Lancet Rheumatology最新文献

{"title":"Clinical phenotypes, classification, and long-term outcomes of childhood-onset Sjögren's disease into adulthood: a single-centre cohort study","authors":"Prof Coziana Ciurtin PhD , Junjie Peng PhD , Ruby Taylor-Gotch BSc , Hannah Peckham PhD , Robert Wilson MRes , Muthana Al Obaidi MBBS , Prof Elizabeth C Jury PhD , PReS Childhood Sjögren's Interest Group","doi":"10.1016/S2665-9913(25)00283-8","DOIUrl":"10.1016/S2665-9913(25)00283-8","url":null,"abstract":"<div><h3>Background</h3><div>Childhood-onset Sjögren's disease is a rare and under-investigated rheumatic condition. The natural course of childhood-onset Sjögren's disease in adulthood in not known. This study aimed to evaluate long-term disease trajectories and complications of childhood-onset Sjögren's disease and explore management strategies.</div></div><div><h3>Methods</h3><div>This combined retrospective and prospective analysis of a childhood-onset Sjögren's disease cohort with long-term follow-up into adulthood was done in individuals aged 13–36 years with childhood-onset Sjögren's disease recruited from a single tertiary adolescent and young adult rheumatology service at University College London Hospital, UK. Participants were either approached consecutively during routine clinical appointments, or their data were collected retrospectively from the time of diagnosis to the time of transition to the service, and prospectively thereafter. We mapped the cohort onto clinical phenotypes defined by the Florida Scoring System at disease onset and stratified them based on the Newcastle Sjögren's Stratification Tool at last assessment. Disease activity, symptom severity, and damage trajectories were assessed using European Alliance of Associations for Rheumatology (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI), EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI), and Sjögren's Syndrome Disease Damage Index (SSDDI), respectively. People with related lived experience were involved in the study design and implementation.</div></div><div><h3>Findings</h3><div>Between March 1, 2020, and June 30, 2024, we identified 30 children and young people diagnosed with childhood-onset Sjögren's disease based on expert opinion. Mean age at onset was 12·7 years (SD 3·3). 28 (93%) of 30 individuals were female and two (7%) were male. The most common disease manifestations at onset were fatigue (22 [73%] of 30 individuals), arthralgia (21 [70%]), dryness (17 [57%]), glandular swelling (15 [50%]), and skin rashes (ten [30%]). Diagnostic delay of more than 3 years from symptoms onset increased the prevalence of reported dryness (nine [100%] of nine <em>vs</em> eight [38%] of 21; p=0·0014). Children and young people with childhood-onset Sjögren's disease had two distinct disease activity and symptom trajectories (high ESSDAI: mean 3·9 [SD 2·2] <em>vs</em> low ESSDAI: mean 0·8 [1·1]; p<0·0001 and high ESSPRI: mean 5·6 [2·7] <em>vs</em> low ESSPRI: mean 3·1 [1·0]; p=0·036), which could not be predicted by sex or age at onset, symptom duration, or duration of follow-up. Damage accrual did not differ based on activity and symptom trajectory (p=0·080 and p=1·0, respectively). At last review, the median ESSDAI score was 2·0 (IQR 2·0–8·0) and the ESSPRI score was 5·3 (3·0–7·0). Four (13%) of 30 patients developed lymphoma and 17 (57%) accumulated damage (SSDDI score ≥1).</div></div><div><h3>Interpretation</h3><div>This preliminary evaluation of long-term","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 3","pages":"Pages e204-e216"},"PeriodicalIF":16.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145582679","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":"Long-term outcomes of abatacept in individuals at risk of developing rheumatoid arthritis (ALTO): a randomised, double-blind, placebo-controlled trial","authors":"Prof Andrew P Cope MD , Marianna Jasenecova MSc , Joana C Vasconcelos MSc , Sumera Qureshi MD , Dr Karin A van Schie PhD , Prof Andrew Filer MD , Prof Karim Raza MD , Prof Maria Antonietta D’Agostino MD , Prof Iain B McInnes MD , Prof Stefan Siebert MD , Prof John D Isaacs MD , Arthur G Pratt MD , Prof Benjamin A Fisher MD , Prof Christopher D Buckley MD , Prof Paul Emery MD , Prof Kulveer Mankia , Pauline Ho MD , Prof Maya H Buch MD , Prof Coziana Ciurtin MD , Prof Dirkjan van Schaardenburg MD , Vanessa Quick","doi":"10.1016/S2665-9913(25)00371-6","DOIUrl":"10.1016/S2665-9913(25)00371-6","url":null,"abstract":"<div><h3>Background</h3><div>Clinical trials aimed at preventing rheumatoid arthritis in individuals at risk have had variable results. The long-term outcomes of disease interception, however, are not known. We aimed to examine the long-term effect of therapeutic intervention, with emphasis on efficacy and safety.</div></div><div><h3>Methods</h3><div>The Arthritis Prevention In the Preclinical Phase of Rheumatoid arthritis with Abatacept (APIPPRA) phase 2b, randomised controlled trial recruited 213 anti-citrullinated protein antibody (ACPA) positive individuals with arthralgia in 28 hospital-based early arthritis clinics in the UK and three in the Netherlands, randomly assigning participants to 52 weekly subcutaneous injections of 125 mg abatacept (n=110) or placebo (n=103), with another 52 weeks of follow-up. The APIPPRA Long-Term Outcome (ALTO) study extended follow-up for between 4 and 8 years and study participants and clinical assessors remained masked to treatment group. The primary outcome was the time from randomisation to development of clinical synovitis in at least three joints, rheumatoid arthritis according to American College of Rheumatology–European Alliance of Associations for Rheumatology 2010 criteria, or first treatment with disease modifying anti-rheumatic drugs, whichever was met first. The primary outcome was also stratified by autoantibody profiles defined at the time of randomisation. People with lived experience of rheumatoid arthritis had input into the APIPPRA study design. The study was registered at ISRCTN (ISRCTN-12680338), and is completed.</div></div><div><h3>Findings</h3><div>Between April 26, 2021, and Jan 31, 2023, 143 APIPPRA study participants enrolled in ALTO: 71 in the abatacept group and 72 in the placebo group (mean age 48·2 years [SD 11·2], 112 [78%] females, 31 [22%] males, 116 [81%] White). Median follow-up time from randomisation was 55 months (IQR 23–74). Primary events increased by 54 to 119. The initial between-group difference in restricted mean arthritis-free survival time observed at 2 years in APIPPRA remained significant at 4 years (4·9 months 95% CI 0·1–9·6; p=0·044), although the magnitude of this difference diminished over time. Assessments of disease activity and patient reported outcomes revealed no significant differences between groups beyond the treatment period. However, although participants with a broad autoantibody profile at baseline were at highest risk of progressing, this subgroup responded better to abatacept. There were 18 serious adverse events in the abatacept group and 13 in the placebo group; none deemed related to study drug.</div></div><div><h3>Interpretation</h3><div>In this at-risk population, 1-year treatment with abatacept delayed progression to rheumatoid arthritis for up to 4 years. Those at highest risk of progression have a broad autoantibody profile but are more responsive to abatacept treatment.</div></div><div><h3>Funding</h3><div>Bristol Myers Squibb.</div></","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 3","pages":"Pages e171-e180"},"PeriodicalIF":16.4,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146041852","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":"Herpetiform laryngeal ulceration in Behçet's disease.","authors":"Hanlin Yin, Xuesong Liu, Yun Qiao, Yunying Lu, Qian Gui, Li Liu, Liangjing Lu","doi":"10.1016/S2665-9913(26)00005-6","DOIUrl":"https://doi.org/10.1016/S2665-9913(26)00005-6","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":16.4,"publicationDate":"2026-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146133359","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":"Trends in mortality due to haemophagocytic lymphohistiocytosis across 29 European countries from 2011 to 2021: a retrospective, international, population-based study","authors":"Leher Gumber MSc , Reza Omidvar MSc , Francesca Gonnelli PhD , Adam Taylor MEng , Thomas Trimble BSc , Mark Bishton PhD , Prof Matthew Collin PhD , Prof Richard Hubbard DM , Peter C Lanyon DM , Rachel S Tattersall PhD , Jessica J Manson PhD , Fiona A Pearce PhD","doi":"10.1016/S2665-9913(25)00292-9","DOIUrl":"10.1016/S2665-9913(25)00292-9","url":null,"abstract":"<div><h3>Background</h3><div>Previous research has suggested that the incidence of haemophagocytic lymphohistiocytosis is increasing in Europe. We aimed to examine rates of mortality due to haemophagocytic lymphohistiocytosis across 29 European countries from 2011 to 2021.</div></div><div><h3>Methods</h3><div>In this retrospective, population-based, descriptive study, we applied to EUROSTAT for publicly available death certificate data for mortality due to haemophagocytic lymphohistiocytosis in countries in Europe. We defined haemophagocytic lymphohistiocytosis mortality as any death recorded with ICD-10 codes of D76.1 or D76.2 as the underlying cause. We calculated age-specific and sex-specific death registration rates from Jan 1, 2011, to Dec 31, 2021, for each country and used Poisson regression to compare Europe-wide rates over time. We used direct standardisation to compare rates between countries. We also searched Scopus Analytics to establish the number of haemophagocytic lymphohistiocytosis publications for each country from Jan 1, 2000, to June 11, 2024, and analysed the correlation between mortality rates and research activity measured by the number of relevant publications.</div></div><div><h3>Findings</h3><div>Of 34 European countries that provided data, five were excluded from the analysis because the data had been censored due to small numbers of deaths. Analysis of 3345 deaths from the remaining 29 countries showed that crude haemophagocytic lymphohistiocytosis mortality increased from 3·9 per 10 000 000 person-years in 2011, to 6·6 per 10 000 000 person-years population in 2021. The age–sex-standardised mortality rate across Europe was 4·7 (95% CI 3·0–6·4) per 10 000 000 person-years, with the highest recorded rate in France (10·1, 0·0–27·3) and the lowest in Romania (0·5, 0·0–13·6). Crude mortality rates were highest in infants aged 0–4 years (17·5, 95% CI 16·1–19·0) and adults aged 80–85 years (15·6, 13·7–17·6). Mortality was higher in male than in female individuals (adjusted rate ratio 1·5, 95% CI 1·4–1·6). Increased haemophagocytic lymphohistiocytosis-related research activity often occurred in countries with higher rates of mortality recorded due to haemophagocytic lymphohistiocytosis than countries with lower rates (Pearson's correlation coefficient 0·4968; p=0·012).</div></div><div><h3>Interpretation</h3><div>Recorded rates of mortality due to haemophagocytic lymphohistiocytosis have nearly doubled over the past decade in Europe. Deaths were most common at the extremes of age and were more common in male than in female individuals. Age-standardised rates between countries differed substantially, suggesting potential under-recognition of the diagnosis of haemophagocytic lymphohistiocytosis. There is a need to increase awareness among clinicians together with implementation of evidence-based guidelines for diagnosis and urgent treatment.</div></div><div><h3>Funding</h3><div>Medical Research Council Rare Disease Platform Node","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 2","pages":"Pages e108-e115"},"PeriodicalIF":16.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145655948","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":"Assessing clinical and cost effectiveness of total versus partial knee replacement (TOPKAT): 10-year follow-up of a multicentre, randomised controlled trial","authors":"Prof David J Beard DPhil , Loretta J Davies DPhil , Prof Jonathan A Cook PhD , Prof Graeme MacLennan MSc , Jemma Hudson PhD , Prof Andrew J Price FRCS , Prof Andrew J Carr FRCS , Matthew Little PhD , Jose Leal PhD , Prof Ray Fitzpatrick DPhil , Prof David W Murray FRCS , Prof Marion K Campbell PhD","doi":"10.1016/S2665-9913(25)00250-4","DOIUrl":"10.1016/S2665-9913(25)00250-4","url":null,"abstract":"<div><h3>Background</h3><div>The Total or Partial Knee Arthroplasty Trial (TOPKAT) aimed to evaluate the difference between total knee replacement (TKR) and partial (unicompartmental) replacement (PKR) for treatment of late-stage medial compartment knee osteoarthritis. As longevity is a key issue for joint replacement, extended follow-up periods are required to fully evaluate the long-term efficacy. In this analysis, we report the 10-year follow-up of the TOPKAT trial.</div></div><div><h3>Methods</h3><div>TOPKAT was a multicentre, randomised, pragmatic comparative effectiveness trial including an expertise component. Patients with medial compartment knee osteoarthritis were enrolled from 27 UK National Health Service (NHS) hospitals and randomly assigned (1:1) to receive PKR or TKR by surgeons who were either expert in and willing to perform both surgeries or by a surgeon with particular expertise in the allocated procedure. Neither surgeons, patients, nor follow-up assessors were masked to allocation, but the implant type was not highlighted at any stage. The primary long-term endpoint was the Oxford Knee Score (OKS) in the intention-to-treat population at 10 years. Cost effectiveness was also assessed. Individuals with relevant lived experience were involved in the study design. This trial is registered with ISRCTN03013488 and <span><span>ClinicalTrials.gov</span><svg><path></path></svg></span>, <span><span>NCT01352247</span><svg><path></path></svg></span>, and is complete.</div></div><div><h3>Findings</h3><div>Between Jan 18, 2010, and Sept 30, 2013, of 962 patients assessed for eligibility, 528 patients (306 [58%] male, 222 [42%] female, mean age 65 years [SD 8·7]) were randomly assigned (PKR n=264; TKR n=264). Follow-up primary outcome response rate for eligible patients (excluding those who had died or withdrew) at 10 years was 326 (73%) of 444. Both operations provided good outcome. The between-group estimates ruled out any individually clinically meaningful differences in mean OKS scores (mean difference 0·27, 95% CI −1·59 to 2·13) or cumulatively over 10 years in the area under the curve analysis (mean difference 0·45, 95% CI −0·98 to 1·88). At 10 years, by treatment received, complications were 53 (22%) of 245 for PKR and 74 (27%) of 270 for TKR, reoperations (including revision) were 21 (9%) for PKR and 23 (9%) for TKR, and revision rates were 15 (6%) for PKR and 11 (4%) for TKR. By treatment allocated, for PKR and TKR respectively, complication occurred in 55 (21%) of 263 and 72 (29%) of 252, reoperations in 20 (8%) and 24 (10%), with revisions in 13 (5%) and 13 (5%) patients. PKR was more cost-effective compared with TKR, being associated with increased health benefits (mean difference in quality-adjusted life years of 0·322, 95% CI −0·069 to 0·712) and lower health-care costs (mean difference in cost −£731, 95% CI −1352 to −110).</div></div><div><h3>Interpretation</h3><div>10-year results comparing TKR and PKR show similar clinical ","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 2","pages":"Pages e116-e126"},"PeriodicalIF":16.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145574819","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":"Time to lift the moratorium on IL-23 inhibitors for axial psoriatic arthritis","authors":"Antonio Tonutti MD ,&nbsp;Kerem Abacar MD ,&nbsp;Tom Macleod PhD ,&nbsp;Carlo Selmi MD PhD ,&nbsp;Prof Dennis McGonagle MD PhD","doi":"10.1016/S2665-9913(25)00287-5","DOIUrl":"10.1016/S2665-9913(25)00287-5","url":null,"abstract":"<div><div>The disappointment stemming from IL-23 inhibition failure in axial spondyloarthritis has resulted in expert panels extrapolating these results to axial psoriatic arthritis, with recommendations against the use of IL-23 inhibitors in this setting. However, post-hoc analyses of clinical trials show clinical improvements in axial psoriatic arthritis following treatment with ustekinumab, guselkumab, and risankizumab. A growing body of real-world evidence also shows substantial amelioration of spinal pain, related outcome measures, and imaging-detected inflammation. Despite negative trials, IL-23 inhibition was associated with C-reactive protein reductions and some improvement in MRI scores in ankylosing spondylitis, indicating some modicum of potential benefit. Furthermore, inadequate response to biological therapies in axial spondyloarthritis was associated with absence of MRI-determined bone marrow oedema, whereas responses to secukinumab in axial spondyloarthritis were independent of bone marrow oedema, pointing to divergent pathophysiological processes. The growing recognition of clinical, microanatomical, and immunological differences between axial spondyloarthritis and axial psoriatic arthritis suggests differential IL-23 pathway dependence. This Personal View aims to provide a deeper examination of these distinctions as a basis to propose reconsidering the current moratorium on IL-23 inhibitors—particularly in phenotypes not linked to HLA-B27—and to potentially expand therapeutic options.</div></div>","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 2","pages":"Pages e144-e156"},"PeriodicalIF":16.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145649824","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":"Thank you to The Lancet Rheumatology's peer reviewers in 2025","authors":"The Lancet Rheumatology Editors","doi":"10.1016/S2665-9913(26)00013-5","DOIUrl":"10.1016/S2665-9913(26)00013-5","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 2","pages":"Pages e78-e82"},"PeriodicalIF":16.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146045230","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":"GLP-1 receptor agonists in rheumatic disease","authors":"The Lancet Rheumatology","doi":"10.1016/S2665-9913(26)00011-1","DOIUrl":"10.1016/S2665-9913(26)00011-1","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 2","pages":"Page e75"},"PeriodicalIF":16.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146045293","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":"Haemophagocytic lymphohistiocytosis mortality across Europe","authors":"Cornelia Lachmann ,&nbsp;Gunnar Lachmann","doi":"10.1016/S2665-9913(25)00339-X","DOIUrl":"10.1016/S2665-9913(25)00339-X","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 2","pages":"Pages e77-e78"},"PeriodicalIF":16.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145655926","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":"Thrombotic microangiopathy as a presentation of anti-synthetase syndrome","authors":"Yu Zuo MD PhD ,&nbsp;Guming Zou MD ,&nbsp;Xiaoming Shu MD PhD","doi":"10.1016/S2665-9913(25)00027-X","DOIUrl":"10.1016/S2665-9913(25)00027-X","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 2","pages":"Pages e157-e158"},"PeriodicalIF":16.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143537504","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}