Lancet Rheumatology最新文献

{"title":"Transcutaneous auricular vagus nerve stimulation versus sham stimulation in patients with erosive hand osteoarthritis (ESTIVAL): a randomised, multicentre, double-blind, sham-controlled trial","authors":"Alice Courties MD PhD ,&nbsp;Sophie Tuffet MSc ,&nbsp;Grégoire Cormier MD ,&nbsp;Prof Christian H Roux MD PhD ,&nbsp;Prof Paul Ornetti MD PhD ,&nbsp;Prof Yves-Marie Pers MD PhD ,&nbsp;Prof Jacques-Éric Gottenberg MD PhD ,&nbsp;Eric Lespessailles MD ,&nbsp;Prof Roland Chapurlat MD PhD ,&nbsp;Denis Arniaud MD ,&nbsp;Prof François Rannou MD PhD ,&nbsp;Prof Daniel Wendling MD PhD ,&nbsp;Prof Florent Eymard MD PhD ,&nbsp;Prof Sylvain Mathieu MD PhD ,&nbsp;Prof Pascal Richette MD PhD ,&nbsp;Prof Alain Saraux MD PhD ,&nbsp;Prof Hubert Marotte MD PhD ,&nbsp;Nicolas Poursac MD ,&nbsp;Prof Anne-Christine Rat PhD ,&nbsp;Prof Jean-Philippe Bastard MD PhD ,&nbsp;Prof Jérémie Sellam MD PhD","doi":"10.1016/S2665-9913(25)00226-7","DOIUrl":"10.1016/S2665-9913(25)00226-7","url":null,"abstract":"<div><h3>Background</h3><div>Erosive hand osteoarthritis is a painful and inflammatory disease without effective treatments. In this study we aimed to investigate the efficacy of transcutaneous auricular vagus nerve stimulation (taVNS) compared with sham stimulation in patients with inflammatory erosive hand osteoarthritis.</div></div><div><h3>Methods</h3><div>ESTIVAL was a multicentre, randomised, double-blind, sham-controlled trial done in 18 French hospital centres (two secondary and 16 tertiary centres). Adults (aged ≥18 years), fulfilling the American College of Rheumatology criteria for hand osteoarthritis with at least one erosive interphalangeal joint and ultrasound-confirmed synovitis were randomly assigned (1:1), stratified by site, using centralised web-based randomisation with computer-generated allocation, to receive either daily 20-min taVNS (VAGUSTIM, Schwa Medico, Rouffach, France) or sham stimulation (sham group; no electrical current) for 12 weeks. The primary endpoint was change in hand pain on a visual analogue scale (VAS) from baseline to week 12. Safety was assessed by recording adverse events and serious adverse events at each visit using a standardised form completed by investigators. The primary outcome and safety were analysed in the intention-to-treat population. The trial was registered with <span><span>ClinicalTrials.gov</span><svg><path></path></svg></span>, <span><span>NCT04520516</span><svg><path></path></svg></span>, and is completed. No individuals with lived experience of hand osteoarthritis were involved in the design or conduct of the study.</div></div><div><h3>Findings</h3><div>Between April 8, 2021, and March 29, 2022, 148 patients were enrolled in the study and 142 (96%) were randomly assigned (73 [51%] to the taVNS group and 69 [49%] to the sham group). Overall, the mean age was 66·5 years (SD 8·4), 125 (88%) of 142 participants were female, and 17 (12%) were male. At week 12, 63 (86%) of 73 participants in the taVNS group and 64 (93%) of 69 participants in the sham group provided primary outcome data. Median change in VAS hand pain was –16·0 mm (IQR –32·0 to 5·0) in the taVNS group versus –6·0 mm (–27·0 to 7·0) in the sham group, giving an adjusted between-group difference of –10·0 mm (95% CI –23·0 to 2·0; p=0·22) at week 12; the primary endpoint was not met. No serious adverse events occurred. Adverse events were reported by 22 (30%) of 73 participants in the taVNS and 16 (23%) and 69 participants in the sham group, with no emerging safety concerns.</div></div><div><h3>Interpretation</h3><div>In participants with erosive hand osteoarthritis, taVNS was safe and well tolerated. Although the primary endpoint was not met, the consistent pain reduction observed in patients with greater synovial inflammation suggests that taVNS merits further investigation in this erosive hand osteoarthritis population.</div></div><div><h3>Funding</h3><div>French Ministry of Health.</div></div>","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 2","pages":"Pages e98-e107"},"PeriodicalIF":16.4,"publicationDate":"2025-12-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145745064","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":"Skin cancer in inflammatory arthritis: should we advise screening?","authors":"Konstantinos D Vassilakis MD ,&nbsp;Kyriaki Lampadaki MD ,&nbsp;Elena Nikiphorou MD PhD ,&nbsp;George E Fragoulis MD PhD","doi":"10.1016/S2665-9913(25)00312-1","DOIUrl":"10.1016/S2665-9913(25)00312-1","url":null,"abstract":"<div><div>Inflammatory arthritis refers to a group of related clinical entities marked mainly by inflammation of the joints, the spine, or both. Rheumatoid arthritis and spondyloarthritis (which includes psoriatic arthritis and axial spondyloarthritis) are the most common forms of inflammatory arthritis. As more studies are conducted, there are accumulating data suggesting the elevated risk of skin cancer, including both melanoma and non-melanoma skin cancer, in inflammatory arthritis compared with the general population. In this context, the question of whether screening for skin cancer should be recommended in individuals with inflammatory arthritis is more relevant than ever. In this Review, we discuss the current knowledge on the risk and possible associations of melanoma and non-melanoma skin cancer in inflammatory arthritis with the aim of raising awareness within the rheumatology community about the risk of skin cancer, screening, and guidance.</div></div>","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 2","pages":"Pages e127-e143"},"PeriodicalIF":16.4,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145679158","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 ,&nbsp;Reza Omidvar MSc ,&nbsp;Francesca Gonnelli PhD ,&nbsp;Adam Taylor MEng ,&nbsp;Thomas Trimble BSc ,&nbsp;Mark Bishton PhD ,&nbsp;Prof Matthew Collin PhD ,&nbsp;Prof Richard Hubbard DM ,&nbsp;Peter C Lanyon DM ,&nbsp;Rachel S Tattersall PhD ,&nbsp;Jessica J Manson PhD ,&nbsp;Fiona A Pearce PhD","doi":"10.1016/S2665-9913(25)00292-9","DOIUrl":"10.1016/S2665-9913(25)00292-9","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Background&lt;/h3&gt;&lt;div&gt;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.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;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.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Findings&lt;/h3&gt;&lt;div&gt;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).&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Interpretation&lt;/h3&gt;&lt;div&gt;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.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Funding&lt;/h3&gt;&lt;div&gt;Medical Research Council Rare Disease Platform Node","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 2","pages":"Pages e108-e115"},"PeriodicalIF":16.4,"publicationDate":"2025-11-29","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":"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":"2025-11-29","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":"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":"2025-11-27","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":"Progressive bone destruction of the hand in Gorham-Stout disease.","authors":"Farzana Shumy, Kotaro Matsumoto","doi":"10.1016/S2665-9913(25)00286-3","DOIUrl":"https://doi.org/10.1016/S2665-9913(25)00286-3","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":16.4,"publicationDate":"2025-11-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145641299","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":"Intensive biological DMARD-first strategy versus standard step-up care in psoriatic arthritis (STAMP): 1-year results from a multicentre, open-label, randomised controlled trial comparing two treat-to-target strategies","authors":"Gonul Hazal Koc MD ,&nbsp;Marc R Kok MD ,&nbsp;Fazira R Kasiem MD ,&nbsp;Jolanda J Luime PhD ,&nbsp;Ilja Tchetverikov MD ,&nbsp;Kim Wilhelm-de Jong BSc ,&nbsp;Lindy A Korswagen MD ,&nbsp;Natasja H A M Denissen MD ,&nbsp;Yvonne P M Goekoop-Ruiterman MD ,&nbsp;Paul Baudoin MD ,&nbsp;Petra Kok MD ,&nbsp;Reinhard Bos MD ,&nbsp;Prof Radboud J E M Dolhain MD ,&nbsp;Marijn Vis MD","doi":"10.1016/S2665-9913(25)00223-1","DOIUrl":"10.1016/S2665-9913(25)00223-1","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Background&lt;/h3&gt;&lt;div&gt;Treat-to-target strategies have previously been shown to improve outcomes in psoriatic arthritis. We aimed to evaluate whether a treat-to-target strategy using early intensive treatment with the IL-17A inhibitor secukinumab improves outcomes compared with a standard step-up treat-to-target approach in patients with psoriatic arthritis.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;This multicentre, open-label, randomised controlled trial was done in 11 general hospitals and one academic hospital in the Netherlands. Eligible patients were aged 18 years or older with newly diagnosed psoriatic arthritis, fulfilled the Classification Criteria for Psoriatic Arthritis (CASPAR) criteria, had a minimum of two swollen joints at initial visit and were naive to any disease-modifying antirheumatic drugs. Participants were randomly assigned (1:1) to receive either early secukinumab treatment or standard of care. The early secukinumab group received subcutaneous secukinumab 300 mg at baseline and then every 4 weeks, plus weekly oral methotrexate 15 mg for a maximum of 12 months. If minimal disease activity (assessed at 3 month intervals) was not reached, secukinumab treatment was switched to a TNF inhibitor, followed by a second TNF inhibitor if necessary, and finally the second TNF inhibitor was stopped and treatment switched to apremilast. The standard of care group received weekly oral methotrexate 15 mg, escalating to 25mg at 6 weeks, for a maximum of 12 months. If minimal disease activity was not reached treatment was escalated according to standard of care. Patients in both groups also received a single intramuscular injection of methylprednisolone 80 mg at baseline. The primary outcome was the proportion of patients with an ACR50 response at 6 months in the intention-to-treat population. The safety set included all patients who received at least one dose of study treatment. People with lived experience of psoriatic arthritis were involved in the study design. This trial was registered with ISRCTN (ISRCTN76054545).&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Findings&lt;/h3&gt;&lt;div&gt;Between Dec 19, 2019, and Oct 19, 2023, 130 patients were screened for eligibilty and 120 patients were enrolled and randomly assigned to receive either early secukinumab treatment (n=60) or standard of care (n=60). Across the two groups, 49 (41%) of 120 patients were female, 71 (59%) were male, and the mean age was 49 years (SD 15). Of the 110 patients for whom ethnicity data were available, 108 (98%) were of Dutch origin. At month 6, ACR50 was reported in 25 (42%) of 60 patients in the early secukinumab group and 21 (35%) of 60 patients in the standard of care group (relative risk 1·19, 95% CI 0·75–1·88; p=0·45); thus, the primary outcome was not met. Adverse events occurred in 30 (50%) of 60 patients in the early secukinumab group and 32 (53%) of 60 patients in the standard of care group. Serious adverse events were reported in six (10%) patients in the early secukinumab group and fi","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"8 1","pages":"Pages e23-e32"},"PeriodicalIF":16.4,"publicationDate":"2025-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145589411","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":"Current status of cardiovascular risk factor control in antiphospholipid syndrome: where are we now?","authors":"Can Huang, Mengtao Li","doi":"10.1016/S2665-9913(25)00285-1","DOIUrl":"https://doi.org/10.1016/S2665-9913(25)00285-1","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":16.4,"publicationDate":"2025-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145582695","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":"Cardiovascular risk factor control in antiphospholipid syndrome, and differences between primary and systemic lupus erythematosus-related antiphospholipid syndrome (SURF-SLE and APS project): a cross-sectional study of 1003 individuals from 11 countries.","authors":"Eleana Bolla, Anne Grete Semb, Michelle Petri, Petros P Sfikakis, Bahar Artim-Esen, Gabriela Hernandez-Molina, Eric Hachulla, Haner Direskeneli, George A Karpouzas, Dina Zucchi, Mohit Goyal, Nathalie Costedoat-Chalumeau, Angela Tincani, Ayten Yazici, Karoline Lerang, Anne Troldborg, Sofia Ajeganova, Tatiana V Popkova, Elisabet Svenungsson, Nikos Pantazis, Maria G Tektonidou","doi":"10.1016/S2665-9913(25)00257-7","DOIUrl":"https://doi.org/10.1016/S2665-9913(25)00257-7","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;The European Alliance of Associations for Rheumatology recommendations for cardiovascular risk management highlighted the importance of traditional cardiovascular risk factor control in antiphospholipid syndrome (APS). However, cardiovascular risk factor target attainment in APS and differences between primary APS and systemic lupus erythematosus (SLE)-related APS remain uncertain.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;Cardiovascular risk factor data were collected from medical records of patients in 17 centres from 11 countries between Jan 1, 2015, and Jan 1, 2020 (extended to 2022 for some centres unable to complete the survey by the end of 2020 due to the COVID-19 pandemic), and analysed cross-sectionally. Included patients were 18 years or older and met the revised Sapporo APS classification criteria. Patients who also met the 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE were classified as having SLE-related APS. Patients with APS in association with systemic autoimmune diseases other than SLE were excluded. Cardiovascular risk was estimated using the Systematic Coronary Risk Evaluation algorithm, and cardiovascular risk factor target attainment was assessed using European Society of Cardiology guidelines. Unadjusted and adjusted mixed effects logistic regression models were fitted. People with lived experience were not involved in the study design.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Findings: &lt;/strong&gt;In total, 1003 patients with APS were included (779 [78%] women and 224 [22%] men; 662 [66%] of 1000 were White), with a median age of 47·0 years (IQR 38·0-57·0) and a median disease duration of 11·0 years (5·0-18·0). 539 (54%) patients had primary APS and 464 (46%) had SLE-related APS. We found a high prevalence of cardiovascular risk factors (hypertension, 411 [41%] of 1003; hyperlipidaemia, 344 [34%] of 1003; obesity, 295 [32%] of 919; current smoking, 186 [19%] of 963) and inadequate individual (blood pressure less than 130/80 mm Hg, BMI, and lipids) and composite cardiovascular risk factor control in all patients. A higher prevalence of hypertension (234 [50%] of 464 vs 177 [33%] of 539; p&lt;0·0001) and hyperlipidaemia (184 [40%] of 464 vs 160 [30%] of 539; p=0·0009) was observed in SLE-related APS versus primary APS, but a lower prevalence of current smoking (72 [16%] of 452 vs 114 [22%] of 511; p=0·012). Patients with primary APS had worse target attainment for smoking cessation (397 [78%] of 511 vs 380 [84%] of 452; p=0·012), blood pressure less than 130/80 mm Hg (246 [48%] of 514 vs 258 [57%] of 456; p=0·0067), and two or more cardiovascular risk factor targets (of smoking, BMI, blood pressure, LDL) than patients with SLE-related APS in the entire group, as well as worse target attainment for smoking cessation, blood pressure less than 130/80 mmHg, BMI, LDL, triglycerides, two or more and three or more targets in the high and very high cardiovascular risk subgroup. Age and arterial ","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":16.4,"publicationDate":"2025-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145582628","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":"Clinical phenotypes, classification, and long-term outcomes of childhood-onset Sjögren's disease into adulthood: a single-centre cohort study.","authors":"Coziana Ciurtin, Junjie Peng, Ruby Taylor-Gotch, Hannah Peckham, Robert Wilson, Muthana Al Obaidi, Elizabeth C Jury","doi":"10.1016/S2665-9913(25)00283-8","DOIUrl":"https://doi.org/10.1016/S2665-9913(25)00283-8","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;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.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;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.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Findings: &lt;/strong&gt;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 vs 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] vs low ESSDAI: mean 0·8 [1·1]; p&lt;0·0001 and high ESSPRI: mean 5·6 [2·7] vs 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).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Interpretation: &lt;/strong&gt;This preliminary evaluation of long-term outcomes of childhood-onset Sjögren's dis","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":16.4,"publicationDate":"2025-11-19","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}