Lancet Rheumatology最新文献

{"title":"Immunosuppressive agents or intravenous immunoglobulin in addition to glucocorticoids in the treatment of Susac syndrome: a French national cohort study","authors":"Alexandra Kachaner MD , Arthur Mageau MD PhD , Tiphaine Goulenok MD , Chrystelle François BSN , Nicole Delory BSN , Marie-Paule Chauveheid MD , Prof Cedric Laouenan MD PhD , Serge Doan MD , Caroline Halimi MD , Isabelle Klein MD PhD , Prof Thomas Papo MD , Prof Karim Sacré MD PhD , French Susac Study Group","doi":"10.1016/S2665-9913(24)00220-0","DOIUrl":"10.1016/S2665-9913(24)00220-0","url":null,"abstract":"<div><h3>Background</h3><div>Susac syndrome is a rare disease affecting mainly young women and is characterised by an occlusive microvessel disease limited to the brain, retina, and inner ear. No randomised controlled trial has been published or declared as ongoing to investigate treatments for Susac syndrome. We aimed to compare the effect of glucocorticoids given alone or in combination with immunosuppressive agents or intravenous immunoglobulin for the prevention of relapse in patients with Susac syndrome.</div></div><div><h3>Methods</h3><div>The Phenotypic and Etiological Characterization of Susac Syndrome—National Clinical Research Hospital Program study is a prospective national cohort study that started enrolling on Nov 29, 2011, and included all consecutive patients aged 18 years or older with Susac syndrome who were referred to the French reference centre (Department of Internal Medicine, Bichat–Claude Bernard Hospital, Paris). Susac syndrome was defined by either the triad of encephalopathy with typical brain MRI abnormalities, cochleo-vestibular damage, and multiple occlusions of retinal central artery branches, or at least two of the three criteria without any alternative diagnosis. Collected data included fundoscopy, retinal angiography, audiometry, cerebrospinal fluid, brain MRI, and treatment received at diagnosis; months 1, 3, 6, and 12 after diagnosis; and then annually for 5 years or in the case of a relapse. The primary outcome was defined as the first relapse occurring within a 36-month follow-up period from the first day of treatment, characterised by new clinical symptoms or signs, and new abnormalities observed on retinal angiography, audiometry, or brain MRI, necessitating treatment intensification. There was no involvement of people with lived experience at any stage. The study is registered at <span><span>ClinicalTrials.gov</span><svg><path></path></svg></span>, <span><span>NCT01481662</span><svg><path></path></svg></span>.</div></div><div><h3>Findings</h3><div>Between Nov 29, 2011, and Dec 2, 2022, 64 patients were included in the study, with a mean age at diagnosis of 35 years (SD 11); 41 (64%) were women and 23 (36%) were men. At diagnosis, 60 patients received glucocorticoids; 40 (63%) of 64 patients received glucocorticoids alone as a first-line therapy while 20 (31%) received glucocorticoids in combination with immunosuppressive agents or intravenous immunoglobulin. Overall, 46 (72%) of 64 patients had a first relapse with a median relapse-free survival time of 3·96 months (95% CI 2·24–16·07). Comparison of relapse-free survival showed no significant difference between the two treatment strategies (hazard ratio [HR] 1·11 [95% CI 0·56–2·17], p=0·76), compared with glucocorticoids alone as the reference group. In patients who first relapsed while treated with glucocorticoids alone, there was no significant difference in second relapse-free survival between those who did or did not receive immunosuppressive agents or ","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e15-e20"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142298934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cenerimod, a sphingosine-1-phosphate receptor modulator, versus placebo in patients with moderate-to-severe systemic lupus erythematosus (CARE): an international, double-blind, randomised, placebo-controlled, phase 2 trial","authors":"Prof Anca D Askanase , Prof David D'Cruz MD , Prof Kenneth Kalunian MD , Prof Joan T Merrill MD , Prof Sandra V Navarra MD , Clélia Cahuzac MD , Peter Cornelisse MSc , Mark J Murphy PhD , Daniel S Strasser PhD , Luba Trokan MD , Ouali Berkani MSN","doi":"10.1016/S2665-9913(24)00246-7","DOIUrl":"10.1016/S2665-9913(24)00246-7","url":null,"abstract":"<div><h3>Background</h3><div>Sphingosine-1-phosphate (S1P) is a signalling molecule that has an inhibitory role in atherosclerosis, inflammation, cell proliferation, and immunity. Cenerimod is a selective S1P<sub>1</sub> receptor modulator under investigation for the treatment of systemic lupus erythematosus (SLE). We aimed to determine the efficacy, safety, and tolerability of four doses of cenerimod in adults with moderate-to-severe SLE receiving standard of care background therapy.</div></div><div><h3>Methods</h3><div>CARE was a double-blind, randomised, placebo-controlled, phase 2 trial, in adults (aged 18–75 years) with moderate-to-severe SLE (a score of at least 6 out of 105 on the SLE disease activity index-2000, modified to exclude leukopenia [mSLEDAI-2K] score). Participants were recruited from 189 hospitals, specialist centres, and outpatient clinics in 22 countries in Asia Pacific, Latin America, Europe, and the USA. Participants were randomly assigned (1:1:1:1:1), using an interactive response technology via balanced block randomisation (block size of 5) and stratified by oral glucocorticoid dose at randomisation and disease activity at screening, to once-daily oral cenerimod at 0·5 mg, 1·0 mg, 2·0 mg, or 4·0 mg or placebo, in addition to stable background SLE therapy, and followed up for 12 months. After 6 months, participants assigned to cenerimod 4·0 mg were randomly assigned again (1:1) to either cenerimod 2.0 mg or placebo for a further 6 months. The primary endpoint was change from baseline to month 6 in mSLEDAI-2K score, assessed in all participants randomly assigned to treatment (full analysis set). To meet the primary endpoint, the doses had to show a significant improvement over placebo, when adjusting for multiplicity, considering the hierarchical testing strategy, per a prespecified plan. Safety analyses included all participants who received at least one dose of study treatment. This study is registered with <span><span>ClinicalTrials.gov</span><svg><path></path></svg></span>, <span><span>NCT03742037</span><svg><path></path></svg></span>, and is complete.</div></div><div><h3>Findings</h3><div>Between Dec 21, 2018, and Aug 25, 2022, 810 patients were screened and 427 were randomly assigned to 0·5 mg (n=85), 1·0 mg (n=85), 2·0 mg (n=86), and 4·0 mg (n=85) cenerimod or placebo (n=86). Median age was 42 years (IQR 33–51), 406 (95%) of 427 participants were women, 21 (5%) were men, and 337 (79%) were White. At month 6, the least squares mean change from baseline in mSLEDAI-2K score was –2·85 (95% CI –3·60 to –2·10) for the placebo group and –3·24 (–3·98 to –2·49; difference <em>vs</em> placebo –0·39 [95% CI –1·45 to 0·68]; p=0·47) for the cenerimod 0·5 mg group, –3·41 (–4·16 to –2·67; difference <em>vs</em> placebo –0·57 [–1·62 to 0·49]; p=0·29) for the 1·0 mg group, –2·84 (–3·58 to –2·09; difference <em>vs</em> placebo 0·01 [–1·05 to 1·08]; p=0·98) for the 2·0 mg group, and –4·04 (–4·79 to –3·28; difference <em>vs</em> placeb","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e21-e32"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142717476","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Inequality and health: all is not equal","authors":"The Lancet Rheumatology","doi":"10.1016/S2665-9913(24)00380-1","DOIUrl":"10.1016/S2665-9913(24)00380-1","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Page e1"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142899366","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Structural racism is a driver of fragmented care and acute care use among people with rheumatic conditions","authors":"João Luiz Bastos","doi":"10.1016/S2665-9913(24)00341-2","DOIUrl":"10.1016/S2665-9913(24)00341-2","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e5-e6"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142693755","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Targeting sphingosine-1-phosphate receptor modulator in SLE: a promising new therapeutic option","authors":"Giuseppe Barilaro , Ricard Cervera","doi":"10.1016/S2665-9913(24)00333-3","DOIUrl":"10.1016/S2665-9913(24)00333-3","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e3-e5"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142717478","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Predictors of pregnancy outcomes in SLE","authors":"Wenqin He , Xiaoyu Tang , Rongjing Shi , Dan Ma , Li-Yun Zhang","doi":"10.1016/S2665-9913(24)00345-X","DOIUrl":"10.1016/S2665-9913(24)00345-X","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e11-e12"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142792625","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Digital health technologies to strengthen patient-centred outcome assessment in clinical trials in inflammatory arthritis","authors":"Dylan McGagh BMBCh ,&nbsp;Kaiyang Song BA ,&nbsp;Hang Yuan DPhil ,&nbsp;Andrew P Creagh DPhil ,&nbsp;Sally Fenton PhD ,&nbsp;Prof Wan-Fai Ng PhD ,&nbsp;Jennifer C Goldsack MBA ,&nbsp;Prof William G Dixon PhD ,&nbsp;Prof Aiden Doherty PhD ,&nbsp;Prof Laura C Coates PhD","doi":"10.1016/S2665-9913(24)00186-3","DOIUrl":"10.1016/S2665-9913(24)00186-3","url":null,"abstract":"<div><div>Common to all inflammatory arthritides, namely rheumatoid arthritis, psoriatic arthritis, axial spondyloarthritis, and juvenile idiopathic arthritis, is a potential for reduced mobility that manifests through joint pain, swelling, stiffness, and ultimately joint damage. Across these conditions, consensus has been reached on the need to capture outcomes related to mobility, such as functional capacity and physical activity, as core domains in randomised controlled trials. Existing endpoints within these core domains rely wholly on self-reported questionnaires that capture patients' perceptions of their symptoms and activities. These questionnaires are subjective, inherently vulnerable to recall bias, and do not capture the granularity of fluctuations over time. Several early adopters have integrated sensor-based digital health technology (DHT)-derived endpoints to measure physical function and activity in randomised controlled trials for conditions including Parkinson's disease, Duchenne's muscular dystrophy, chronic obstructive pulmonary disease, and heart failure. Despite these applications, there have been no sensor-based DHT-derived endpoints in clinical trials recruiting patients with inflammatory arthritis. Borrowing from case studies across medicine, we outline the opportunities and challenges in developing novel sensor-based DHT-derived endpoints that capture the symptoms and disease manifestations most relevant to patients with inflammatory arthritis.</div></div>","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e55-e63"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141876414","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Factors associated with biological and targeted synthetic disease-modifying antirheumatic drug initiation for rheumatoid arthritis in underserved patient groups in England and Wales, UK: a national cohort study","authors":"Mark D Russell PhD ,&nbsp;Mark Gibson MBBS ,&nbsp;Benjamin Zuckerman MBBS ,&nbsp;Kanta Kumar PhD ,&nbsp;Shirish Dubey MBBS ,&nbsp;Maryam A Adas MBBS ,&nbsp;Edward Alveyn BMBCh ,&nbsp;Samir Patel MBBS ,&nbsp;Zijing Yang MSc ,&nbsp;Katie Bechman PhD ,&nbsp;Elizabeth Price MD ,&nbsp;Sarah Gallagher ,&nbsp;Prof Andrew P Cope PhD ,&nbsp;Sam Norton PhD ,&nbsp;Prof James B Galloway PhD","doi":"10.1016/S2665-9913(24)00221-2","DOIUrl":"10.1016/S2665-9913(24)00221-2","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Background&lt;/h3&gt;&lt;div&gt;Quantifying health-care inequality is essential to addressing the imbalance in outcomes attributable to age, sex, race or ethnicity, and multimorbidity. In this study, we analysed differences in the initiation of biological or targeted synthetic disease-modifying antirheumatic drugs (DMARDs) in patients with rheumatoid arthritis within the universal health-care system of England and Wales, UK.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;An observational cohort study was conducted using the National Early Inflammatory Arthritis Audit (NEIAA) dataset. We included all patients with rheumatoid arthritis who were enrolled in NEIAA between May 8, 2018, and April 30, 2022, and who had 12-month follow-up data available. Modified Poisson regression was used to explore factors associated with the initiation of biological and targeted synthetic DMARDs within 12 months of initial rheumatology assessment. The factors evaluated included age, sex, ethnicity, socioeconomic status (index of multiple deprivation), smoking status, and relevant comorbidities (lung disease, cardiovascular disease, cancer, and depression). NEIAA is supported by people with lived experience of rheumatoid arthritis, who contributed to study design and the interpretation of findings.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Findings&lt;/h3&gt;&lt;div&gt;6098 patients in NEIAA had new diagnoses of rheumatoid arthritis and available follow-up data. The mean age was 59·2 years (SD 14·9); 3912 (64·2%) patients were women and 2186 (35·8%) were men. 6047 (99·2%) patients had available ethnicity data, of whom 5215 (86·2%) were White, 152 (2·5%) were Black, 478 (7·9%) were Asian, and 202 (3·3%) were of mixed or other ethnicities. 508 (8·3%) of 6098 patients initiated biological and targeted synthetic DMARDs within 12 months. Patients younger than 40 years were more likely to be initiated on biological and targeted synthetic DMARDs than individuals older than 65 years (multivariable-adjusted risk ratio 2·41 [95% CI 1·83–3·19]; p&lt;0·0001). Asian individuals were less likely to be initiated on biological and targeted synthetic DMARDs than White individuals (0·52 [0·36–0·76]; p=0·0007), which persisted after adjustment for socioeconomic status, comorbidities, baseline disease severity, and the initial response to conventional synthetic DMARDs. These differences were evident for Asian women but not Asian men. Black individuals were more likely to be initiated on biological and targeted synthetic DMARDs than White individuals (1·54 [1·10–2·16]; p=0·012), which became non-significant after adjusting for baseline disease severity and autoantibody status.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Interpretation&lt;/h3&gt;&lt;div&gt;The initiation of biological and targeted synthetic DMARDs for patients with newly diagnosed rheumatoid arthritis varies markedly by ethnicity and age in the universal health-care system of England and Wales. This study demonstrates the importance of providing tailored information and ensuring equitable access to high-","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e44-e54"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142478081","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Predictors of pregnancy outcomes in SLE","authors":"Maria I Zervou ,&nbsp;George N Goulielmos","doi":"10.1016/S2665-9913(24)00346-1","DOIUrl":"10.1016/S2665-9913(24)00346-1","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e12-e13"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142792627","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dermatology response to the Hyperinflammation and HLH Across Specialty Collaboration consensus guideline – Authors' reply","authors":"Aisling S Carr ,&nbsp;Ben Carpenter ,&nbsp;Ian Proctor ,&nbsp;Bethany Brockbank ,&nbsp;Evangelia Panagou ,&nbsp;Rachel Tattersall ,&nbsp;Satyen Gohil ,&nbsp;Jessica J Manson","doi":"10.1016/S2665-9913(24)00375-8","DOIUrl":"10.1016/S2665-9913(24)00375-8","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e10-e11"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142899356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}