Lupus Science & Medicine最新文献

{"title":"Effect of Benson relaxation response technique on the quality of life among patients with systemic lupus erythematous: quasi-experimental study.","authors":"Arwa Masadeh, Basema Mohammad Nofal, Rami Masa'deh","doi":"10.1136/lupus-2024-001301","DOIUrl":"10.1136/lupus-2024-001301","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the effect of Benson relaxation response technique (BRRT) on the quality of life (QOL) among patients with systemic lupus erythematous (SLE).</p><p><strong>Methodology: </strong>A quasi-experimental design was used to conveniently recruit 170 patients with SLE. Participants were divided into two groups, the control and the intervention group for which the BRRT intervention was administered. Utilising an online questionnaire, the QOL was assessed among the two groups, before and 2 months after the intervention, using the Arabic version of the short form 36-item health survey.</p><p><strong>Results: </strong>After 2 months of the intervention, the intervention group exhibited significantly higher levels in both components of QOL; physical (<i>t</i>(143.31)=15.35, p<0.001); and mental component (<i>t</i>(143.58)=12.35, p<0.001). Additionally, for the intervention group, the results revealed a statistically significant increase in the levels of both components from baseline measurement; physical (<i>t</i>(84)=-16.24, p<0.001) and mental component (<i>t</i>(84)=-12.93, p<0.001).</p><p><strong>Conclusion: </strong>The findings demonstrate a notable positive impact of BRRT on QOL among patients with SLE. Healthcare professionals can potentially improve the overall well-being of patients with SLE and complement traditional treatment by implementing BRRT into their care.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11752001/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984026","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":"Neutrophil gelatinase-associated lipocalin (NGAL) in lupus nephritis and beyond.","authors":"Marina Barguil Macedo, Ting Wang, Andreas Jönsen, Anders A Bengtsson, Iva Gunnarsson, Elisabet Svenungsson, Christian Lood","doi":"10.1136/lupus-2024-001418","DOIUrl":"10.1136/lupus-2024-001418","url":null,"abstract":"<p><strong>Objectives: </strong>To study neutrophil gelatinase-associated lipocalin (NGAL) levels in peripheral blood in SLE, and to propose a mechanism by which neutrophils secrete NGAL on stimulation with immune complexes (IC).</p><p><strong>Methods: </strong>NGAL was measured by ELISA in two independent Swedish SLE cohorts acting as exploratory and validation cohort (n=124 and n=308, respectively), disease controls (n=38) and healthy controls (n=77). NGAL levels were measured in supernatant from IC-stimulated neutrophils in the presence or absence of a toll-like receptor 8 inhibitor (TLR8i).</p><p><strong>Results: </strong>In the exploratory cohort, serum levels of NGAL were increased in patients with SLE as compared with healthy controls (p=0.021), and associated with histological-proven membranoproliferative lupus nephritis (LN) (p=0.018). In the validation cohort, plasma levels of NGAL were elevated in patients with a history of LN (p=0.0048), as well as in patients with SLE with secondary antiphospholipid syndrome (APS) compared with those without (p=0.0022). In both cohorts, NGAL was able to discriminate patients with a creatinine clearance <60 mL/min (chronic kidney disease stage 3 or more) with high accuracy, with an area under the curve of 0.92 (p<0.0001) and 0.94 (p=0.0088), respectively. Neutrophils stimulated with IC secrete more NGAL, when compared with baseline, and this process was blocked by adding a TLR8i.</p><p><strong>Conclusion: </strong>Blood levels of NGAL are increased in patients with SLE with decreased kidney function, and in those with secondary APS. The mechanism behind NGAL increase in SLE may be related to TLR8 pathway activation by circulating RNA-containing IC.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11751952/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984031","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":"Postmarketing safety evaluation of belimumab: a pharmacovigilance analysis.","authors":"Huqun Li, Wenlong Xie, Chongshu Wang, Cuilian Guo","doi":"10.1136/lupus-2024-001400","DOIUrl":"10.1136/lupus-2024-001400","url":null,"abstract":"<p><strong>Objective: </strong>The present study aimed to provide a comprehensive evaluation of the postmarketing safety of belimumab based on the Food and Drug Administration Adverse Event Reporting System (FAERS) database.</p><p><strong>Methods: </strong>Adverse event (AE) reports in the FAERS database from January 2021 to December 2023 were extracted to perform the disproportionality analysis by calculating the reporting OR. The clinical characteristics and onset times of AEs were investigated. The differences across ages and regions in belimumab-related AEs were also explored.</p><p><strong>Results: </strong>A total of 4 974 201 AE reports were retrieved from the FAERS database, among which 9782 reports were related to belimumab. 485 positive safety signals related to belimumab were identified. In addition to the labelled AEs, such as depression and infections, new unexpected AEs, including product dose omission issue and inappropriate schedule of product administration, were identified. The median onset time of belimumab-related AEs was 75 days. Moreover, our analysis revealed frequently reported AEs in paediatric patients, such as systemic lupus erythematosus, and in adult patients, such as injection site pain. Additionally, AEs such as drug ineffective were commonly reported in patients of North America, Asia and Europe, while AEs, including an inappropriate schedule of product administration, had a high incidence in patients of South America.</p><p><strong>Conclusion: </strong>The current study provides a valuable evaluation of the postmarketing safety of belimumab. Further studies are required to validate and confirm these findings. Clinicians should be vigilant regarding these potential AEs and pay more attention to the proper dosage regimen of belimumab in clinical practice.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11751785/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142965516","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":"Interplay of NF-κB and PPAR-γ transcription factors in patients with juvenile systemic lupus erythematosus.","authors":"Sinem Durmus, Sezgin Sahin, Amra Adrovic, Kenan Barut, Remise Gelisgen, Hafize Uzun, Ozgur Kasapcopur","doi":"10.1136/lupus-2024-001263","DOIUrl":"10.1136/lupus-2024-001263","url":null,"abstract":"<p><strong>Objective: </strong>Juvenile SLE (jSLE) is an autoimmune disease characterised by the presence of high levels of autoantibodies, predominantly targeting nuclear antigens, resulting in a breakdown of self-tolerance. However, its pathogenesis is multifactorial and poorly understood. The aim of this study was to evaluate the potential of nuclear factor-kappa B (NF-κB) and peroxisome proliferator-activated receptor-gamma (PPAR-γ) as biomarkers for jSLE.</p><p><strong>Methods: </strong>In this study, serum NF-κB and PPAR-γ levels were determined by immunoassay in 42 patients with jSLE. In addition, 19 juvenile systemic sclerosis (jSSc) and 25 age-matched healthy children were selected as patient control and healthy control, respectively.</p><p><strong>Results: </strong>Serum NF-κB levels in patients with jSLE demonstrated a positive trend towards elevation compared with the controls with no significant difference (p=0.030). In addition, serum NF-κB levels in patients with jSSc were significantly higher than that of the healthy controls (p=0.005). Serum PPAR-γ levels were tend to be lower in both patients with jSLE and jSSc compared with the controls, with no significant difference. Specifically, NF-κB levels were significantly higher in patients with jSLE with cumulative damage (PedSDI≥1) compared with those without, at p=0.044. Logistic regression showed that PPAR-γ levels lower than 2.42 ng/mL were associated with the development of jSLE (OR 7.59) and lower than 2.16 ng/mL for jSSc (OR 10.90). The combined high levels of NF-κB with low PPAR-γ increased the risk of developing jSSc by 21.33-fold.</p><p><strong>Conclusions: </strong>The observed trend of elevated NF-κB levels and decreased PPAR-γ levels in our study suggests their potential as biomarkers associated with increased proinflammatory signalling in jSLE and jSSc. However, our findings must be regarded as hypothesis-generating and confirmed in larger datasets. Moreover, their roles in monitoring the course of a disease and guiding therapeutic strategies in juvenile systemic autoimmune diseases need to be clearly investigated. Further extension of these findings may lead to better management and improvement in the outcomes of such patients.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11751921/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142950798","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":"Belimumab versus telitacicept in sequential treatment after rituximab for refractory lupus nephritis: a real-world multicentre study.","authors":"Yiting Chen, Xin Lei, Jianhang Xu, Xiaochan Chen, Hong Pan, Qiankun Zhang, Junni Wang, Pingping Ren, Lan Lan, Nan Shi, Liangliang Chen, Yaomin Wang, Jianghua Chen, Lie Jin, Yi Yang, Jing Xue, Fei Han","doi":"10.1136/lupus-2024-001296","DOIUrl":"10.1136/lupus-2024-001296","url":null,"abstract":"<p><strong>Objective: </strong>Both belimumab and telitacicept are recognised blockers for B lymphocyte activation, both of which have been approved as add-on therapies for SLE in China. The aim of this study is to compare the efficacy of rituximab (RTX) followed by belimumab or telitacicept in a real-world cohort.</p><p><strong>Methods: </strong>A total of 49 refractory lupus nephritis patients were enrolled from four independent centres, subsequently categorised into two treatment groups: belimumab group (n=35) and telitacicept group (n=14) based on their treatment following RTX. The outcomes of renal response rates were evaluated.</p><p><strong>Results: </strong>In this study cohort, 63.3% presented with anti-dsDNA antibody positivity and 79.6% exhibited hypocomplementemia, with a mean Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) Score of 13±6, estimated glomerular filtration rate (eGFR) of 76.2 (30.2, 113.7) mL/min and urinary protein creatinine ratio (uPCR) of 2.45 (0.77, 5.19) g/g. There was no significant differences between groups. After a follow-up duration of 26±12 months, renal objective remission rate was 80.0% (28 patients) in belimumab group and 85.7% (12 patients) in telitacicept group (difference, 5.7 percentage points, 95% CI, -25.8 to 26.8, p=1.000). Renal complete response was 54.3% (19 patients) in belimumab group and 78.6% (11 patients) in telitacicept group (difference, 24.3 percentage points, 95% CI, 9.7 to 47.8, p=0.194). The anti-dsDNA antibody, complement, eGFR, uPCR and SLEDAI-2K Score were improved in both groups with a significant reduction in prednisone dose. Major adverse effects included immunoglobulin deficiency, respiratory tract infection and urinary tract infection. No death occurred.</p><p><strong>Conclusions: </strong>The sequential treatment of belimumab or telitacicept following RTX may represent a promising therapeutic approach in the management of refractory lupus nephritis. Further investigation is necessary to establish optimal protocols and long-term benefits.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11751942/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143024096","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":"Low versus high initial oral glucocorticoid dose for lupus nephritis: a pooled analysis of randomised controlled clinical trials.","authors":"Amit Saxena, Cristina Sorrento, Peter Izmirly, Janine Sullivan, Monica Gamez-Perez, Jammie Law, Howard Michael Belmont, Jill P Buyon","doi":"10.1136/lupus-2024-001351","DOIUrl":"10.1136/lupus-2024-001351","url":null,"abstract":"<p><strong>Objective: </strong>Traditional initial treatment regimens for lupus nephritis (LN) used oral glucocorticoids (GC) in starting doses up to 1.0 mg/kg/day prednisone equivalent with or without a preceding intravenous methylprednisolone pulse. More recent management guidelines recommend lower starting oral GC doses following intravenous pulse therapy. As there have been no large studies directly comparing patients receiving low versus high initial oral GC doses, this pooled analysis of high-quality randomised controlled trials (RCTs) aims to evaluate differences in efficacy and safety.</p><p><strong>Methods: </strong>Published data were analysed from RCTs that assessed variable GC doses in the standard of care (SOC) treatment arms. Patients receiving starting prednisone doses up to 0.5 mg/kg/day (low dose) were compared with 1.0 mg/kg/day (high dose). Complete renal response requiring urine protein-creatinine ratio <0.5 mg/mg (CRR 0.5), CRR or partial renal response (PRR), serious adverse events (SAE) and SAE due to infections at 12 months of treatment were compared between groups.</p><p><strong>Results: </strong>417 patients from SOC arms of five studies were exposed to low-dose initial GC after intravenous pulse, while 521 patients from four studies were treated with high-dose oral GC. In patients with low-dose oral GC, 25.2% achieved CRR 0.5 at 12 months compared with 27.2% in high-dose groups, p=0.54. CRR or PRR was attained in 48.7% low-dose vs 43.6% high-dose patients, p=0.14. SAEs and infection SAEs were less common in the low-dose GC group (19.4% vs 31.6%, p<0.001 and 9.8% vs 16.5%, p=0.012, respectively).</p><p><strong>Conclusions: </strong>Based on pooled RCT data, there was no significant difference in 12-month renal responses between patients receiving low-dose prednisone following intravenous GC compared with those receiving initial high doses. SAEs were less frequent in patients receiving low-dose initial GC. These findings support the use of lower oral GC doses in LN treatment.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11752037/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142950814","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":"Racial differences in clinical trial perceptions among a large, predominantly Black cohort of people with systemic lupus erythematosus in the Southeastern USA.","authors":"Jessica Nicole Williams, Hilton Mozee, Gaobin Bao, Charmayne Dunlop-Thomas, Kim Schofield, Cristina Drenkard, Sung Sam Lim","doi":"10.1136/lupus-2024-001357","DOIUrl":"10.1136/lupus-2024-001357","url":null,"abstract":"<p><strong>Objective: </strong>Black people in the USA have a higher incidence and severity of SLE and worse outcomes, yet they are significantly under-represented in SLE clinical trials. We assessed racial differences in clinical trial perceptions among a large cohort of predominantly Black people with SLE.</p><p><strong>Methods: </strong>Georgians Organised Against Lupus (GOAL) is a population-based, prospective cohort of people with a validated diagnosis of SLE living in Atlanta. The 2021-2022 GOAL survey included questions assessing knowledge, perceptions and experiences of lupus clinical trials involving drug therapy. Self-reported race was categorised as Black or non-Black. Survey responses by race were compared using χ² analyses. Among Black respondents, factors associated with willingness to participate in clinical trials were examined using univariable logistic regression.</p><p><strong>Results: </strong>A total of 767 individuals responded to the 2021-2022 GOAL survey, of whom 80% were Black. There were 720 female respondents and 47 male respondents. There was no significant difference in willingness to participate in clinical trials between Black and non-Black respondents (28% vs 31%, p=0.071). Black respondents were less likely to correctly identify the definition of a clinical trial (34% vs 70%, p<0.001). Male gender, unemployed or disabled status, governmental health insurance and higher disease activity were associated with willingness to participate in clinical trials among Black respondents.</p><p><strong>Conclusions: </strong>We found that only 28% of respondents were willing to participate in lupus clinical trials, with no difference by race. Efforts must continue to engage those resistant to trial participation, regardless of race. Our findings also indicate that further research is warranted to assess whether strategies such as clinical trial education and diversification of study staff may be helpful to increase Black patient recruitment. Sociodemographic factors (gender, work status, insurance status) and disease-related factors (lupus activity) may also play important roles in clinical trial participation among Black people.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7,"publicationDate":"2025-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11751829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142927463","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":"Real-world treatment patterns, healthcare resource utilisation and costs in patients with SLE in the USA.","authors":"Prajakta P Masurkar, Jennifer Reckleff, Nicole Princic, Brendan Limone, Hana Schwartz, Elaine Karis, Eric Zollars, Karen Costenbader","doi":"10.1136/lupus-2024-001290","DOIUrl":"10.1136/lupus-2024-001290","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the treatment patterns, medication adherence, concomitant corticosteroid use, factors influencing sequence of therapies (SOTs), healthcare resource utilisation (HCRU) and associated costs in adults with SLE in the USA.</p><p><strong>Methods: </strong>Claims data from the Merative MarketScan Commercial and Medicare Supplemental Database between 2011 and 2019 were used to identify patients with incident SLE. The date of first claim with SLE was defined as the index date, with a 24-month pre-index and ≥24-month post-index period. Descriptive statistics were used to evaluate patient demographics and baseline clinical characteristics, treatment patterns, adherence, HCRU and cost. Multivariable-adjusted logistic regression models were used to identify factors associated with transition between SOTs.</p><p><strong>Results: </strong>Overall, 2476 patients received SLE treatment. The mean (SD) age was 46.9 (14.1) years and the mean (SD) follow-up duration was 47.8 (15.7) months. High corticosteroid use was prevalent in all SOTs (≥1 corticosteroid; average dose, 16.8-19.3 mg/day; 50%-60% patients). Antimalarials were most commonly prescribed in SOT 1 (85.7%), and immunosuppressants in SOT 2 and 3 (85.4% and 77.5%, respectively). Transition frequency from SOT 1-2 (38.4%) and SOT 2-3 (16.9%) was influenced by immunosuppressant prescription, concomitant corticosteroid use, sex, severe disease activity, non-persistence and age. Adherence was highest for biologics, followed by antimalarials and immunosuppressants. SLE-related HCRU and associated costs increased with SOT progression (mean (SD) at baseline vs SOT 3, US$19 489 (US$45 336) vs US$23 201 (US$39 628)).</p><p><strong>Conclusion: </strong>SLE treatment regimens with greater adherence and reduced corticosteroid use, HCRU and associated costs are needed.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11683911/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142885360","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 randomised controlled trials: evidence gaps and under-represented groups.","authors":"Alberto Nordmann-Gomes, Gabriel Cojuc-Konigsberg, Adriana Hernández-Andrade, Valeria Navarro-Sánchez, Juan Carlos Ramírez-Sandoval, Brad Rovin, Juan M Mejia-Vilet","doi":"10.1136/lupus-2024-001331","DOIUrl":"10.1136/lupus-2024-001331","url":null,"abstract":"<p><strong>Objective: </strong>We performed a scoping review of randomised clinical trials (RCTs) assessing pharmacological therapies for the initial management of lupus nephritis (LN), focusing on study design, included populations and outcome definitions, to assess the generalisability of their results and identify gaps in the evidence.</p><p><strong>Methods: </strong>RCTs evaluating pharmacological interventions for the initial therapy of LN published between 2000 and 2024 were evaluated. Extracted variables included study design, selection criteria, outcome definitions, populations recruited and clinical characteristics of participants. Each study arm was included as intervention and segregated into guideline-recommended regimens (cyclophosphamide (CYC), mycophenolic acid analogues (MPAAs), calcineurin inhibitors and belimumab) or other regimens. Data were analysed by descriptive statistics, and Fragility Index (FI) was estimated to assess robustness of studies.</p><p><strong>Results: </strong>We included 124 intervention arms within 61 RCT, involving 7058 participants. Seventy-nine arms (63.7%) corresponded to guideline-recommended therapies: 33 (26.6%) MPAA, 28 (22.6%) NIH-CYC and 7 (5.6%) triple-drug therapies. While 100% of triple-drug therapies RCT were multinational, only 7.1% of NIH-CYC and 0% of tacrolimus RCTs were conducted in more than one country. Only 9 (14.8%) had follow-up ≥24 months. Ten (16.4%) RCTs exclusively included participants with severe or refractory LN. Only 29 (47.5%) reported serious adverse events, and few described patient-reported outcomes. Black and other race participants were under-represented, as well as participants from Middle East, North Africa, and the sub-Saharan African region. Response was variably defined and assessed at different intervals. Robustness of RCTs evaluating double-drug guideline-recommended therapies were mostly low, with FI ranging from 1 to 3.</p><p><strong>Conclusions: </strong>Considering new recommendations for the management of LN, we call for broader inclusion of under-represented populations and homogenisation of study design. This study provides the rationale for evaluating unexplored treatment comparisons and conducting research on newer interventions in clinical settings where evidence is currently lacking.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11664369/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142872477","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":"Efficacy of initial combination with belimumab in newly diagnosed childhood-onset lupus nephritis: a single-centre historical control study.","authors":"Yinv Gong, Shuhua Liu, Haimei Liu, Yu Shi, Yifan Li, Wanzhen Guan, Qiaoqian Zeng, Qianying Lv, Xiaomei Zhang, Qijiao Wei, Jing Chen, Qian Shen, Hong Xu, Li Sun","doi":"10.1136/lupus-2024-001350","DOIUrl":"10.1136/lupus-2024-001350","url":null,"abstract":"<p><strong>Objective: </strong>To explore the efficacy of initial treatment of newly diagnosed childhood-onset lupus nephritis (cLN) with combination of belimumab and either cyclophosphamide, mycophenolate mofetil, tacrolimus or multitargeted therapy.</p><p><strong>Methods: </strong>A historical control study was conducted on children aged 5-17 years with newly diagnosed cLN. All patients recruited met the 2012 Systemic Lupus International Collaborating Clinics and/or 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for SLE, and the 2003 International Society of Nephrology/Renal Pathology Society histopathological criteria for LN. The primary endpoint was primary efficacy renal response (PERR) at 12 months, and secondary endpoints included complete renal response (CRR), lupus low disease activity state (LLDAS) and remission (Definitions of Remission in Systemic Lupus Erythematosus (DORIS)) at 12 months, changes of SLE Disease Activity Index (SLEDAI) and dose of glucocorticoid (GC).</p><p><strong>Results: </strong>A total of 101 patients were included with 38 patients in the belimumab group and 63 patients in the standard immunotherapy group. There were no statistically significant differences between the two groups at baseline. At 12 months, compared with the standard immunotherapy group, more patients in the belimumab group had a higher PERR (97.1% vs 80.0%, χ²=3.965, p=0.046), CRR (94.1% vs 76.6%, χ²=4.679, p=0.031), LLDAS (75.0% vs 18.6%, χ²=27.84, p<0.001) and DORIS (34.4% vs 11.9%, χ²=6.626, p=0.01). The belimumab group had faster and greater reductions in SLEDAI and dose of GC (p<0.05), with a significantly higher proportion of patients with dose of GC ≤7.5 mg/day (82.9% vs 30.4%, χ²=19.737, p<0.001). In the standard immunotherapy group, 4 patients (6.3%) experienced a decline in estimated glomerular filtration rate of 30% or more at 12 months, while no patients in the belimumab group experienced worsening of renal function. There were no serious adverse events reported in two groups, and there was no significant difference in the occurrence of infection between the two groups.</p><p><strong>Conclusion: </strong>This study reported for the first time the effectiveness of combined belimumab therapy in a Chinese cohort of patients with cLN. The strategy of initial combination with belimumab helps achieve treatment targets earlier and faster GC tapering. And initial combination therapy in children with cLN with high disease activity may yield more significant benefits.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11647295/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142828819","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}