Lupus最新文献

{"title":"Clinical and laboratory differences in pediatric-onset versus adult-onset lupus nephritis: A retrospective cohort analysis.","authors":"Joanna Kosałka-Węgiel, Radosław Dziedzic, Andżelika Siwiec-Koźlik, Magdalena Spałkowska, Lech Zaręba, Stanisława Bazan-Socha, Mariusz Korkosz","doi":"10.1177/09612033251352706","DOIUrl":"10.1177/09612033251352706","url":null,"abstract":"<p><p>IntroductionLupus nephritis (LN) is a severe manifestation of systemic lupus erythematosus (SLE) and is associated with significant morbidity and mortality. However, the course of LN that develops at a younger age is less well understood. Therefore, we evaluated the clinical and laboratory characteristics of adult LN patients and compared them, depending on whether the disease started in childhood (pediatric-onset LN [pLN]) or in adulthood (adult-onset LN [aLN]).Patients and MethodsWe retrospectively analyzed the medical records of all adult LN patients treated at the University Hospital in Kraków, Poland, from 2012 to 2022. All included patients met the European League Against Rheumatism/American College of Rheumatology criteria for SLE from 2019.ResultsAmong 343 LN patients, pLN was stated in 46 cases (13.41%). pLN and aLN had a comparably high female-to-male ratio (6.67 vs 4.82, respectively; <i>p</i> = 0.07) and similarly often presented with more advanced kidney involvement in histology. In turn, lupus malar rash and serositis were more frequent in pLN than aLN (<i>p</i> = 0.048 and <i>p</i> = 0.015, respectively). End-stage kidney disease (ESKD) was documented in pLN patients 2.72 times more frequently (<i>p</i> = 0.036); however, the person-year rate did not differ. Interestingly, in pLN we reported a more common presence of anti-cardiolipin antibodies in the IgM class (<i>p</i> = 0.042). The occurrence of other SLE-specific autoantibodies did not differ between both analyzed groups. Regarding immunosuppressive treatment, glucocorticosteroids were the most frequently utilized in both subgroups. Still, pLN cases were more frequently treated with chloroquine or hydroxychloroquine, azathioprine, and immunoglobulins than aLN patients (<i>p</i> < 0.05, for all).ConclusionspLN is characterized by more severe SLE clinical manifestations. Therefore, SLE patients who developed LN in childhood likely require more aggressive immunosuppressive treatment.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1039-1048"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144484916","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":"Mortality among inpatients with systemic lupus erythematosus in a tertiary care hospital.","authors":"Mohan Kumar Hanumanthappa, Manisha Gulia, Vikas Sharma, Navneet Arora, Godasi Srsnk Naidu, Valliappan Muthu, Vishal Sharma, Varun Dhir, Manish Rathi, Harbir Singh Kohli, Sanjay Jain, Aman Sharma","doi":"10.1177/09612033251360287","DOIUrl":"10.1177/09612033251360287","url":null,"abstract":"<p><p>BackgroundSystemic lupus erythematosus (SLE) is associated with significant morbidity and mortality, particularly during hospitalisation. Differentiating between infection and disease activity is crucial but challenging. Regional variations in infection rates and disease manifestations necessitate region-specific studies.ObjectivesTo evaluate whether infections at or during hospitalisation are associated with increased mortality among SLE patients and identify additional predictors of adverse outcomes.MethodsThis retrospective case-control study included SLE patients hospitalised between January 2012 and December 2021. Cases (<i>n</i> = 111) were patients who expired during hospitalisation, matched 1:2 with controls (<i>n</i> = 222) discharged alive, based on age (±3 years) and sex, stratified annually. SLE diagnosis was based on ACR 1997 or SLICC 2012 criteria. COVID-19 cases were excluded. Patients were categorised by admission cause as 'Infection', 'Disease-associated', or 'Mixed'. Data on demographics, clinical features, laboratory parameters, and treatments were extracted, and multivariable logistic regression identified independent predictors of mortality.ResultsThe hospital mortality rate was 8.9%. Infection (with or without disease activity) significantly contributed to hospital admissions among non-survivors (57%) compared to survivors (25%; <i>p</i> < 0.001). Acinetobacter baumannii was the most frequent pathogen. Multivariable analysis showed infection at hospitalisation (OR 3.37, 95% CI 1.85-6.13), pulmonary involvement (OR 3.06, 95% CI 1.52-6.18), cardiac involvement (OR 2.13, 95% CI 1.07-4.25), and serum creatinine levels as independent predictors of mortality. Higher serum albumin was protective (OR 0.53, 95% CI 0.35-0.79). Juvenile lupus subgroup analysis (<i>n</i> = 38) revealed similar infection-related mortality patterns.ConclusionsOur study highlights the significant impact of infections, particularly hospital-acquired infections, on mortality among hospitalised SLE patients. Enhanced clinical vigilance, early interventions, and rigorous infection control measures are needed to improve outcomes in hospitalised SLE patients.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1080-1087"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144642911","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":"Immunosuppressive therapy withdrawal in lupus nephritis, is it possible?: A systematic review.","authors":"Stefanus Gunawan Kandinata, Awalia, Yuliasih, Lita Diah Rahmawati, Cahyo Wibisono Nugroho, Arinditia Triasti Putri, Angela Kimberly Tjahjadi, Mega Kahdina, Mandana Nikpour","doi":"10.1177/09612033251352709","DOIUrl":"10.1177/09612033251352709","url":null,"abstract":"<p><p>IntroductionMaintenance of immunosuppressants (IST) is critical for sustaining remission in lupus nephritis (LN) patients. However, long-term use is associated with an increased risk of side effects such as infection. Yet, early IST withdrawal also poses a high risk of flare. This study aims to provide an overview of the impact of optimal IST withdrawal in patients with LN who have achieved remission.MethodWe conducted a systematic review of randomized controlled trials (RCTs) and observational studies regarding the discontinuation of IST in patients with proliferative LN who had been in remission for at least 1 year. Data from PubMed, ProQuest, and Web of Science were extracted on patient demographics, baseline characteristics, treatment regimens, and outcomes, including flare rates, renal function, and biopsy findings. The risk of bias was assessed using the Newcastle-Ottawa Scale and the JADAD Score.ResultsFive studies with 310 patients were included. The mean age of participants ranged from 26 to 38 years. Overall, flares following IST withdrawal occurred in an average of 28.7% of patients. Between the two groups (flare and no-flare), baseline serum creatinine was comparable, but baseline proteinuria and C3 & C4 levels were varied across studies. The duration of lupus before study entry was approximately 4-10 years, with a duration of complete remission of 12-59.5 months before IST withdrawal. Follow-up periods ranged from 24 to 215 months. The Biopsy Activity Index and Chronicity Index at baseline also showed variation but generally indicated a higher level of chronic damage in the flare group.DiscussionDiscontinuation of IST is feasible but may be associated with an increased risk of severe flares, often requiring reintroduction of induction therapy. Careful assessment and monitoring of both histologic and clinical activity are essential when evaluating remission and considering IST withdrawal, as a low activity index may guide safer withdrawal strategies.ConclusionIST withdrawal is feasible in patients with LN who have achieved remission, but careful monitoring is required due to the risk of relapse and potential progression of chronic kidney damage. Histological confirmation and predictive tools may support safer withdrawal decisions.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1003-1012"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144317338","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":"Intravenous immunoglobulin as a treatment for severe, refractory cutaneous lupus erythematosus requiring hospitalization: Three cases and review of the literature.","authors":"Srona Sengupta, Chirag Vasavda, Sach Thakker, Andrea Fava, Nayimisha Balmuri, Ana-Maria Orbai, Jun Kang","doi":"10.1177/09612033251352711","DOIUrl":"10.1177/09612033251352711","url":null,"abstract":"","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1088-1094"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144575797","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":"Dual benefit of belimumab in post-transplant lupus nephritis: Glucocorticoid withdrawal and extrarenal disease control.","authors":"Irene Martin Capon, J F Colina-García, Ana Huerta, Susana Mellor, María Galindo, Enrique Morales","doi":"10.1177/09612033251353088","DOIUrl":"10.1177/09612033251353088","url":null,"abstract":"<p><p>IntroductionLupus nephritis (LN), a severe organ impairment associated with systemic lupus erythematosus (SLE), significantly affects patient prognosis. Despite therapeutics advances, up to 15% of patients progress to end-stage kidney disease (ESKD) within 15 years, with kidney transplantation emerging as the preferred renal replacement therapy. Conversely, belimumab has shown promise in enhancing remission rates and improving renal outcomes in LN. however, there is little information in the literature regarding the use of belimumab in kidney transplant patients and its potential interaction with other immunosuppressive therapies.MethodsThis report presents three clinical cases of renal transplant recipients with LN who received belimumab following transplantation to manage their systemic disease The aim of this case report was to evaluate the efficacy and safety of belimumab administration in renal transplant patients.ResultsBelimumab successfully discontinued or reduced the dose of glucocorticoids, resolved the clinical manifestations of SLE and maintained stable renal function throughout follow-up.ConclusionThese cases highlight belimumab's potential as an effective and safe therapeutic option in managing extrarenal SLE symptoms and reducing glucocorticoid dependence in kidney transplant recipients. Although limited data exists, these findings align with previous evidence supporting belimumab's safety and efficacy in LN.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1067-1072"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144528621","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":"COVID-19 vaccine-induced systemic lupus erythematosus: The underlying immunological and genetic mechanisms.","authors":"Ping Wang, Shanzhao Jin, Xue Li, Huaqun Zhu, Fanlei Hu, Zhanguo Li","doi":"10.1177/09612033251357627","DOIUrl":"10.1177/09612033251357627","url":null,"abstract":"<p><p>ObjectiveSystemic lupus erythematosus (SLE) is complexed with multi systemic involvements associated with genetic and environmental factors. Herein, we delve into the molecular mechanism behind COVID-19 vaccine-induced SLE.MethodsTo elucidating the molecular mechanism, we employed the whole genome sequencing (WGS) and immune cell RNA sequencing of CD4⁺ T, CD8⁺ T, and B cells.ResultsOur findings suggest that in genetically predisposed individuals, COVID-19 vaccination might provoke SLE by triggering host-harbored virus signaling and cytokine pathway-associated immune response.ConclusionsThis study deepens the underlying mechanism and highlights the importance of evaluating the genetic susceptibility to autoimmune disease before COVID-19 vaccination.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1061-1066"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144659573","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":"Anti-LGI1 antibody-positive autoimmune encephalitis concurrent with neuropsychiatric systemic lupus erythematosus.","authors":"Atsuhiko Sunaga, Takashi Kida, Shunsuke Fujieda, Shunya Kaneshita, Yuta Kojima, Takahiro Seno, Makoto Wada, Masataka Kohno, Yutaka Kawahito","doi":"10.1177/09612033251356106","DOIUrl":"10.1177/09612033251356106","url":null,"abstract":"<p><p>Anti-leucine-rich glioma-inactivated protein 1 (LGI1) antibody-positive limbic encephalitis is a form of autoimmune encephalitis (AE). The importance of anti-LGI1 antibody in neuropsychiatric systemic lupus erythematosus (NPSLE) remains unclear. We present a 65-year-old woman with amnesia and disorientation, positive anti-LGI1 and anti-Smith antibodies, hyponatremia, leukopenia, and hypocomplementemia. Magnetic resonance imaging findings were consistent with limbic encephalitis and cervical myelitis. She was diagnosed with NPSLE with anti-LGI1-positive AE. High-dose glucocorticoids and intravenous cyclophosphamide led to sustained remission. This case suggests that NPSLE may coexist with anti-LGI1-positive AE, highlighting the potential importance of anti-LGI1 antibody testing in NPSLE and expanding the management of AE.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1073-1079"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144608718","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 profile and risk factors of posterior reversible encephalopathy syndrome in systemic lupus erythematosus: A case-control study.","authors":"Kaustav Bhowmick, Rashmi Roongta, Sumantro Mondal, Dipendra Nath Ghosh, Soumya Dey, Gautam Raj Panjabi, Angan Karmakar, Mavidi Sunil Kumar, Sonali Dey, Sudipta Ghorai, Partha Ghorai, Hiramanik Sit, Subhankar Haldar, Pradyot Sinhamahapatra, Biswadip Ghosh, Geetabali Sircar, Parasar Ghosh","doi":"10.1177/09612033251366401","DOIUrl":"10.1177/09612033251366401","url":null,"abstract":"<p><p>ObjectivesTo investigate the clinical profile and risk factors of Posterior Reversible Encephalopathy Syndrome (PRES) in patients with Systemic Lupus Erythematosus (SLE).MethodsIn this retrospective study, the SLE patients admitted with PRES in the Department of Clinical Immunology and Rheumatology, IPGME&R, Kolkata, India from January 1, 2017 to January 31, 2024, were identified. Fifty-three control participants with neuropsychiatric symptoms as per the 1999 American College of Rheumatology case definition criteria were selected from the departmental SLE cohort.Results26 episodes of PRES were identified in 25 patients. Seizures (88.46%) and headaches (53.85%) were the commonest presenting manifestations with the occipital (96.15%) and parietal lobes (84.62%) being the commonly involved sites. Nephritis (96.15% vs 60.38%, <i>p</i> = 0.001), hypertension at PRES onset (76.92% vs 18.87%, <i>p</i> = 0.000), Direct Coomb's test (DCT) positivity (50.00% vs 20.75%, <i>p</i> = 0.008), high extra-neurological disease activity (SELENA-SLEDAI - <i>N</i>: 20.62±8.77 vs 14.15 ± 5.51, <i>p</i> = 0.002), lupus enteritis (15.38% vs 1.89%, <i>p</i> = 0.038), and neutrophilia (73.08% vs 20.75%, <i>p</i> = 0.000) were identified as risk factors for PRES on univariate analysis. Multivariate analysis found hypertension [<i>p</i> = 0.014], DCT positivity (<i>p</i> = 0.013), neutrophilia (<i>p</i> = 0.025), and lupus enteritis (0.038) as independent risk factors. The presence of U1RNP/Sm and prior thrombotic events were found to be protective against PRES on univariate analysis.ConclusionPRES in SLE in our study was associated with high extra-neurological disease activity. Hypertension, DCT positivity, neutrophilia, and lupus enteritis were identified as important risk factors for the condition.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1029-1038"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144775772","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":"CAR-modified cells and the sisyphus myth: A new panacea for lupus?","authors":"Carlos Antonio Moura, Lucas Gonçalves, Tiago Thalles de Freitas, Carlos Geraldo Moura, Luiz Henrique de Assis","doi":"10.1177/09612033251356442","DOIUrl":"10.1177/09612033251356442","url":null,"abstract":"","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1095-1097"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144528620","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":"Primary antiphospholipid syndrome in pediatrics: New criteria, new opportunities.","authors":"Alfonso Ragnar Torres Jimenez, Berenice Sanchez Jara, Adriana Ivonne Cespedes Cruz, Virginia Ramirez Nova, Alejandra Velazquez Cruz, Guadalupe Del Consuelo Cortina Olvera, Vilma Carolina Bekker Méndez, Francisco Xavier Guerra Castillo","doi":"10.1177/09612033251361040","DOIUrl":"10.1177/09612033251361040","url":null,"abstract":"<p><p>ObjectiveWe present the clinical, laboratory, and treatment characteristics of patients diagnosed with primary antiphospholipid syndrome according to the 2023 ACR/EULAR APS classification criteria.Material and methodsA retrospective study was conducted in patients under 18 years of age, diagnosed with primary antiphospholipid syndrome according to the 2023 ACR/EULAR classification criteria, at the General Hospital of the National Medical Center La Raza, Mexico, from January 2013 to January 2025. Data on clinical manifestations, laboratory, and treatment were collected.ResultsWe present data from 40 patients, 22 female and 18 male, with a mean age at diagnosis of 12.8 years. The time to diagnosis was 15.4 weeks. Thrombosis occurred in 14 patients. Thrombocytopenia in 34. Autoimmune hemolytic anemia in 18, Fisher Evans syndrome in 17. Livedo in 33, skin ulcers in 2, and Raynaud's phenomenon in 15. Epileptic seizures in 2 and chorea in 1. We did not find cardiac valvular involvement. Renal involvement was found in 4. Prolonged aPTT in 39 (97.5%), positive lupus anticoagulant in 39, positive anticardiolipin IgG in 32, positive anticardiolipin IgM in 18, anti-B2GPI IgG performed in 17 patients, positive in 15, anti-B2GPI IgM performed in 12, positive in 4. Treatment consisted of anticoagulation in 13 patients, antiplatelet agents in 29, steroids in 35 immunosuppressants in 33, and rituximab in 6. One patient died from an alveolar hemorrhage.ConclusionsThe 2023 ACR/EULAR APS criteria improve the classification of pediatric patients by including non-thrombotic criteria; however, there are other non-thrombotic manifestations that are common in children that should be taken into account in this population.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1024-1028"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144618729","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}