Lupus最新文献

{"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":null,"pages":null},"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":null,"pages":null},"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}

{"title":"Different patterns of longitudinal changes in antinuclear antibodies titers in children with systemic lupus erythematosus and Sjögren's syndrome.","authors":"Patricia Morán Álvarez, Claudia Bracaglia, Rebecca Nicolai, Luigi Giovannelli, Ivan Caiello, Alessandra Boni, Valentina Matteo, Gian Marco Moneta, Virginia Messia, Fabrizio De Benedetti, Emiliano Marasco","doi":"10.1177/09612033241298729","DOIUrl":"https://doi.org/10.1177/09612033241298729","url":null,"abstract":"<p><strong>Objective: </strong>to investigate the trend of autoantibody titers during a 2-year follow-up in pediatric systemic lupus erythematosus (pSLE) and pediatric Sjögren's syndrome (pSS).</p><p><strong>Methods: </strong>Autoantibodies testing was performed every 3-4 months during 2 years from disease onset in a cohort of children with pSLE and pSS.</p><p><strong>Results: </strong>We enrolled 21 children with pSLE and 22 children with pSS. All pSLE patients at 2 years showed ANA titers significantly lower compared to disease onset. Eleven patients (73%) were still ANA positive at 2 years, while 4 (26%) became ANA negative. At diagnosis, 12 (80%) patients showed a homogeneous pattern, while 3 (20%) patients showed a speckled pattern. The latter remained ANA positive with the same pattern; only 2 patients with a homogenous pattern converted to speckled, 4 patients with a homogeneous pattern became ANA negative. ANA negative pSLE patients showed lower levels of interferon score compared to ANA positive patients. Anti-dsDNA titers declined equally in the two groups. All patients with pSS, at disease onset, were ANA and anti-Ro positive and 14 (66%) were anti-La positive. After 2 years of follow-up, 100% remained ANA positive but showed significant lower titers. During follow-up anti-Ro and anti-La titers remained stable.</p><p><strong>Conclusion: </strong>different patterns in changes of ANA and ENA titers in pSLE and pSS were shown. At 2 years of follow-up, all pSLE patients had a lower ANA titer and 26% became negative; however, all pSS patients remained both ANA and ENA positive. This evidence may be due to different pathogenetic pathways in SLE and pSS.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142567510","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":"Severe lupus enteritis: A diagnostic and therapeutic enigma.","authors":"Neha Singh, Jyothi Janardhanan, Sudhir Kale, Jayalakshmi Krishnegowda, Harish Kumar, Syed Mohammed Naushad Ali, Karthik Arigela, Chetan Ginigeri, Sagar Bhattad","doi":"10.1177/09612033241290579","DOIUrl":"10.1177/09612033241290579","url":null,"abstract":"<p><p>Lupus enteritis refers to the gastrointestinal involvement in systemic lupus erythematosus (SLE). It presents with diverse symptoms that frequently overlap with those of other acute abdominal conditions, posing diagnostic challenges. We describe an adolescent female, with lupus pancreatitis and nephritis, who later developed severe lupus enteritis during the course of her illness. She was treated with pulse methylprednisolone and intravenous cyclophosphamide and gradually improved over 3 weeks. Our case highlights the need to consider lupus enteritis in patients with severe pain abdomen and intractable vomiting. Presence of lupus pancreatitis and nephritis are risk factors for development of enteritis.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142372234","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":"Psychometric properties evaluation of the Persian version of the self-report outcome scale for lupus disease Lupus_PRO version 1.7.","authors":"Bahareh Mahmoudzadeh, Youkhabeh Mohammadian, Shirin Assar, Seyyed Mojtaba Ahmadi","doi":"10.1177/09612033241288011","DOIUrl":"10.1177/09612033241288011","url":null,"abstract":"<p><strong>Purpose: </strong>Systemic Lupus Erythematosus (SLE) significantly affects both the quality of life related to health and non-health aspects. This study aims to evaluate the psychometric properties of the Persian version of the Lupus_PRO questionnaire, version 1.7. The questionnaire serves as a specific self-report outcome scale for assessing the treatment outcomes of Lupus disease.</p><p><strong>Methods: </strong>The Lupus_PRO questionnaire, version 1.7, was translated into Persian using the standard forward-backward method. After being completed by 218 patients with Lupus, the psychometric properties of the instrument were examined. The Convergent and Discriminant Validity of the scale were assessed using Average Variance Extracted and Cross Loadings, respectively. The construct validity of the questionnaire was also evaluated through Confirmatory Factor Analysis (CFA). Cronbach's alpha was calculated to assess the reliability of the questionnaire dimensions.</p><p><strong>Findings: </strong>Out of the 218 patients with lupus who participated in the current study, 13 (6%) were male, and 205 (94%) were female. The mean (SD) age of participants was 40.29 (10.94) years. The Average Variance Extracted (AVE) for HRQOL and Non-HRQOL constructs, except for the Coping and Cognition dimension, was greater than 0.50, indicating satisfactory convergent validity. The interdimensional correlation coefficient (Discriminant Validity) for each dimension with other questionnaire dimensions was less than 0.10, indicating that the questionnaire has good convergent and discriminant validity. Additionally, the results of the Confirmatory Factor Analysis (CFA) indicated that the questionnaire was a suitable fit. In terms of reliability, Cronbach's alpha for the various questionnaire dimensions ranged from 0.51 to 0.91, indicating good internal consistency.</p><p><strong>Conclusion: </strong>The Persian version of the Lupus_PRO questionnaire, version 1.7, demonstrates acceptable validity and reliability in the Iranian population. This instrument can effectively measure various aspects of the quality of life in patients with lupus.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142349416","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":"Needs for discharge planning and facilitators-barriers to implementing discharge plans for patients with SLE: A qualitative study.","authors":"Shu-Ting Liu, Xue-Ying Xia, Zi-Cheng Song, Jia-Jia Hu, Wei Zhao","doi":"10.1177/09612033241286991","DOIUrl":"10.1177/09612033241286991","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the needs of patients with SLE in terms of discharge planning, to outline the practical perspectives of clinical healthcare professionals regarding the enablers and impediments to SLE patients' discharge planning, and to establish a basis for the creation of SLE discharge plans for subsequently diagnosed patients.</p><p><strong>Methods: </strong>Descriptive qualitative research methodology was used in this study, with the researcher herself as the research instrument. Healthcare professionals formally employed in the rheumatology and immunology department, as well as SLE patients admitted to a tertiary-level hospital in Anhui Province between August and December 2023, were chosen for the study using a purposeful sample technique. In-person, semi-structured in-depth interviews were carried out, and used thematic analysis to analyze the interview data and distil themes.</p><p><strong>Result: </strong>A total of 17 patients and 13 healthcare professionals were interviewed. Five themes and sixteen sub-themes in all were extracted: ①the needs of SLE patients for discharge planning; ②the present state of discharge planning implementation; ③factors conducive to the implementation of discharge planning; ④factors hindering the implementation of discharge planning; ⑤recommendations for implementing of discharge planning.</p><p><strong>Conclusion: </strong>Planning for the discharge of SLE patients is essential, and in order to support patients' successful discharge, management should strengthen training, thoroughly evaluate the needs of SLE patients, and create customized discharge plans.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142290392","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":"Treatment of discoid lupus with lenalidomide and anifrolumab: Case report and review of the literature.","authors":"Connor S Centner, Nicole M Robertson, Jun Kang, Inbal B Sander, Homa Timlin","doi":"10.1177/09612033241289489","DOIUrl":"10.1177/09612033241289489","url":null,"abstract":"<p><p>Discoid lupus erythematosus (DLE) is a devastating autoimmune disease with few therapies available. For patients with little to no symptom improvement with initial treatment, the literature surrounding further treatment options and their efficacy remains limited. Here we report a 46-year-old patient with lupus and refractory DLE, who failed numerous medications since her initial diagnosis in 2014. She had a robust response to lenalidomide with further improvement after adding anifrolumab (ANI), in conjunction with the standard of care hydroxychloroquine. Furthermore, she was able to taper off steroids without interval flares. The patient has not experienced any major infections since the initiation of treatment. No previous case reports describing outcomes of lenalidomide and ANI have been reported, yet the combinational approach has potential. Future clinical trials are needed to investigate the safety of the combination of lenalidomide and ANI in lupus patients with refractory DLE.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142381224","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":"Correlation between ABO blood group and clinical characteristics of patients with lupus nephritis.","authors":"Qianqian Chen, Jiaqi Liu, Keyi Zhou, Xiaojun Kong, Wei Wang","doi":"10.1177/09612033241286604","DOIUrl":"10.1177/09612033241286604","url":null,"abstract":"","PeriodicalId":18044,"journal":{"name":"Lupus","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142290391","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":"Does the standard proteinuria cut-off for renal biopsy in lupus nephritis as per the current guidelines hold good for Asian population? A single-centre study from South India.","authors":"Shivraj Padiyar, Chandu As, Mahasampath Gowri, John Mathew","doi":"10.1177/09612033241289806","DOIUrl":"10.1177/09612033241289806","url":null,"abstract":"<p><strong>Introduction: </strong>Current rheumatology and nephrology society guidelines in lupus nephritis do not recommend renal biopsy for proteinuria of less than 500 mg/24 h. This might lead to a significant delay in the early diagnosis of lupus nephritis.</p><p><strong>Aim: </strong>The main aim of this study is to determine the nature of renal lesions in patients with low-grade proteinuria and to analyze the predictors for clinically significant lupus nephritis.</p><p><strong>Methods: </strong>This was a single-center, retrospective study. All consecutive patients of lupus nephritis, with low-grade proteinuria (200 mg to 500 mg/24 h) undergoing renal biopsy were enrolled in this study. The renal biopsies were classified into significant lesions (Class III/IV/V) and non-significant lesions (Class I and II). Treatment naïve groups and treatment-modified groups were analyzed separately. Predictive factors for significant renal lesions were determined by univariate and multivariate analysis.</p><p><strong>Results: </strong>We identified 183 patients of lupus with proteinuria between 200 and 500 mg / 24 h. Mean (SD) age was 30.2 (11.39) years with 167 (91.2%) of them being females. The mean (SD) baseline proteinuria was 351.03 (98.1) mg/24 h 85 patients (46.5%) had proliferative lupus nephritis where whereas 17 patients (9.3%) had membranous nephropathy. Crescents and fibrinoid necrosis were seen in 10 (5.46%) and 24 (13.11 %) patients respectively. Isolated proteinuria without any other sediments was seen in 95 patients (51.9%) of which 29 patients had proliferative lupus nephritis. Elevated Anti-double stranded DNA (anti-dsDNA), low C3, low C4 and the presence of urinary sediments were significantly associated with significant renal lesions in biopsy.</p><p><strong>Conclusion: </strong>Significant renal lesions were seen in around half of the patients with low-grade proteinuria underscoring the importance of performing a renal biopsy in this set of patients. Low C3 and C4, urinary sediments, and elevated anti-dsDNA were predictors for significant renal lesions.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142365752","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":"Identification of common MicroRNAs expression signatures in antiphospholipid syndrome and thromboembolic disease: A scoping review.","authors":"Camila de Oliveira Vaz, Bruna Cardoso Jacintho, Gabrielle de Mello Santos, José Diogo de Oliveira, Bruna Moraes Mazetto, Murilo Vieira Geraldo, Fernanda A Orsi","doi":"10.1177/09612033241286601","DOIUrl":"10.1177/09612033241286601","url":null,"abstract":"<p><strong>Background: </strong>Antiphospholipid syndrome (APS) is an acquired autoimmune disorder characterized by distinct pathophysiological mechanisms leading to heterogeneous manifestations, including venous and arterial thrombosis. Despite the lack of specific markers of thrombosis risk in APS, some of the mechanisms responsible for thrombosis in APS may overlap with those of other thromboembolic diseases. Understanding these similarities is important for improving the assessment of thrombosis risk in APS. MicroRNAs (MiRNAs) are RNA molecules that regulate gene expression and may influence the autoimmune response and coagulation.</p><p><strong>Purpose: </strong>In this scoping review we aimed to investigate shared miRNAs profiles associated with APS and other thromboembolic diseases as a means of identifying markers indicative of a pro-thrombotic profile among patients with APS.</p><p><strong>Data collection and results: </strong>Through a comprehensive search of scientific databases, 45 relevant studies were identified out of 1020 references. miRs-124-3p, 125b-5p, 125a-5p, and 17-5p, were associated with APS and arterial thrombosis, while miRs-106a-5p, 146b-5p, 15a-5p, 222-3p, and 451a were associated with APS and venous thrombosis. Additionally, miR-126a-3p was associated with APS and both arterial and venous thrombosis.</p><p><strong>Conclusion: </strong>We observed that APS shares a common miRNAs signature with non-APS related thrombosis, suggesting that miRNA expression profiles may serve as markers of thrombotic risk in APS. Further validation of a pro-thrombotic miRNA signature in APS is warranted to improve risk assessment, diagnosis, and management of APS.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142349415","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}