2024. Individuals with coexisting psoriasis were identified via the International Classification of Diseases code L40 and subsequent confirmation through chart review in the regional cohort. In the NPR, 7490 subjects with SLE living in Sweden in 2022 were identified, as well as therapies obtained from the Prescribed Drug Register.</p><p><strong>Results: </strong>We identified 12 subjects with SLE and coexisting psoriasis (3.4%) in the regional cohort and 367 patients (4.9%) in the nationwide cohort. Men were proportionally more common in the group with comorbid psoriasis in both cohorts. Patients with psoriasis reported more pain on a visual analogue scale (median 45.5/100 mm, IQR 23.3-58.3) compared with those without coexisting psoriasis (median 27.0/100 mm, IQR 7.0-50.5, p<0.04). We observed no differences in damage accrual or clinical phenotypes between the two groups. Subjects with psoriasis were more frequently prescribed methotrexate in the nationwide cohort.</p><p><strong>Conclusion: </strong>The prevalence of coexisting psoriasis in patients with SLE in Sweden was estimated to be 3.4-4.9%. Individuals with comorbid psoriasis reported more pain and were more likely to be prescribed methotrexate than those without psoriasis.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12142130/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144234486","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":"Pregnancy characteristics of patients with systemic lupus erythematosus with different onset times and their risk of adverse pregnancy outcomes: a retrospective cohort study.","authors":"Yinghua Xu, Xujie Deng, Tao Zhang, Minyi Zhang, Jiaxin Huo, You Peng, Qian Yin, Shujie Liu, Haotong Ouyang, Lien Ma, Ruiyan Liu, Jun Chen, Ruhao Xie, Guifang Hu, Haoyue Hu, Mei Zhong","doi":"10.1136/lupus-2025-001529","DOIUrl":"10.1136/lupus-2025-001529","url":null,"abstract":"<p><strong>Objective: </strong>SLE is prevalent among women of reproductive age, increasing the risk of adverse pregnancy outcomes (APOs). However, the correlation between the onset time of SLE and APOs remains unclear. This study aimed to analyse and compare pregnancy outcomes and clinical characteristics among three groups of patients with SLE: those with childhood onset, onset in adulthood before pregnancy and onset in adulthood during pregnancy.</p><p><strong>Methods: </strong>A retrospective analysis was conducted on pregnant women with SLE admitted to Nanfang Hospital of Southern Medical University from 2010 to 2024. Patients were categorised based on the onset time of SLE. Clinical features, laboratory characteristics, medication and pregnancy outcomes were compared among three groups. Logistic regression analyses were used to explore the relationship between the onset time of SLE and APOs.</p><p><strong>Results: </strong>The study included a total of 251 pregnancies from 223 women. Pregnant women with SLE onset in adulthood during pregnancy had more pronounced multisystem disorders, higher disease activity and an increased incidence of APOs. SLE onset in adulthood during pregnancy was associated with a higher risk of fetal loss (OR=5.342, 95% CI 1.629 to 17.520, p=0.006) and premature birth (OR=6.390, 95% CI 1.244 to 32.828, p=0.026).</p><p><strong>Conclusions: </strong>Patients with SLE onset in adulthood during pregnancy exhibit more aggressive disease manifestations and higher rates of APOs, while women with childhood-onset or pre-pregnancy-onset SLE had a lower risk. The incidence of APOs does not correlate with disease duration if maternal disease is quiescent in the period before conception. Closer monitoring and tailored management strategies are needed for these patients.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12142142/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144216258","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":"Deucravacitinib, an oral, selective, allosteric, tyrosine kinase 2 inhibitor, in patients with active SLE: efficacy on patient-reported outcomes in a phase II randomised trial.","authors":"Marta Mosca, Laurent Arnaud, Anca Askanase, Coburn Hobar, Brandon Becker, Shalabh Singhal, Subhashis Banerjee, Samantha Pomponi, Jiyoon Choi, Vibeke Strand","doi":"10.1136/lupus-2025-001517","DOIUrl":"10.1136/lupus-2025-001517","url":null,"abstract":"<p><strong>Objective: </strong>In PAISLEY, a 48-week, phase II, randomised controlled trial that assessed deucravacitinib in patients with active SLE, all primary and secondary endpoints were met with the deucravacitinib 3 mg two times per day dose. Changes in patient-reported outcomes, collected as exploratory endpoints, were evaluated in this study.</p><p><strong>Methods: </strong>Patients with SLE (n=363) were randomised to placebo (n=90) or deucravacitinib 3 mg two times per day (n=91), 6 mg two times per day (n=93) or 12 mg once daily (n=89). Patients assessed pain levels on a Numeric Rating Scale and completed the Patient-Reported Outcomes Measurement Information System Fatigue Short Form 7a and 36-Item Short Form Health Survey (SF-36) at scheduled intervals. These outcomes were stratified by Systemic Lupus Erythematosus Responder Index 4 (SRI-4) and British Isles Lupus Assessment Group-based Composite Lupus Assessment (BICLA) response. Missing data were imputed using control-based pattern imputation.</p><p><strong>Results: </strong>At week 48, greater mean improvement in pain and fatigue scores from day 1 were reported across all deucravacitinib dose groups compared with placebo. Regardless of treatment group, SRI-4 and BICLA responders reported greater improvements in pain and fatigue than non-responders at week 48. Additionally, deucravacitinib-treated patients generally saw greater SRI-4 and BICLA response rates than placebo-treated patients. Pain decreased by 1.3 points vs 2.2-2.3 points and fatigue scores decreased by 3.4 points vs 5.9-7.3 points in the placebo versus deucravacitinib dose groups, respectively. Mean SF-36 physical scores were 41.5 vs ≥44.6 and mean SF-36 mental scores were 45.2 vs ≥46.3 with placebo versus deucravacitinib dose groups, respectively. A greater proportion of patients receiving deucravacitinib also reported clinically meaningful improvements in SF-36 scores compared with placebo.</p><p><strong>Conclusion: </strong>Patients with SLE experienced greater improvements in pain, fatigue and health-related quality-of-life scores at week 48 with deucravacitinib versus placebo treatment.</p><p><strong>Trial registration number: </strong>NCT03252587.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12142172/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144199525","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":"Glucocorticoid tapering early in the course of juvenile SLE: association with lupus low disease activity state and outcomes.","authors":"Ozge Baba, Hakan Kısaoğlu, Dilara Unal, Umit Gul, Özge Basaran, Sezgin Sahin, Ozgur Kasapcopur, Seza Ozen, Mukaddes Kalyoncu","doi":"10.1136/lupus-2024-001415","DOIUrl":"10.1136/lupus-2024-001415","url":null,"abstract":"<p><strong>Objective: </strong>To determine the feasibility and risk of flares by achieving successful glucocorticoid (GC) tapering during the first year of juvenile SLE and the value of early achievement of childhood lupus low disease activity state (cLLDAS).</p><p><strong>Methods: </strong>The medical charts of children with moderate-to-severe SLE between 2012 and 2022 were retrospectively analysed. Successful tapering was defined as the employment of a prednisolone equivalent dose, lower dose of either ≤7.5 mg/day or ≤0.15 mg/kg/day, as per the cLLDAS definition. A linear mixed-effects model was used to determine the fixed effects affecting the GC dose over the first year. Cox regression analysis was used to identify whether successful tapering increased the risk of flares, and logistic regression was used to determine the odds of flares after the twelfth month of treatment.</p><p><strong>Results: </strong>Successful GC tapering was observed in 50 out of 80 patients (62.5%) within the first year of treatment, and flares were observed in 23 (28.8%) patients. The GC tapering trajectories over time were similar based on flare observations (p>0.05). Furthermore, successful tapering did not increase the risk of flares. Additionally, patients without flares received significantly higher GC doses as the initial treatment (p=0.046). Achievement of cLLDAS was observed in 40 (50%) patients at the twelfth month; however, achievement was not protective against future flares, and positive anti-double-stranded DNA antibodies at the twelfth month increased the odds of flares (OR: 4.8, p=0.008).</p><p><strong>Conclusion: </strong>Successful GC tapering is feasible and does not increase the risk of flares during the early disease phase. However, flares are common and adversely affect GC tapering. Thus, the identification of children with an increased risk of flares on GC tapering is needed to reduce the GC burden.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12121582/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144174368","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":"Long-term efficacy and safety of the Lupus-Cruces Nephritis protocol: a propensity score study of the Lupus-Cruces and Lupus-Bordeaux cohorts.","authors":"Guillermo Ruiz-Irastorza, Beatriz Marín-García, Luis Dueña-Bartolomé, Diana Paredes Ruiz, Amaia Osorio, Estibaliz Lazaro","doi":"10.1136/lupus-2025-001562","DOIUrl":"10.1136/lupus-2025-001562","url":null,"abstract":"<p><strong>Objective: </strong>To assess the efficacy and toxicity of the Lupus-Cruces Nephritis (LCN) protocol compared with standard of care (SOC) with cyclophosphamide (CYC) or mycophenolate in patients with lupus nephritis (LN) during an extended follow-up time up to 10 years.</p><p><strong>Methods: </strong>Patients with biopsy-proven class III, IV or V LN treated with LCN were compared with SOC. Patients in the LCN were treated with a CYC plus repeated methylprednisolone pulse-based regimen. The achievement of complete renal response (CRR) and the progression to chronic kidney disease (CKD) were the two main outcomes. Glucocorticoid (GC)-related toxicity, major infections and damage accrual were also analysed. A propensity score (PS)-adjusted multivariate analysis was used to overcome the confounding-by-indication bias.</p><p><strong>Results: </strong>147 patients were included in this study (47 LCN and 100 SOC). CRR at 12 months was 85% vs 44%, respectively (p<0.001). Eventually, 96% patients in the LCN group achieved CRR vs 74% patients in the SOC (p=0.002). In the multivariate PS-adjusted Cox model, LCN patients were more likely to eventually achieve CRR (PS-adjusted HR 3.5, 95% CI 2.2 to 5.5, p<0.001). The risk of progression to CKD was lower in LCN patients (PS-adjusted HR 0.3, 95% CI 0.11 to 0.82, p=0.019). The risks of GC-induced toxicity, renal or GC-related damage accrual and major infections were also lower in the LCN group: adjusted HR 0.09, 95% CI 0.02 to 0.39; PS-adjusted HR 0.14, 95% CI 0.04 to 0.4; PS-adjusted HR 0.2, 95% CI 0.046 to 0.95; respectively.</p><p><strong>Conclusions: </strong>This study confirms the LCN protocol as an effective and safe, in addition to widely available and affordable, regimen for the induction therapy of LN.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12107586/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144159833","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}