Lupus最新文献

{"title":"Association of antiphospholipid syndrome with infective endocarditis and sepsis induced by Staphylococcus aureus: A U.S. nationwide inpatient sample analysis.","authors":"Bahy Abofrekha, Jessica Schwartz, Chloe Lahoud, Ahmad Mustafa, Chapman Wei, Salman Khan, Mitchell Weinberg, Martin Miguel Amor","doi":"10.1177/09612033251366391","DOIUrl":"10.1177/09612033251366391","url":null,"abstract":"<p><p>BackgroundAntiphospholipid syndrome (APS) predisposes patients to thrombosis and cardiac valve lesions such as Libman-Sacks endocarditis. These vegetations are sterile yet can provide a nidus for infection; the risk of infective endocarditis (IE) and other serious infections in APS patients remains poorly quantified in large populations, representing a knowledge gap.ObjectiveTo quantify the risk of the primary outcome, IE, and secondary outcomes of MRSA sepsis and MSSA sepsis, associated with APS using a large, nationally representative inpatient database.MethodsWe conducted a cross-sectional study using the National Inpatient Sample (NIS) database from 2016 to 2020. Hospitalized patients aged 18-75 with APS were compared to those without APS. Patients with major pre-existing risks for IE or significant confounders (e.g., prosthetic valves, specific congenital/rheumatic heart diseases, ESRD) were excluded. Multivariable logistic regression was used to calculate adjusted odds ratios (aORs) with 95% confidence intervals (CIs).Results297,459 patients fitted our inclusion criteria. On multivariate analysis, APS was significantly associated with over double the odds of IE (aOR 2.03; 95% CI 1.22-3.37). Importantly, APS also conferred considerably increased risks of MRSA sepsis (aOR 1.75; 95% CI 1.18-2.58) and MSSA sepsis (aOR 1.86; 95% CI 1.28-2.70).ConclusionAPS emerged as a significant independent risk factor for IE, Methicillin-resistant (MRSA), and Methicillin-sensitive <i>Staphylococcus aureus (MSSA)</i> sepsis. This suggests a broader vulnerability to infection, possibly linked to underlying endothelial dysfunction or immune dysregulation inherent in APS. These findings highlight the critical need for increased clinical suspicion, vigilant monitoring, and potentially tailored prophylactic or treatment approaches for severe infections in patients with APS.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1158-1165"},"PeriodicalIF":1.9,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144799522","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Overexpression of IRF8 and Pu.1 in B cells of systemic lupus erythematosus patients.","authors":"Amin Azizan, Elham Farhadi, Seyedeh Tahereh Faezi, Majid Alikhani, Ahmadreza Jamshidi, Mohammad Vodjgani, Mahdi Mahmoudi","doi":"10.1177/09612033251366400","DOIUrl":"10.1177/09612033251366400","url":null,"abstract":"<p><p>BackgroundSystemic Lupus Erythematosus (SLE), a complex autoimmune disorder characterized by altered immune regulation, particularly involving B cells, which exhibit increased survival and developmental dysregulation. This study aimed to investigate the expression of IRF4, IRF8, SP1, and PU.1 in B cells of SLE patients, as these factors are known to play critical roles in the development, function, and differentiation of B cells.MethodsB cells were isolated, cultured, and activated using anti-IgM. The mRNA expression of <i>IRF4</i>, <i>IRF8</i>, <i>SP1</i>, and <i>PU</i>.<i>1</i> was assessed using reverse transcription polymerase chain reaction (RT-PCR) at baseline and after B cell activation. Correlations between transcription factor expression and clinical parameters were analyzed.ResultsUpon B cell activation, <i>IRF4</i> expression increased significantly in SLE patients, unlike at baseline, where no changes were observed between groups. <i>IRF8</i> expression was significantly raised in active SLE and increased upon activation. <i>SP1</i> expression remained stable across all groups and conditions. <i>PU</i>.<i>1</i> gene expression was higher in active SLE at baseline and increased further upon B cell activation. Positive correlations were found between <i>IRF4</i> and <i>IRF8</i>, as well as between <i>PU</i>.<i>1</i> and <i>SP1</i>. <i>PU</i>.<i>1</i> expression correlated with SLE disease activity indices.Conclusion<i>IRF4</i>, <i>IRF8</i>, and <i>PU</i>.<i>1</i> expression in B cells is altered in SLE. <i>PU</i>.<i>1</i> shows a positive correlation with SLEDAI and anti-dsDNA titers. These findings highlight their potential roles in the pathogenesis of SLE in B cells.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1135-1146"},"PeriodicalIF":1.9,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144784625","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparison of clinicopathological characteristics and outcomes between patients with late and early-onset systemic lupus erythematosus with lupus nephritis: A North African study.","authors":"Sanda Mrabet, Raja Boukadida, Sidina Emah, Olfa Mahfoudh, Awatef Azzebi, Wissal Sahtout, Nesrine Thabet, Rihem Dahmene, Narjes Ben Aicha, Sonia Dziri, Dorsaf Zellama, Abdellatif Achour","doi":"10.1177/09612033251374770","DOIUrl":"10.1177/09612033251374770","url":null,"abstract":"<p><p>ObjectivesThe aim of this study was to compare the clinicopathological characteristics and outcomes of lupus nephritis (LN) between late-onset and early-onset systemic lupus erythematosus (SLE) patients.MethodsWe reviewed the clinical, serological and histological characteristics of all patients with LN admitted to our nephrology unit between 2007 and 2024. Our patients were divided into two groups according to their age at diagnosis: Early-onset SLE (younger than 50 years) and late-onset SLE (50 years or older).ResultsA total of 231 patients were recruited, of whom 43 had late-onset SLE and 188 had early-onset SLE. The mean age at diagnosis of SLE was 58.36 ± 7.61 years in the late-onset SLE group and 29.23 ± 9.03 years in the early-onset SLE group. There was no difference in the time from SLE diagnosis to LN. Compared with early-onset group, late-onset group had a higher prevalence of discoid rash but a lower frequency of leukopenia. Late-onset patients had higher serum creatinine levels and lower prevalence of anti-SSA and anti-RNP antibodies. The frequency of class VI LN was statistically significantly higher in the late-onset group. The use of oral corticosteroids, hydroxychloroquine and immunosuppressive drugs was significantly lower in late-onset SLE patients than in early-onset SLE patients. The latter were significantly less likely to progress to chronic kidney disease.ConclusionOur results indicate that SLE patients have different clinical, serological and histological manifestations depending on the age at onset of the disease. Late-onset SLE patients are more likely to have rheumatoid arthritis at onset. They have more severe chronic renal lesions and a worse renal outcome.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1305-1312"},"PeriodicalIF":1.9,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144959394","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epstein-Barr virus infection and its association with systemic lupus erythematosus: Systematic review and meta-analysis.","authors":"Shovit Ranjan, Sunil Kumar, Hrushikesh Nayak, Aditya K Panda","doi":"10.1177/09612033251371333","DOIUrl":"10.1177/09612033251371333","url":null,"abstract":"<p><p>BackgroundSystemic lupus erythematosus (SLE) is a chronic autoimmune disorder characterized by the production of autoantibodies that target most of the organ systems and lead to their dysfunction. The exact etiology of SLE remains unclear; however, genetic and environmental factors are believed to play significant roles. Viral infections, particularly Epstein-Barr virus (EBV), have been implicated as environmental triggers in SLE pathogenesis however, the observations remained inconsistent among studies and populations. The present study uses a meta-analysis approach to explore the prevalence of EBV infection in the general population and their role in the pathogenesis of SLE.Materials and MethodsVarious databases such as PubMed, Scopus, and ScienceDirect were searched to obtain eligible studies based on predetermined inclusion and exclusion criteria. The Newcastle-Ottawa Scale (NOS) was used for quality assessment of the eligible studies, and Comprehensive Meta-Analysis (CMA) v4 software was used for the analysis. Publication bias was assessed with funnel plots and Egger's regression, while heterogeneity was evaluated with Cochrane Q and I² statistics.ResultsIn the present investigation, a total of 28 studies comprising of 3926 healthy controls and 2968 SLE patients were included. EBV infections were prevalent in the healthy controls. While comparing the frequency of EBV DNA or antibodies positivity, the SLE patients had a higher positivity rate than the healthy controls, indicating that EBV infection is a risk factor for developing SLE. Furthermore, the sensitivity analysis also revealed that the meta-analysis was robust.ConclusionThe majority of healthy subjects were previously exposed to EBV, and the infection could be a potential risk factor in SLE pathogenesis. However, future research is required to elucidate the possible mechanisms of EBV reactivation in SLE patients and examine potential preventive measures, such as antiviral therapies, in mitigating SLE risk.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1261-1274"},"PeriodicalIF":1.9,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144959420","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Potential involvement of IL-40 in kidney disease associated to systemic lupus erythematosus.","authors":"Chiara Rizzo, Lidia La Barbera, Marianna Lo Pizzo, Federica Camarda, Leila Mohammadnezhad, Francesco Dieli, Francesco Ciccia, Serena Fasano, Giuliana Guggino","doi":"10.1177/09612033251361037","DOIUrl":"10.1177/09612033251361037","url":null,"abstract":"<p><p>ObjectivesAim of this study was to investigate the expression of interleukin (IL)-40, a cytokine associated with B cells homoeostasis and immune response, in Systemic lupus erythematosus (SLE) and SLE associated nephritis.Methods32 patients with SLE and 21 controls were enrolled. Serum concentration of IL-40 was assessed by ELISA and cellular sources of IL-40 were determined by flow-cytometry. An in vitro assay with recombinant IL-40 (rIL-40) was performed to detect the effect on cytokine production from peripheral blood mononuclear cells (PBMC). In kidney biopsies obtained from patients with lupus nephritis (LN) immunofluorescence was performed to assess IL-40 expression and detect its cellular sources at tissue level.ResultsSerum IL-40 levels show a decreasing trend in SLE with LN, compared with SLE without LN and controls. No differences between SLE and controls were evidenced in urinary IL-40 levels; in the percentage of CD3⁺IL40⁺, CD19⁺IL40⁺ and CD14⁺IL40⁺; and in the production of pro-inflammatory cytokines by PBMC stimulated with rIL-40. IL-40 expression was markedly increased in kidney tissue from LN patients, with a significant increase of IL40⁺ cells in LN samples compared to controls, as confirmed by the quantification analysis.ConclusionTo the best of our knowledge this is the first demonstration of IL-40 expression at kidney level in SLE. These preliminary data suggest a possible role of IL-40 in LN. In particular, IL-40 could be a marker of kidney tissue damage, although its specific mechanism of action needs to be further elucidated.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1178-1183"},"PeriodicalIF":1.9,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144659574","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical, pathological and immunological features of toxic-epidermal necrolysis like lupus erythematosus: A systematic review and proposal for diagnostic criteria.","authors":"Sweta Subhadarshani, Taylor Griffith, Zachary Thornton, Andrew Nicholas","doi":"10.1177/09612033251371801","DOIUrl":"10.1177/09612033251371801","url":null,"abstract":"<p><p>BackgroundToxic epidermal necrolysis like lupus erythematosus (TEN-like LE) is a dermatological emergency associated with significant morbidity and mortality. It requires prompt recognition and differentiation from its mimics including drug induced toxic epidermal necrolysis, erythema multiforme, drug rash with eosinophilia and systemic symptoms, and reactive infectious mucocutaneous eruptions.ObjectiveTo assess systemically the clinical, pathological, and immunological features of patients with TEN-like LE.MethodsWe performed a structured search systematic review from April-May 2024, of studies on TEN-like LE published between 1977 and 2024., searching PubMed, Medline, Embase, SCOPUS, VHL, ProQuest, clinicaltrials.gov, COCHRANE, POPLINE, ISI, mRCT, UC library, and ResearchGate.ConclusionThere were 16 case series and 52 case reports/conference papers yielding 112 unique cases. The mean age was 43.39 years, and 86.6% of cases were female. TEN-like LE was the presenting manifestation of lupus erythematosus (LE) in 48.21% of patients. Antinuclear antibody (ANA) was positive in all 109 patients where it was reported with SSA/Ro being most commonly positive (60.55%), followed by dsDNA (50%). Lesions were reported to be photodistributed in 78.57%, palmoplantar involvement was seen in 20.54%, and mucosal involvement was seen in 56.25%, with 26.78% patients having significant mucosal involvement. Lupus non-specific lesions were observed in 25% patients. Anemia, pulmonary and GI involvement were positively associated with hemodynamic instability, and anti-Ro antibodies were negatively associated. Positive dsDNA, La positivity, and low C4 were associated with increased odds of systemic involvement, whereas Ro positivity was associated with reduced odds. Seven patients had a paraneoplastic association. TEN-like LE can be the first manifestation of LE in up to half of cases. Photodistribution, lupus non-specific lesions, ANA positivity, or change in ANA type/titre can be important diagnostic clues. Unlike previously believed, mucosal involvement is not uncommon and can be severe.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":"34 12","pages":"1221-1229"},"PeriodicalIF":1.9,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12464354/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145186191","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Level of CD4⁺ central memory T cells and its clinical correlation in childhood-onset systemic lupus erythematosus.","authors":"Feng-Qiao Gao, Xiao-Zhen Zhao, Shi-Peng Li, Chao Li, Jiang-Hong Deng, Jun-Mei Zhang, Xiao-Hua Tan, Xuan-Yi Liu, Cai-Feng Li","doi":"10.1177/09612033251366398","DOIUrl":"10.1177/09612033251366398","url":null,"abstract":"<p><p>ObjectivesThis study aims to investigate CD4⁺ central memory T cells (CD4⁺ TCM) levels in childhood-onset systemic lupus erythematosus (cSLE) and their association with disease activity, clinical features, and treatment responses.MethodsA total of 202 children with newly diagnosed, untreated rheumatic diseases were recruited, comprising 64 cases of cSLE, 71 cases of juvenile idiopathic arthritis, 31 cases of juvenile dermatomyositis, 36 cases of autoinflammatory diseases, and 22 healthy controls. Lymphocyte subsets were analyzed using multi-color flow cytometry, and clinical data and laboratory test results were collected. The correlation between CD4⁺ TCM levels and SLEDAI scores, clinical manifestations, autoantibodies, and kidney injury markers was examined. Subsequently, 21 cSLE patients underwent follow-up assessments and retesting post-treatment.ResultsThe proportion of CD4⁺ TCM (44.3 ± 11.5%) in cSLE was significantly higher compared to those with other pediatric rheumatic diseases (<i>p</i> < .05). A negative correlation was observed between the level of CD4⁺ TCM and the SLEDAI-2000 score (r = -0.255, <i>p</i> = .021), indicating that higher disease activity was associated with lower CD4⁺ TCM levels. Furthermore, CD4⁺ TCM levels were negatively correlated with oral ulcers (r = -0.285, <i>p</i> = .011) and positively correlated with leukopenia (r = 0.302, <i>p</i> = .008). In terms of laboratory indicators, CD4⁺ TCM showed negative correlations with anti-dsDNA antibodies (r = -0.294, <i>p</i> = .009) and anti-histone antibodies (r = -0.232, <i>p</i> = .033), while exhibiting a positive correlation with anti-Sm antibodies (r = 0.245, <i>p</i> = .025). Additionally, CD4⁺ TCM demonstrated significant negative correlations with early renal injury markers, urinary transferrin (r = -0.315, <i>p</i> = .008), and urinary microalbumin (r = -0.284, <i>p</i> = .015). CD4⁺ TCM was strongly negatively correlated with CD4⁺ Naive cells (r = -0.831, <i>p</i> < .001), positively correlated with other memory cell subsets, and negatively correlated with IFN-α levels (r = -0.364, <i>p</i> = .031). Longitudinal analysis revealed a time-dependent biphasic pattern in CD4⁺ TCM levels. Cyclophosphamide-treated patients showed significantly increased CD4⁺ TCM levels compared to non-cyclophosphamide groups (<i>p</i> = .034).ConclusionsCD4⁺ TCM likely plays a central immune regulatory role in cSLE, with its levels closely associated with disease activity, specific autoantibody production, and early organ damage. Post-treatment changes in CD4⁺ TCM levels may indicate therapeutic efficacy and suggest their potential as biomarkers, offering a fresh perspective on immune memory regulation in cSLE and exploring novel treatment approaches.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1166-1177"},"PeriodicalIF":1.9,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144775773","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Echocardiographic assessment of pediatric lupus nephritis and its correlation with disease activity in a tertiary care hospital.","authors":"Afroza Begum, Amina Akter, Nadira Sultana, Abdullah Al Mamun, Tahmina Jesmin, Syed Saimul Huque, Shanjida Sharmim, Sharmin Akter Luna, Shamsunnahar Shanta, Samina Masud Santa, Sabina Sultana, Ranjit Roy","doi":"10.1177/09612033251367175","DOIUrl":"10.1177/09612033251367175","url":null,"abstract":"<p><p>Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multisystem involvement. Lupus Nephritis (LN) is the most serious presentation. Cardiovascular complications in SLE and LN can cause significant morbidity and mortality. An echocardiographic (ECHO) screening allows early detection and intervention, so that early treatment can be provided.MethodThis cross-sectional analytical study evaluated 50 children with LN at the Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from January 2017 to July 2023. All patients fulfilled the Systemic Lupus International Collaborating Clinics (SLICC, 2012) criteria for SLE diagnosis and had evidence of renal involvement. Cardiac evaluation was performed by color doppler echocardiography. Systemic lupus erythematosus disease activity index (SLEDAI) was used to see the disease activity.ResultsThe mean age of presentation was 12.4 ± 2.79 years, with a male-to-female ratio of 1:3. Cardiac abnormalities were found in 20 patients (40%), with tricuspid regurgitation being the most frequent (38%), followed by pericardial effusion (28%), pulmonary hypertension (24%), and mitral, aortic and pulmonary regurgitation in descending order. Abnormal echocardiographic findings were significantly associated with higher SLEDAI scores and lower C3 levels.ConclusionsCardiac abnormalities are prevalent in pediatric LN and valvular dysfunction particularly tricuspid regurgitation being the most common followed by pericardial effusion and pulmonary hypertension. These abnormalities correlate with higher disease activity and lower C3 levels, emphasizing the critical need for routine echocardiographic screening for early detection and management.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1285-1291"},"PeriodicalIF":1.9,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144804397","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neonatal ischemic stroke potentially associated with transplacental passage of maternal anti-cardiolipin immunoglobulin G antibodies: A case report.","authors":"Hiroshi Oiwa, Hideyuki Hawaka, Yuichiro Fujieda, Naoko Ueno, Seishi Sumi","doi":"10.1177/09612033251386089","DOIUrl":"https://doi.org/10.1177/09612033251386089","url":null,"abstract":"<p><p>A baby girl, born to a 35-year-old mother, was admitted at 14.5 hours of life due to apnoea and seizures. Diffusion-weighted magnetic resonance imaging revealed multiple ischemic lesions. Laboratory screening for thrombophilia showed elevated levels of anti-cardiolipin IgG antibodies (aCL-IgG). The mother also tested positive for aCL-IgG, raising the possibility of transplacental antibody transfer. The neonate's aCL-IgG levels gradually declined and normalized by 4 months of age. Although causality cannot be established, this case suggests a potential association between transient maternal antiphospholipid antibodies and neonatal ischemic stroke.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251386089"},"PeriodicalIF":1.9,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145186150","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Longitudinal assessment of disease burden in juvenile systemic lupus erythematosus: A multicenter study of activity and damage scores.","authors":"Duygu Aydın, Eray Tunce, Gülşah Kavrul Kayaalp, Hande Ilgaz Tüzen, Dorukcan Alkan, Görkem Oğuz, Yasin Karali, Sıla Atamyıldız Uçar, Gülçin Otar Yener, Hatice Kübra Dursun, Tolga Kasap, Mustafa Çakan, Ferhat Demir, Hatice Adıgüzel Dündar, Burcu Bozkaya Yücel, Figen Çakmak, Nihal Şahin, Selçuk Yüksel, Kübra Öztürk, Sara Sebnem Kilic, Serkan Türkuçar, Semanur Özdel, Belde Kasap Demir, Nuray Aktay Ayaz, Betül Sözeri, Hafize Emine Sönmez","doi":"10.1177/09612033251386091","DOIUrl":"https://doi.org/10.1177/09612033251386091","url":null,"abstract":"<p><p>IntroductionJuvenile Systemic Lupus Erythematosus (jSLE) is a rare pediatric rheumatic disease characterized by systemic inflammation that can lead to organ damage. Compared to adults, it often has a more severe course in children. Both disease activity and treatments may result in temporary or permanent damage.ObjectivesTo evaluate risk factors associated with damage occurrence in patients with jSLE.MethodsThis multicenter, retrospective study included patients with jSLE followed for at least 12 months. Low-dose corticosteroid therapy was defined as prednisolone 0.01-0.03 mg/kg/day (max 7.5 mg/day). The annual cumulative steroid dose was calculated by dividing the total steroid intake by 365.25 times the number of follow-up years. Collected data included SLEDAI and SDI scores at initial and final visits, laboratory parameters, and flare characteristics.ResultsA total of 158 patients (86.7% female) from 17 centers were included. Median age at diagnosis was 13.8 years, with a median follow-up of 35 months. Organ damage was present in 14 patients at diagnosis and in 23 at final visit. Damage types included proteinuria, cognitive dysfunction (each 3.2%), and others such as cataracts, erosive arthritis, avascular necrosis, optic atrophy, and vertebral collapse. Patients with damage had significantly higher SLEDAI scores at both time points, delayed transition to low-dose steroids, and a lower rate of achieving Lupus Low Disease Activity State (LLDAS) (<i>p</i> = .006).ConclusionPersistent disease activity and delayed control are major contributors to organ damage in jSLE. Early and sustained disease suppression is critical to prevent long-term complications.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251386091"},"PeriodicalIF":1.9,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145186169","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}