Lupus Science & Medicine最新文献

{"title":"Non-criteria antiphospholipid antibody profiles and thrombotic outcomes in a cohort of patients with systemic lupus erythematosus.","authors":"Alistair Murray, Eric J Campbell, Ann Elaine Clarke, Megan R W Barber, Tania Pannu, Marvin J Fritzler, Michelle Jung, Yvan St Pierre, Leslie Skeith","doi":"10.1136/lupus-2024-001174","DOIUrl":"10.1136/lupus-2024-001174","url":null,"abstract":"<p><strong>Objectives: </strong>Antiphospholipid syndrome (APS) is characterised by the presence of antiphospholipid antibodies (aPLs) and clinical outcomes of thrombosis and/or obstetric morbidity and is associated with systemic lupus erythematosus (SLE). IgG antiphosphatidylserine/prothrombin complex (aPS/PT), IgM aPS/PT and IgG anti-beta 2 glycoprotein 1-domain 1 (aβ2GP1-D1) are novel aPLs that have been associated with thrombosis; however, conclusive data are still lacking. It remains unclear how best to incorporate non-criteria autoantibodies into clinical decision-making. The aim of this study was to assess whether these novel aPLs were associated with an increased risk of thrombosis in patients with SLE.</p><p><strong>Methods: </strong>We evaluated 341 patients enrolled in the SouThern Alberta Registry for Lupus EryThematosus database with SLE by the American College of Rheumatology or Systemic Lupus International Collaborating Clinics classification criteria. Medical records were reviewed between March 2006 and January 2021 for thrombotic events and serology results for lupus anticoagulant, IgG anticardiolipin, IgG anti-beta 2 glycoprotein 1 (aβ2GP1), IgG aPS/PT, IgM aPS/PT and IgG aβ2GP1-D1.</p><p><strong>Results: </strong>Among 341 patients with SLE, 59 (17%) met the revised Sapporo lab criteria, and of those 29 (49%) had a major thrombotic event (OR 3.5, 95% CI 1.9 to 6.3). Among 142 patients who had at least one positive non-criteria autoantibody, 45 (32%) had a major thrombotic event (OR 1.6, 95% CI 0.97 to 2.6). In a univariate analysis, the IgG aPS/PT and IgG aβ2GP1-D1 were associated with major and all thrombotic events. In a multivariate analysis that controlled for age, sex, prednisone use, SLE disease activity (Systemic Lupus Erythematosus Disease Activity Index-2K and the revised Sapporo lab criteria, among the non-criteria aPLs, only IgG aPS/PT was associated with an increased risk of a major thrombosis (OR 2.2, 95% CI 1.1 to 4.5).</p><p><strong>Conclusions: </strong>In our multivariate analysis, IgG aPS/PT was associated with a modestly increased risk of thrombotic events.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11647292/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142822222","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":"Patients achieving low lupus disease activity state, systemic lupus erythematosus disease control or remission showed lower rates of organ damage during longitudinal follow-up: analysis of the Hopkins Lupus Cohort.","authors":"Jacob Hunnicutt, Mary Elizabeth Georgiou, Anna Richards, Holly Quasny, Laurence Magder, Daniel Goldman, Michelle A Petri","doi":"10.1136/lupus-2024-001206","DOIUrl":"10.1136/lupus-2024-001206","url":null,"abstract":"<p><strong>Objective: </strong>One key target of treating patients with systemic lupus erythematosus (SLE) is to prevent organ damage. This analysis quantified the association between time spent in four specific SLE low disease activity (LDA) states and organ damage rate.</p><p><strong>Methods: </strong>This retrospective real-world data analysis (GSK Study 207168), undertaken to help contextualise the BLISS-BELIEVE clinical trial, included adults with SLE enrolled for≥1 year in the Hopkins Lupus Cohort and treated with standard therapy in a specialist care centre between 1987 and 2019. LDA states (Lupus Low Disease Activity State (LLDAS), disease control, clinical and complete remissions) were defined using SLE Disease Activity Index (SLEDAI)/Physician Global Assessment scores, prednisone-equivalent dose and medication use criteria combinations. Time spent in each LDA state was expressed as a percentage of total follow-up (0%; >0-<25%; 25-49%; 50-74%; ≥75%). Pooled logistic models were used to estimate adjusted rate ratios (aRR) between time spent in LDA states and organ damage rate (assessed using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)).</p><p><strong>Results: </strong>Overall, 1632 patients experienced 1246 organ damage events. Follow-up time (calculated from days of follow-up) totalled 9841.1 person-years. At baseline, the mean (SD) SLEDAI score was 2.8 (3.3) and the mean (SD) SDI score was 1.7 (1.9). Organ damage rates were lower in patients who achieved an LDA state versus those who did not. Rates decreased with increasing time spent in each LDA state. Even a small percentage of time (>0-<25% vs 0%) spent in an LDA state was associated with reduced damage (aRR (95% CI): LLDAS, 0.75 (0.61, 0.91); disease control, 0.80 (0.68, 0.93); clinical remission, 0.73 (0.60, 0.88); complete remission, 0.80 (0.68, 0.93)).</p><p><strong>Conclusions: </strong>Regardless of definition, achieving and maintaining a low disease activity state was associated with reduced organ damage in patients with SLE.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11647352/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142813672","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":"Treatment trends of systemic lupus erythematosus from 2007 to 2023 in the USA.","authors":"Gabriel Figueroa-Parra, Herbert C Heien, Kenneth J Warrington, Nilay D Shah, Cynthia S Crowson, Rozalina G McCoy, Alí Duarte-García","doi":"10.1136/lupus-2024-001317","DOIUrl":"10.1136/lupus-2024-001317","url":null,"abstract":"<p><strong>Objective: </strong>To characterise the changing trends in the pharmacological management of SLE in the USA between 2007 and 2023 as new treatment options emerged.</p><p><strong>Methods: </strong>In a retrospective cohort study using data from OptumLabs Data Warehouse, we characterised the annual prevalent (ie, all) and incident (ie, new) use of antimalarials, glucocorticoids and immunosuppressive medications among patients with SLE from 2007 to 2023 and assessed for changing trends over time.</p><p><strong>Results: </strong>We identified 19 122 adults with SLE; they were 51.2 (SD 16.1) years of age, 89% were female, 61.3% were White, 18.5% were Black and 13.1% were Hispanic. The proportion of prevalent users of antimalarials has decreased from 79.4% in 2007 to 77.2% in 2023 (p=0.0055), while the proportion of incident users fluctuated between a lowest 5.8% in 2021 and a highest 8.1% in 2008 (p=0.008). The proportion of prevalent users of glucocorticoids increased from 64.6% in 2007 to 66.7% in 2023 (p=0.0132), as did the proportion of incident users (12.4% in 2007 to 21.7% in 2023; p<0.0001). The use of cyclophosphamide (2.0% in 2007 to 0.4% in 2023, p<0.0001) has decreased; the use of mycophenolate mofetil (7.7% in 2007 to 10.3% in 2023, p<0.0001), rituximab (1.4% in 2007 to 2.1% in 2023, p<0.0001) and belimumab (0.8% in 2011 to 6.1% in 2023, p=0.0001) has increased.</p><p><strong>Conclusions: </strong>Despite increasing availability of alternative treatment options, patients with SLE in the USA increasingly rely on glucocorticoid-based therapy. Efforts to improve the use of antimalarials and steroid-sparing immunosuppressants are needed.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11629018/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142801446","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":"Interferon-α as a biomarker to predict renal outcomes in lupus nephritis.","authors":"Laura Patricia Whittall Garcia, Dafna D Gladman, Murray Urowitz, Dennisse Bonilla, Raphael Schneider, Zahi Touma, Joan Wither","doi":"10.1136/lupus-2024-001347","DOIUrl":"10.1136/lupus-2024-001347","url":null,"abstract":"<p><strong>Objective: </strong>To determine if serum interferon (IFN)-α levels at the time of a lupus nephritis (LN) flare are associated with renal outcomes.</p><p><strong>Methods: </strong>Patients with an LN flare who had a preflare estimated glomerular filtration rate (eGFR) ≥60 mL/min were included in the study. The following outcomes were ascertained: (1) Time to first and second LN flares during follow-up, (2) Time to a sustained decline in eGFR by 30% and 50%, and progression to end-stage renal disease (ESRD, <15 mL/min), and (3) Time to an adverse renal event (≥2 renal flares and/or at least a 30% sustained decline in eGFR during follow-up). Serum IFN-α was measured by Simoa.</p><p><strong>Results: </strong>92 patients with active LN were included in the study. Elevated serum baseline levels of IFN-α predicted poor renal outcomes. Patients with higher baseline IFN-α had a greater risk of having two or more subsequent LN flares (HR: 1.31 (1.08-1.59), p=0.006), sustained 30% decline in eGFR (HR: 1.27 (1.14-1.40), p<0.001), 50% decline in eGFR (HR: 1.27 (1.12-1.33), p<0.001) and progressing to ESRD (HR: 1.29 (1.14-1.47), p<0.001). Receiver operating characteristic analysis identified an IFN-α cut-off, 0.6 pg/ml, for predicting an adverse renal event.</p><p><strong>Conclusions: </strong>Elevated serum IFN-α levels measured at the time of an LN flare are associated with poor renal outcomes, including the development of ≥2 LN flares, and a clinically meaningful decline in kidney function.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-11-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11605838/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142755032","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":"Renal response status to predict long-term renal survival in patients with lupus nephritis: results from the Toronto Lupus Cohort.","authors":"Murray Urowitz, Mary E Georgiou, Zahi Touma, Jiandong Su, Juan Pablo Diaz-Martinez, Qinggong Fu, Roger A Levy, Kerry Gairy, Anne MacKinnon, Nicole Anderson, Patricia C Juliao","doi":"10.1136/lupus-2024-001264","DOIUrl":"10.1136/lupus-2024-001264","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate modified versions of the Belimumab International Study in Lupus Nephritis (BLISS-LN) belimumab study primary efficacy renal response (mPERR) and complete renal response (mCRR) criteria (excluding mandatory corticosteroid tapering) as predictors of real-world, long-term renal outcomes among patients with lupus nephritis (LN).</p><p><strong>Methods: </strong>This retrospective, observational study (GSK Study 212866) used deidentified data between 1970 and 2015 from the University of Toronto Lupus Cohort from adults diagnosed with systemic lupus erythematosus and biopsy-proven Class III±V, IV±V or V LN. At 24 months postbiopsy, patients were retrospectively indexed as responders/non-responders based on mPERR (estimated glomerular filtration rate (eGFR) ≤20% below biopsy value/≥60 mL/min/1.73 m² and urine protein:creatinine ratio (uPCR) ≤0.7 g/day) or mCRR (eGFR ≤10% below biopsy value/≥90 mL/min/1.73 m² and uPCR ≤0.5 g/day) criteria. The association between index mPERR (primary outcome) or mCRR (secondary outcome) status and long-term (up to 25 years, until censoring or death) renal survival (no progression to end-stage kidney disease (eGFR <30 mL/min/1.73 m², dialysis or transplant) or death) was assessed.</p><p><strong>Results: </strong>Overall, 179 patients were included in the analysis (mPERR responders, n=128; non-mPERR responders, n=51). Most patients were female (87.2%); the mean (SD) age was 34.1 (11.3) years.Long-term renal survival was attained for 78.9% of mPERR responders and 60.8% of non-mPERR responders; achieving mPERR was associated with an increased likelihood of long-term renal survival versus not achieving mPERR (log-rank p=0.0119). Overall, 102 patients were mCRR responders, and 77 were non-mCRR responders. Long-term renal survival was attained for 80.4% of mCRR responders and 64.9% of non-mCRR responders; achieving mCRR was associated with an increased likelihood of long-term renal survival than not achieving mCRR (log-rank p=0.0259).</p><p><strong>Conclusions: </strong>Achieving mPERR or mCRR was associated with improved long-term renal survival, highlighting that these statuses are suitable predictors of long-term renal outcomes in patients with LN.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11603717/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142751331","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":"HNF-1β alleviates podocyte injury in lupus nephritis by maintaining endoplasmic reticulum homeostasis.","authors":"Hui-Mei Zou, Jie Yu, Yuan-Yuan Ruan, Ying Xie, Xiao-Min An, Pei-Lei Chen, Ying-Qin Luo, Ming-Jun Shi, Miao Liu, Li-Fen Xu, Jun Liu, Bing Guo, Fan Zhang","doi":"10.1136/lupus-2024-001349","DOIUrl":"10.1136/lupus-2024-001349","url":null,"abstract":"<p><strong>Objective: </strong>The current study aims to elucidate the critical function of hepatocyte nuclear factor 1-beta (HNF1-β) in lupus nephritis (LN) by investigating its modulation of the Derlin-1/valosin-containing protein (VCP)/VCP-interacting membrane selenoprotein (VIMP) complex, endoplasmic reticulum (ER) stress and podocyte apoptosis.</p><p><strong>Methods: </strong>In vitro and in vivo models of LN were established using glomerular podocytes treated with LN serum and MRL/lpr mice, respectively. The expression levels of HNF1-β were analysed in kidney tissues from patients with LN and MRL/lpr mice. To assess the effects of HNF1-β inhibition, an adeno-associated virus vector carrying HNF1-β short hairpin was administered to MRL/lpr mice. In vitro, glomerular podocytes were transfected with HNF1-β small interfering RNA (siRNA) or HNF1-β overexpression plasmids to explore their regulatory effects on the Derlin-1/VCP/VIMP complex and podocyte apoptosis. Dual-luciferase reporter assays and chromatin immunoprecipitation (ChIP) assays were performed to investigate the transcriptional activation of Derlin-1 and VCP promoters by HNF1-β.</p><p><strong>Results: </strong>A significant decrease in HNF1-β levels was observed in kidney tissues from patients with LN while MRL/lpr mice exhibited an initial compensatory increase followed by a subsequent decrease in renal HNF1-β expression. Overexpression of HNF1-β transcriptionally upregulated Derlin-1 and VCP mitigating LN serum-induced ER stress and podocyte apoptosis. In contrast, HNF1-β inhibition exacerbated renal dysfunction and structural damage in MRL/lpr mice. Interestingly, HNF1-β inhibition transcriptionally repressed ERP44, leading to calcium ions (Ca²⁺) release-mediated disruption and inactivation of the Derlin-1/VCP/VIMP complex. This finding suggests that HNF1-β not only regulates the expression of key proteins in the Derlin-1/VCP/VIMP complex but also influences their assembly through Ca²⁺ release regulation.</p><p><strong>Conclusion: </strong>This study provides novel insights into the regulatory mechanisms of HNF1-β in LN emphasising its impact on the Derlin-1/VCP/VIMP complex, ER stress and podocyte apoptosis. These findings have the potential to inform the development of new diagnostic tools and therapeutic strategies for LN.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11603702/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142751330","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":"Altered structural and functional homotopic connectivity associated with cognitive changes in SLE.","authors":"Sha Ni, Ning An, Chunlei Li, Yue Ma, Pengfei Qiao, Xueying Ma","doi":"10.1136/lupus-2024-001307","DOIUrl":"10.1136/lupus-2024-001307","url":null,"abstract":"<p><strong>Objective: </strong>Previous studies have revealed functional changes within the cerebral hemispheres of patients with SLE; however the changes between cerebral hemispheres are still unknown. The present study aimed to explore the functional and structural changes between bilateral hemispheres using functional MRI and find their relationship with cognition in patients with SLE.</p><p><strong>Methods: </strong>54 patients with SLE and 32 age-matched and sex-matched healthy controls (HCs) underwent MRI scanning and neuropsychological testing, and clinical data was collected in patients with SLE. Voxel-mirrored homotopic connectivity (VMHC) values and grey matter volume were calculated for all subjects. Correlation analysis was established to determine the relationship between VMHC values, grey matter volume and cognitive scores, blood biochemical markers in patients with SLE.</p><p><strong>Results: </strong>Compared with HCs, patients with SLE showed increased VMHC values in the insula and parahippocampal gyrus, while grey matter volume were reduced in these regions. Correlation analysis demonstrated that the increased VMHC values in insula was negatively correlated with decreased orientation function and positively correlated with decreased attention function. The grey matter volume in insula was negatively correlated with decreased attention and abstraction. The VMHC values and grey matter volume in insula and parahippocampal gyrus were negatively associated with lupus-specific antibodies.</p><p><strong>Conclusion: </strong>The structural and functional changes of insula and parahippocampal gyrus might be potential neuroimaging markers, and specific antibodies associated with lupus might be involved in the pathophysiological mechanisms of brain dysfunction.</p><p><strong>Trial registration number: </strong>NCT06226324.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-11-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11590855/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142710542","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":"Evaluation and randomised controlled trial of home urinalysis testing in patients with SLE at elevated risk for developing lupus nephritis: a study protocol.","authors":"Heather T Gold, Omar El Shahawy, Peter M Izmirly, Mala Masson, Brooke Cohen, Jill P Buyon","doi":"10.1136/lupus-2024-001390","DOIUrl":"10.1136/lupus-2024-001390","url":null,"abstract":"<p><strong>Introduction: </strong>Lupus nephritis (LN) is a frequent complication of SLE, occurring in up to 60% of adult patients and ultimately progressing from acute inflammation to chronicity with fibrosis and end-stage kidney failure in 10%-30% of patients. Racial/ethnic minority patients with lupus have worse long-term outcomes, including progression to end-stage renal disease and overall mortality. A major challenge in the management of patients with SLE is delayed identification of early kidney disease, which ultimately leads to a greater burden on both patients and the health system.</p><p><strong>Methods and analysis: </strong>Using a mixed methods approach, this study will develop, adapt and evaluate a home urine sampling protocol with a text-messaging reminder and data capture system for patients at elevated risk of de novo LN or relapse. First, a feasibility pilot using a single-group trial design (n=18) will be implemented, with a feasibility assessment and qualitative, debriefing interviews with patients to further refine the intervention. The second phase is a comparative effectiveness trial of the intervention (n=160) with the primary outcome of biopsy eligibility, that is, the participant has a clinical indication for a kidney biopsy (urine protein-creatinine ratio≥0.5), whether or not the patient actually undergoes the biopsy procedure. The randomised trial includes an economic evaluation of the adapted home urinalysis protocol.</p><p><strong>Discussion and dissemination: </strong>It is unknown whether weekly home-based urine sampling can identify proteinuria sooner than standard care; if found sooner, kidney problems could be diagnosed earlier, hopefully leading to earlier care for less-involved disease and subsequent reduced morbidity. The data collected in this trial will inform future feasibility and effectiveness of text-messaging-based home urine sampling interventions.</p><p><strong>Trial registration number: </strong>The randomised trial will be registered with ClincialTrials.gov prior to enrolment start.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11590779/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142693276","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":"Role of intravenous immunoglobulins in the management of systemic lupus erythematosus: a single-centre experience.","authors":"Mehmet Nur Kaya, Özlem Kılıç, Muhammet Canbaş, Merve Sungur Özgünen, Ezgi Çimen Güneş, Sedat Yılmaz","doi":"10.1136/lupus-2024-001402","DOIUrl":"10.1136/lupus-2024-001402","url":null,"abstract":"<p><strong>Objectives: </strong>Systemic lupus erythematosus (SLE) is a chronic autoimmune disease of unknown aetiology that can affect almost any organ in the body. Although there are no specific guidelines for the use of intravenous immunoglobulin (IG) in the treatment of patients with SLE, it is thought to be an effective treatment. Our study aimed to evaluate the effectiveness and safety of intravenous IG and to describe the possible profile of patients with SLE who are candidates for intravenous IG treatment.</p><p><strong>Methods: </strong>This study was designed to retrospectively analyse patients with SLE treated with 2 g/kg/month of intravenous IG (divided across 5 consecutive days). We collected demographic, clinical, laboratory and treatment data from the patient files. The side effects of the intravenous IG treatment, changes in the immunosuppressive therapy used and changes in the clinical and laboratory parameters after the intravenous IG treatment were evaluated.</p><p><strong>Results: </strong>This study included 31 patients with SLE. The main indication for intravenous IG treatment was haematological involvement (20, 64.5%) and thrombocytopenia in particular (8, 25.8%). Intravenous IG was initiated mainly for refractory disease. At the end of the treatment, the acute phase values, proteinuria, complement levels and anti-double-stranded DNA decreased significantly (p<0.001). In most cases, the side effects were mild and usually manifested as myalgia or a fever.</p><p><strong>Conclusion: </strong>Despite its high cost, intravenous IG has demonstrated effectiveness in treating refractory SLE, especially when there is haematological involvement. Specific clinical features at baseline may identify the patients who are more likely to respond to this therapy.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11580280/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142687468","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":"Myocardial Performance Index to assess cardiac function in autoimmune connective tissue disease: a systematic review and meta-analysis.","authors":"Rudy Hidayat, Sally Aman Nasution, Faisal Parlindungan, Naomi Niari Dalimunthe, Steven Alvianto, Nicolas Daniel Widjanarko, Ummi Kultsum, Cristopher Efendi, Yovita Gotama","doi":"10.1136/lupus-2024-001272","DOIUrl":"10.1136/lupus-2024-001272","url":null,"abstract":"<p><strong>Objectives: </strong>This study aimed to evaluate cardiac function using Myocardial Performance Index (MPI) in autoimmune connective tissue disease (ACTD) patients without cardiovascular abnormalities.</p><p><strong>Methods: </strong>A systematic search of databases including Medline, Google Scholar, ProQuest, Scopus and Cochrane Library was conducted to identify relevant studies on ACTD and MPI from 1995 to 2023. ACTD included in the search were rheumatoid arthritis (RA), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), Sjögren syndrome (SjD), polymyositis and dermatomyositis. Quality assessment was performed using the Newcastle-Ottawa Scale, followed by meta-analysis computation of mean differences (MDs) of MPI using Review Manager V.5.4.</p><p><strong>Results: </strong>A total of 22 studies for qualitative and 19 for quantitative synthesis were included. We found six studies on RA, eight studies on SSc, five studies on SLE, two studies on SjD and one on mixed connective tissue disorder. Conventional echocardiography and tissue Doppler imaging (TDI) were used to assess the MPI. Both conventional MPI and tissue Doppler MPI values were elevated compared with healthy control (MD=0.11, 95% CI 0.08 to 0.14, p value<0.00001 and MD=0.06, 95% CI 0.03 to 0.10, p value=0.00001, respectively).</p><p><strong>Conclusions: </strong>We found elevated MPI values in patients with ACTD compared with healthy controls. MPI assessment has the potential for early detection and management of cardiac dysfunction in patients with ACTD, but further studies are required to corroborate these findings.</p><p><strong>Prospero registration number: </strong>CRD42023490643.</p>","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"11 2","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11580312/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142687464","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}