Lupus最新文献

{"title":"SLE inflammatory musculoskeletal abnormalities, confirmed by MRI, show a specific profile with a worse health-related quality of life.","authors":"Patricia Corzo, Anna Agustí Claramunt, Ivan Garcia-Duitama, Irene Carrión-Barberá, Salvatore Marsico, Xavier Duran Jordà, Jordi Monfort Faure, Tarek-Carlos Salman-Monte","doi":"10.1177/09612033241301515","DOIUrl":"https://doi.org/10.1177/09612033241301515","url":null,"abstract":"<p><strong>Objectives: </strong>To determine if there is a clinicodemographic or serological profile associated with MRI-confirmed inflammatory musculoskeletal abnormalities in SLE patients. To investigate the relationship between these alterations and HRQoL.</p><p><strong>Methods: </strong>patients with SLE from our previous study in whom a wrist and hand MRI with contrast was performed were included. Sociodemographic, clinical, therapeutic, serological data and PROs were collected and correlated with MRI findings.</p><p><strong>Results: </strong>83 patients were analysed. Erosions and synovitis were more common in older patients (55 ± 12.61 vs 45.06 ± 12.18 years, <i>p</i> .001, 52.78 ± 12.99 vs 44.95 ± 12.49 years, <i>p</i> .011). Synovitis was less frequent in patients with nephritis (6.7% vs 24.3%, <i>p</i> .031). Treatment received showed some associations: patients with bone edema received more methotrexate (25% vs 6.3%, <i>p</i> .033), those with erosions and peritendonitis received less mycophenolic acid (5.6% vs 22.9%, <i>p</i> .034; 0% vs 12.8%, <i>p</i> .026). Peritendonitis correlated with higher SLEDAI-2K (7 ± 2.45 vs 3.64 ± 3.34, <i>p</i> .018).</p><p><strong>Worse haq: </strong>Patients with synovitis, tenosynovitis, peritendonitis and bone edema reported higher pain (6.03 ± 2.57 vs 4.26 ± 2.49, <i>p</i> .005; 6.56 ± 1.95 vs 4.76 ± 2.75, <i>p</i> .017; 8.80 ± 1.30 vs 4.95 ± 2.55, <i>p</i> .001; 6.47 ± 2.62 vs 4.83 ± 2.58, <i>p</i> .026, respectively). Patients with synovitis reported higher fatigue numerical values (2.32 ± 0.82 vs 1.91 ± 0.84, <i>p</i> .035), with tenosynovitis worse FSS-9 (61.50 ± 1.73 vs 45.70 ± 16.80, <i>p</i> .015), and with both synovitis and peritendonitis worse HAQ (1.14 ± 0.69 vs 0.75 ± 0.65, <i>p</i> .031; 1.69 ± 0.07 vs 0.90 ± 0.69, <i>p</i> .018).</p><p><strong>Conclusion: </strong>SLE patients with confirmed musculoskeletal alterations on MRI were generally older, less likely to have lupus nephritis, and received different treatments. They reported a worse HRQoL in terms of pain, fatigue and functional disability.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033241301515"},"PeriodicalIF":1.9,"publicationDate":"2024-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142676166","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":"A rare case of late-onset spondyloenchondrodysplasia with immune dysregulation presenting as adult-onset monogenic lupus.","authors":"Esra Firat Senturk, Bilal Berke Ayvaz, Sinem Firtina, Serdal Ugurlu","doi":"10.1177/09612033241301506","DOIUrl":"10.1177/09612033241301506","url":null,"abstract":"<p><strong>Background: </strong>Spondyloenchondrodysplasia is classified as an interferonopathy resulting from recessive mutations in the <i>ACP5</i> gene and manifests with various clinical features, including distinctive skeletal dysplasia, neurological abnormalities, immune dysfunction resembling systemic lupus erythematosus (SLE) and Sjogren's syndrome. While SLE is typically considered multifactorial and more prevalent in adulthood, a subset of approximately 10%-25% of childhood cases arise from monogenic form. Among these, spondyloenchondrodysplasia accounts for only a rare fraction of monogenic lupus cases, with only 22 reported instances in the literature.</p><p><strong>Case report: </strong>This paper presents a new case of spondyloenchondrodysplasia-immune dysregulation (SPENCDI) in an adult patient born to nonconsanguineous parents. The patient was diagnosed with SPENCDI and exhibited immune neutropenia, anti-dsDNA positivity, platyspondyly, immune deficiency, and a homozygous variant (c.155 A > C, p. Lys52Thr) in the <i>ACP5</i> gene, previously classified as pathogenic. Notably, the patient achieved successful clinical management through the initiation of baricitinib treatment, a Janus kinase inhibitor.</p><p><strong>Conclusion: </strong>SPENCDI represents an uncommon cause of SLE in adulthood. Clinicians should be vigilant of underlying Mendelian inheritance when encountering patients with associated features. While treatments for both Mendelian and non-Mendelian SLE are similar, Janus kinase inhibitors like baricitinib show potential for managing interferon-signature monogenic SLE cases.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033241301506"},"PeriodicalIF":1.9,"publicationDate":"2024-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142668519","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":"Current smoking is related to severe damage in systemic lupus erythematosus patients.","authors":"M A Cosatti, S A Muñoz, M T Tamborenea, M García, A Curti, A Cappuccio, O Rillo, P M Imamura, E Schneeberger, F Dal Pra, M Ballent, M L Cousseau, J Velasco Zamora, V Saurit, S Toloza, M C Danielsen, V I Bellomio, C Graf, S Paira, J Cavallasca, B Pons Estel, J L Cristian Moreno, M Díaz, P Alba, M Verando, G Tate, E Mysler, J Sarano, E E Civit, F Risueño, P Álvarez Sepúlveda, M S Larroude, M F Méndez, A Conforti, D Sohn, C A Helling, S Roverano, S Malm-Green, D Medina Bornachera, A Alvarez, A Eimon, G Pendón, M Mayer, J Marin, C N Pisoni","doi":"10.1177/09612033241301182","DOIUrl":"https://doi.org/10.1177/09612033241301182","url":null,"abstract":"<p><strong>Objective: </strong>To assess the relationship between smoking exposure and organ damage accrual measured by Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index for Systemic Lupus Erythematosus score (SLICC-SDI) in consecutive patients with systemic lupus erythematosus (SLE) from Argentina.</p><p><strong>Methods: </strong>623 consecutive SLE patients (fulfilling ≥4, 1997 ACR criteria) were included in this cross-sectional study. Sociodemographic and disease related variables including SLICC-SDI score and smoking status were collected. Patients currently smoking were considered \"smokers\", and \"non-smokers\" those who never smoked and former smokers. SLICC-SDI was divided into two categories: <3 and ≥3 was defined as severe damage.</p><p><strong>Results: </strong>Six hundred and 23 patients were included in the analysis, 89% women. Eighty-four per cent were non-smokers and 16 % were current smokers 83 percent of patients had SLICC-SDI <3 and 17 % had SLICC-SDI ≥3. Twenty one percent of patients with SLICC-SDI ≥3 and 15% with <3 SLICC-SDI were current smokers (<i>p</i> 0.081). In the multiple regression analysis, current smoking (OR 1.82, CI 95% 1.01-3.31, <i>p</i> 0.046), older age (OR 1.04, CI 95% 1.00-1.05, <i>p</i> 0.034), disease duration (OR 1.03, CI 95% 1.00-1.07, <i>p</i> 0.021) and cyclophosphamide exposure (OR 2.97, CI 95% 1.49-5.88, <i>p</i> 0.002) were related to SLICC-SDI ≥3.</p><p><strong>Conclusion: </strong>In our sample of patients, current smoking, older age, disease duration and cyclophosphamide were related to severe damage (SLICC-SDI ≥3).</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033241301182"},"PeriodicalIF":1.9,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142648349","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":"Understanding patient perspectives on health-related searches on the internet: Insights from an online survey of Korean patients with systemic lupus erythematosus.","authors":"Seung Min Jung, Sung-Hoon Park, Jisoo Lee, Yune-Jung Park, Seung Cheol Shim","doi":"10.1177/09612033241301172","DOIUrl":"10.1177/09612033241301172","url":null,"abstract":"<p><strong>Background: </strong>With the increasing prevalence of digital devices and internet access, digital resources have become essential for educating patients with chronic diseases. We explored the patient perspective on health-related internet searches among Korean patients with systemic lupus erythematosus (SLE).</p><p><strong>Methods: </strong>We collected data through a Google Survey from 344 SLE patients. The survey covered demographics, preferred digital devices and sources, and digital information content, and participants' views on digital resources. We analyzed patient characteristics associated with digital resource usage.</p><p><strong>Results: </strong>Of the 344 patients, 270 reported using the internet to acquire disease-related information, including the association between nutrition and SLE, general information on SLE, and coping strategies for SLE management. SLE-related searches on the internet were more common in patients younger than 40 years (<i>p</i> = 0.002), those with fewer than 15 years of disease duration (<i>p</i> < 0.001), and those with higher education levels (<i>p</i> = 0.022). Disease duration was independently associated with internet use. Patients reported that internet searches for information on SLE improved their understanding of the disease in 181 cases and motivated self-management in 166 cases. In addition, 98 patients found it helpful to make a shared decision with physicians.</p><p><strong>Conclusion: </strong>Health-related searches on the internet are widely used by SLE patients to gather comprehensive information on the disease and to address unmet needs. The positive impact of SLE-related internet searches on disease understanding and self-management emphasizes the importance of developing high-quality digital resources to improve patient education and self-care for the disease.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033241301172"},"PeriodicalIF":1.9,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142668520","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":"Reversibility of pulmonary hypertension in systemic lupus erythematosus after induction immunosuppressive therapy: An inflammatory manifestation?","authors":"Ana Paula Luppino-Assad, Jose Leonidas Alves Junior, Emily Figueiredo Neves Yuki, Luciana Parente Costa Seguro, Sandra Gofinet Pasoto, Caio Julio Cesar Dos Santos Fernandes, Juliana Sobral-Alves, Carlos Viana Poyares Jardim, Eloisa Bonfá, Rogerio Souza, Eduardo F Borba","doi":"10.1177/09612033241301183","DOIUrl":"https://doi.org/10.1177/09612033241301183","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the possible reversibility of PAH to a normopressoric state in SLE after induction immunosuppressive (IS) and predictors of response.</p><p><strong>Methods: </strong>We retrospectively evaluated all SLE-PAH patients who underwent IS therapy at our center. PAH reversion was defined as the normalization of pulmonary arterial pressure (PAP), either by the presence of systolic PAP <40 mmHg on echocardiogram or mean PAP <20 mmHg on right heart catheterization (RHC). SLE patients were divided in Reversion and No-Reversion of SLE-PAH groups for comparative analysis at baseline and after IS.</p><p><strong>Results: </strong>Among 2,074 SLE patients, 28 SLE-PAH received IS therapy (1.3%). Ten patients (35.7%) achieved SLE-PAH reversion. Demographic data, disease duration, SLEDAI-2K, and SDI Damage scores were similar between Reversion and No-Reversion of SLE-PAH groups (<i>p</i> > 0.05). At baseline, Reversion of SLE-PAH had lower sPAP (<i>p</i> = 0.032), lower right ventricle dilatation (<i>p</i> = 0.003) and hypokinesia (<i>p</i> = 0.017) frequencies on echocardiogram, and also lower BNP levels (<i>p</i> = 0.041) and risk stratification score (<i>p</i> = 0.014). Hemodynamic parameters were similar among groups (<i>p</i> > 0.05). After IS, a significant decrease in CRP levels was identified only in Reversion of SLE-PAH (<i>p</i> = 0.013), although both groups had a significant reduction in SLEDAI-2K (<i>p</i> < 0.05). Both groups had significant improvement in risk stratification score (<i>p</i> = 0.009 and <i>p</i> < 0.001) with a better survival rate in Reversion of SLE-PAH (<i>p</i> = 0.047).</p><p><strong>Conclusion: </strong>This is the first study that identified that more than one third of SLE-PAH had a complete reversion of PAH after IS therapy with a significant impact on their survival. These findings strongly support the notion of an underlying inflammatory etiology of this condition, which reinforces the use of immunosuppressive treatment for all SLE patients at PAH onset.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033241301183"},"PeriodicalIF":1.9,"publicationDate":"2024-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142644387","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":"Real world application of the 2023 ACR/EULAR antiphospholipid antibody syndrome classification criteria in a pharmacist directed anticoagulation clinic.","authors":"Catherine DeFazio, Lara Horvath, Jennifer Morgan, Meaghan B Murphy, Ashley N Shtoyko, Elizabeth M Phillips","doi":"10.1177/09612033241301173","DOIUrl":"https://doi.org/10.1177/09612033241301173","url":null,"abstract":"<p><strong>Objectives: </strong>To determine the number of patients that met classification for antiphospholipid antibody syndrome (APS) after applying the 2023 American College of Rheumatology and the European Alliance for Associations of Rheumatology (ACR/EULAR) classification criteria, to identify reasons patients did not meet the new criteria, and determine the number of patients who were single, double, or triple positive based on laboratory criteria.</p><p><strong>Methods: </strong>A single center, retrospective chart review of patients with APS on anticoagulation managed by ambulatory care clinical pharmacists. Data collected included patient demographics, type of anticoagulation, and clinical and laboratory criteria for APS as defined by the 2023 ACR/EULAR criteria. Data is presented using descriptive statistics.</p><p><strong>Results: </strong>A total of 51 patients previously diagnosed with APS were included. There were 42 patients on warfarin (82.3%), 4 patients on direct oral anticoagulants (19%), 3 patients on low molecular weight heparin (5.8%), and 2 patients on fondaparinux (3.9%). Of the 51 patients, 12 (23.5%) met classification criteria, 33 (64.7%) did not meet classification criteria and 6 (11.7%) had insufficient data. Of the 27 patients that did not meet criteria, 13 patients did not meet the laboratory criteria (39.4%), 6 patients did not meet the clinical criteria (18.2%) and 14 patients did not meet both laboratory and clinical criteria (42.4%). Of the 12 patients that met classification criteria, 2 patients were triple positive (16.7%), 3 were double positive (25%), and 7 were single positive (58.3%).</p><p><strong>Conclusions: </strong>Results from this study indicate that APS continues to be a complex disease state with challenges in diagnosis and classification. Since only a small number of patients in our clinic continued to meet the classification criteria, opportunities for patient re-evaluation of management strategies at our institution could be considered.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033241301173"},"PeriodicalIF":1.9,"publicationDate":"2024-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142622935","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":"Fibromyalgia, mood disorders and chronic damage are the main determinants of worse quality of life in systemic lupus erythematosus patients: Results from a cross-sectional analysis.","authors":"Fulvia Ceccarelli, Claudia Ciancarella, Carmelo Pirone, Francesco Natalucci, Licia Picciariello, Cristina Garufi, Silvia Mancuso, Simona Truglia, Francesca Romana Spinelli, Cristiano Alessandri, Fabrizio Cont","doi":"10.1177/09612033241299978","DOIUrl":"https://doi.org/10.1177/09612033241299978","url":null,"abstract":"<p><strong>Objective: </strong>As suggested by the EULAR recommendations, a comprehensive management of Systemic Lupus Erythematosus (SLE) should include the evaluation of disease activity, chronic damage, and quality of life (QoL). QoL is significantly impaired in SLE patients, even in those achieving a state of remission, suggesting the possible contribution of other factors. Thus, in the present study we aimed at analyzing QoL in a large SLE cohort by using LupusQoL, and at identifying the main determinant of poorer QoL.</p><p><strong>Methods: </strong>We conducted a cross-sectional study by including consecutive SLE patients diagnosed according to the 2019 ACR/EULAR criteria. Clinical, laboratory and therapeutical data were collected. Disease activity was assessed by SLEDAI-2k, while chronic damage by the SLICC Damage Index (SDI). The diagnosis of fibromyalgia was made in accordance with the ACR criteria (2016). At the time of the enrollment, all patients completed the following questionnaires: LupusQoL to assess quality of life and hospital anxiety and depression scale (HADS) for anxiety and depression.</p><p><strong>Results: </strong>Our analysis included 237 SLE patients [92.4% female, median age 46 years (IQR 19.5), median disease duration 156.8 months (IQR 180.6)]. At the time of enrollment, we found a mean SLEDAI-2k of 1.7 (DS 2.4); 104 patients (43.9%) had chronic damage, with a mean SDI value of 0.8 (DS 1.3). Patients diagnosed with fibromyalgia were 69 (29.1%); moreover, HADS questionnaire identified a condition of anxiety and depression in 112 (47.3%) and 94 (39.7%) patients, respectively. The most compromised domain in the LupusQoL resulted \"fatigue\", followed by \"burden to others\". Patients with SDI ≥ 1 showed lower quality of life than patients without chronic damage, as demonstrated by significantly lower values in all items of the LupusQoL (<i>p</i> < .01). Furthermore, significantly lower values in all the LupusQoL domains were observed in patients with fibromyalgia, anxiety and depression, in comparison to those patients without these manifestations (<i>p</i> < .0001). No association was demonstrated between QoL and disease activity. Finally, the linear regression analysis confirmed mood disorders, in particular depression, and fibromyalgia as the main determinants of worse quality of life in our cohort.</p><p><strong>Conclusions: </strong>The present study demonstrated the influence of different factors in the quality of life of SLE patients. In particular, the presence of mood disorders, fibromyalgia and chronic damage resulted the main determinants of poorer QoL. This evidence reinforces the need for a comprehensive patient care.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033241299978"},"PeriodicalIF":1.9,"publicationDate":"2024-11-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142622780","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":"Belimumab safety in adult and paediatric Chinese patients with systemic lupus erythematosus: A Phase 4, multicentre, observational study.","authors":"Zhuoli Zhang, Zhizhong Ye, Shanzhi He, Lin Tang, Chuanmei Xie, Songlou Yin, Shuhong Chi, Jing Yang, Qinghong Yu, Min Yang, Xuefeng Zhao, Yifan He, Jingwen Hu, Weibo Wang, Annie Tung","doi":"10.1177/09612033241299175","DOIUrl":"https://doi.org/10.1177/09612033241299175","url":null,"abstract":"<p><strong>Objective: </strong>Although belimumab has been widely used in patients with systemic lupus erythematosus (SLE) globally, real-world safety data among Chinese patients are limited, particularly for children. This study assessed the safety and tolerability of belimumab in adult and paediatric patients with SLE in China in real-world clinical practice.</p><p><strong>Methods: </strong>This Phase 4, multicentre, prospective, observational study enrolled patients prescribed intravenous belimumab by their physicians in tertiary hospitals, independent of a clinical study, during routine clinical visits between May 2021 and May 2022. Patients could have been receiving belimumab prior to enrolment. The primary objective was to describe the incidence of adverse events (AEs), serious AEs (SAEs), adverse drug reactions (ADRs) and AEs of special interest (AESIs) over the 24-week follow-up period. Data were collected at enrolment and approximately 4, 12 and 24 weeks post-enrolment, during routine clinical visits. AEs, ADRs and SAEs were collected as independent events. The safety population comprised patients who received ≥1 dose of belimumab and completed ≥1 follow-up visit.</p><p><strong>Results: </strong>Overall, 417 patients were included in the analysis (safety population); 89.2% were female and mean (standard deviation) age was 35.5 (11.9) years. AEs were reported in 158 patients (37.9%) and were mostly mild (79.1%). The most common AEs were upper respiratory tract infections (<i>n</i> = 19, 4.6%) and hypokalaemia (<i>n</i> = 18, 4.3%; all mild). Among 22 patients (5.3%) with SAEs, four patients (1.0%) had drug-related SAEs (pneumonia, respiratory tract infection, gingivitis and decreased white blood cell and neutrophil count). ADRs were experienced by 25 patients (6.0%), most commonly urinary tract infections (<i>n</i> = 5, 1.2%). There were no fatal SAEs. AESIs occurred in 14 patients (3.4%), including infections of interest (<i>n</i> = 5, 1.2% all herpes zoster), serious selected psychiatric events (<i>n</i> = 3, 0.7%) and infusion-related systemic and anaphylactic reactions (<i>n</i> = 7, 1.7%). Of 14 paediatric patients enrolled, six experienced AEs, zero ADRs, two SAEs and one AESI.</p><p><strong>Conclusion: </strong>Belimumab was generally well tolerated in adult and paediatric patients with SLE in this real-world setting in China, with no new safety signals identified. Future assessment of long-term belimumab safety in China beyond 24 weeks will extend this current body of evidence.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033241299175"},"PeriodicalIF":1.9,"publicationDate":"2024-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142622747","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":"Health seeking behaviour and diagnostic delays in SLE: A multi-ethnic Malaysian cohort study.","authors":"Fatimah Zanirah Nordin, Syahrul Sazliyana Shaharir, Mohd Shahrir Mohamed Said, Rozita Mohd, Rajalingham Sakthiswary, Tengku Amatullah Madeehah Tengku Mohd, Mohd Hafiz Jaafar, Wong Chin Yew","doi":"10.1177/09612033241297548","DOIUrl":"10.1177/09612033241297548","url":null,"abstract":"<p><strong>Introduction: </strong>Heterogeneity of the clinical manifestations of systemic lupus erythematosus (SLE) may lead to diagnostic delays. This study is aimed at determining the health-seeking behaviour patterns and factors associated with diagnostic delays in a multi-ethnic SLE cohort in Malaysia.</p><p><strong>Methodology: </strong>This was a cross-sectional study involving SLE patients who visited our institute between January 2020 and June 2021. A review of the medical records and face-to-face interviews were conducted to obtain sociodemographics, SLE disease characteristics and the intervals from the first symptoms to the diagnosis. Health-seeking behaviours were assessed by asking about the patients' first action during the initial symptoms and were divided into: (i) seeking professional health personnel; (ii) self-treatment; and (iii) the use of the internet as a primary source of information. Diagnostic delays were defined as the interval between initial symptoms and SLE diagnosis of more than 6 months. Low-level disease activity state (LLDAS) at 12 months was assessed from the medical records. Univariate and multivariate logistic regression analysis was subsequently conducted to determine factors associated with diagnostic delays.</p><p><strong>Results: </strong>Among the 154 patients included in the study, 24% (<i>n</i> = 37) had delayed diagnosis. The delay was significantly higher among the Indian versus Malay versus Chinese (42.9% vs 28% vs 10.8%, <i>p</i> = 0.037). Patients with rash tend to have delayed diagnosis (37.8% vs 22.2%, <i>p</i> = 0.08) while fewer patients with frothy urine had delayed diagnosis (8.1% vs 21.4%, <i>p</i> = 0.09). No significant association was found between health-seeking behaviours and diagnostic delays. The rate of LLDAS at 12 months was significantly lower among patients with delayed diagnosis (43.2% vs 70.0%, <i>p</i> = 0.006). Chinese ethnicity remained the only significant factor associated with lesser diagnostic delays in the multivariate analysis, with OR 0.30 (CI 0.09-0.93), <i>p</i> = 0.037.</p><p><strong>Conclusion: </strong>There were ethnic disparities in the early diagnosis of SLE in Malaysia, with Indian patients having a longer interval between the first symptom and diagnosis while the Chinese were associated with lower diagnostic delays. Early diagnosis predicted early attainment of LLDAS, suggesting that prompt recognition of the initial SLE symptoms is important.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033241297548"},"PeriodicalIF":1.9,"publicationDate":"2024-11-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142591023","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":"Rituximab in lupus anticoagulant hypoprothrombinemia syndrome: A case report.","authors":"Jacopo Agnelli Giacchello, Nicol Francesca Trincheri, Patrizia Sciancalepore, Laura Contino, Roberto Mario Santi, Vittorio Pengo","doi":"10.1177/09612033241299619","DOIUrl":"https://doi.org/10.1177/09612033241299619","url":null,"abstract":"<p><strong>Background: </strong>Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare autoimmune condition characterized by acquired prothrombin (FII) deficiency associated with antiphospholipid syndrome (APS) and life-threatening bleeding. We present the case of a 34-year-old woman with heavy menstrual bleeding (HMB), positive Lupus anticoagulant (LA) test, and high titer anticardiolipin antibodies Immunoglobulin G (ACA IgG) and anti-β2 glycoprotein I antibodies IgG (antiB2GPI IgG). Severe iron deficiency anemia necessitated recurrent blood transfusions and intravenous iron infusions from 2018 to 2021.</p><p><strong>Results: </strong>In January 2022, she was admitted to our clinic. Von Willebrand disease screening and platelet function analysis (PFA100) were normal. FII and FIX deficiencies were detected, without factor IX inhibitors. Anti-phosphatidylserine/prothrombin antibodies were confirmed by Padua University lab. To reduce antibody titers and menstrual bleeding, immunosuppressive therapy (Rituximab 375 mg/m2 weekly ×4 weeks) and hormonal therapy (desogestrel 75 mcg/day) were initiated.</p><p><strong>Conclusion: </strong>After 1-year, complete remission of clinical symptoms was achieved, with normalization of FII and FIX values and moderate reduction of aPS/PT titers, especially IgM isotype.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033241299619"},"PeriodicalIF":1.9,"publicationDate":"2024-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142582789","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}