Rheumatology最新文献

{"title":"Validation of childhood lupus specific targets: ensuring accurate assessment of disease control in younger, lighter paediatric patients","authors":"Chandni Sarker, Andrea L Jorgensen, Kukatharmini Tharmaratnam, Eslam Al-Abadi, Kate Armon, Kathryn Bailey, Marek Bohm, Mary Brennan, Coziana Ciurtin, Janet Gardner-Medwin, Daniel P Hawley, Alison Kinder, Alice Leahy, Gulshan Malik, Zoe McLaren, Elena Moraitis, Ellen Mosley, Athimalaipet V Ramanan, Satyapal Rangaraj, Annie Ratcliffe, Philip Riley, Heather Rostron, Ethan S Sen, Christian M Hedrich, Michael W Beresford, Eve MD Smith","doi":"10.1093/rheumatology/keaf057","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf057","url":null,"abstract":"Objectives To validate novel childhood-onset systemic lupus erythematosus (cSLE) T2T targets including Childhood Lupus Low Disease Activity State (cLLDAS), cSLE Clinical Remission on steroids (cCR), and cSLE Clinical Remission off steroids (cCR-0), as compared with adult-onset SLE (aSLE) targets. Methods Attainment of the aforementioned cSLE-specific and aSLE-specific targets (LLDAS, DORIS 2021 Remission) was assessed at each visit, in UK JSLE Cohort Study patients. Univariable and multivariable Prentice-Williams-Peterson (PWP) gap-time models investigated the impact of target attainment on new damage and severe flare. Results The cohort included 430 cSLE patients. Attainability was comparable between corresponding cSLE and aSLE targets. Achieving cLLDAS (HR 0.18 [0.14, 0.23]), cCR (HR 0.18 [0.13, 0.23]) and cCR-0 (HR 0.17 [0.13, 0.23]) reduced the risk of severe flare (all p < 0.001). Risk of new damage was reduced in those reaching cLLDAS (HR 0.22 [0.11, 0.44]), cCR (HR 0.25 [0.13, 0.49]) and cCR-0 (HR 0.30 [0.15, 0.60]) (all p < 0.001). Inappropriate attainment of LLDAS and DORIS remission occurred at 35 and 52 visits, respectively, in younger (median age 7.3 and 8.8 years) and lighter patients (median weight 26.8 and 37.1 kg) whilst on prednisolone doses that precluded cSLE target attainment (median 0.17 [IQR 0.16–0.24] and 0.13 [IQR 0.11–0.16] mg/kg respectively). Conclusions This study validates novel paediatric-specific targets, demonstrating that achieving cLLDAS, cCR, and cCR-0 reduce risks of new damage and severe flare, which is comparable to aSLE targets. Using cSLE-specific targets prevents misclassification of disease activity in paediatric patients, enabling more accurate disease control assessments in younger, lighter patients.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"14 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143258636","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comment on: Vasculitis associated with VEXAS syndrome.","authors":"Riccardo Bixio, Roberto Padoan","doi":"10.1093/rheumatology/keaf060","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf060","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080317","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comment on: Renal Arteriosclerosis in kidney biopsies associated with higher 10-year atherosclerotic cardiovascular disease in lupus nephritis.","authors":"Seher Sener, Yusuf Ziya Sener","doi":"10.1093/rheumatology/keaf059","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf059","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143190489","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tacrolimus as first line therapy in a U.S. cohort of idiopathic inflammatory myopathies related interstitial lung disease.","authors":"Kamaree J R Harris, Erica A Ludtke, Blaire Goldberg, Natalie N McCall, Justin C Hewlett, Erin M Wilfong","doi":"10.1093/rheumatology/keaf063","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf063","url":null,"abstract":"<p><strong>Objectives: </strong>Calcineurin inhibitors are commonly used to treat idiopathic inflammatory myopathies associated interstitial lung disease (IIM-ILD) in Asia but not in the U.S. Here, we evaluate the efficacy of tacrolimus (TAC) as first-line immunosuppressive therapy in a U.S cohort of IIM-ILD.</p><p><strong>Methods: </strong>This retrospective, single-centre cohort study evaluated the change in absolute forced vital capacity (FVCabs) in IIM-ILD participants after 12 months of treatment with mycophenolate mofetil (MMF), azathioprine (AZA), or TAC. Participants were naïve to immunosuppressive agents other than glucocorticoids and/or intravenous immunoglobulin. Treatment with MMF, AZA, or TAC was at the discretion of their primary clinician. Additional outcomes were transplant free survival, prednisone use, and supplemental oxygen use at 12 months.</p><p><strong>Results: </strong>Thirty-one participants were included in this study. The TAC group was younger and had a shorter disease duration than the MMF/AZA group. All MDA5 patients were treated with TAC. After 12 months of therapy, the FVCabs was unchanged in the MMF/AZA group (2.4 l [95%CI 2.0, 3.2 l] vs 2.3 l [95%CI 2.0, 2.8 l]) and improved in TAC group (2.5 l [95%CI 2.0, 3.0 l] vs.1.9L [95%CI 1.0, 2.6 l]). Transplant free survival was 100% in both groups. All patients in the MMF/AZA group remained on supplemental oxygen at 12 months, but 4/5 patients in the TAC group resolved their oxygen requirement.</p><p><strong>Conclusion: </strong>TAC is effective as a first-line agent in a small, non-randomized U.S. cohort of IIM-ILD enriched for MDA5+ participants. Further work is needed to investigate the relative efficacy of calcineurin inhibitors compared with MMF/AZA in the U.S. population.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080410","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of patient-based disease activity score (PDAS) in the Japanese rheumatoid arthritis patient registry (NinJa registry)","authors":"Hirofumi Shoda, Toshihiro Matsui, Shigeto Tohma, Tetsuji Sawada","doi":"10.1093/rheumatology/keaf067","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf067","url":null,"abstract":"Objective Patient-reported outcomes (PROs) should be regarded as an important factor in the management of rheumatic diseases. The Patient-based disease activity score (PDAS) was developed as a clinically reliable composite measure for evaluating PROs in rheumatoid arthritis (RA) patients. To replicate and further characterize PDAS, we analyzed PDAS and its clinical relevancy in the National Database of Rheumatic Diseases in Japan (NinJa). Methods Clinical data from the 2022 version of NinJa were analyzed. PDAS1 was calculated for each patient, and statistical analyses, including correlation analyses and multiple regression analysis, were conducted to evaluate the relationship between PDAS1 and other clinical measures. Propensity score (PS) matching was used to compare patients treated with different types of disease-modifying anti-rheumatic drugs (DMARDs). Results The number of included patients was 11983. PDAS1 demonstrated strong correlations with DAS28(ESR) (R = 0.89, p&amp;lt; 2.2x1016) and CDAI, indicating its utility in assessing disease activity. The majority of patients (71.8%) achieved PDAS1-defined remission, aligning closely with DAS28 and CDAI remission. PDAS1 was significantly associated with serum rheumatoid factor (RF) titers (R = 0.25, p&amp;lt; 0.001), and RF-positive patients exhibited higher PDAS1 scores. Notably, PS matched comparison revealed that PDAS1 was lower in patients treated with IL-6 inhibitors, compared with those treated with TNF inhibitors, reflecting differences in lower patient global assessment. Conclusion PDAS1 is a reliable and useful tool for evaluating both disease activity and the functional state of RA patients, particularly from the perspective of PROs. Additionally, PDAS1 can be used for conducting clinical studies in RA patients.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"9 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143191902","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association between large vessel vasculitis and inflammatory bowel disease: a case-control study.","authors":"François Maillet, Yann Nguyen, Olivier Espitia, Laurent Perard, Carlo Salvarani, Etienne Rivière, Dieynaba Ndiaye, Cécile-Audrey Durel, Philippe Guilpain, Luc Mouthon, Anna Kernder, Javier Loricera, Pascal Cohen, Isabelle Melki, Claire de Moreuil, Nicolas Limal, Arsène Mékinian, Nathalie Costedoat-Chalumeau, Nathalie Morel, Jonathan Boutemy, Loïc Raffray, Jean-Sébastien Allain, Valérie Devauchelle, Isabelle Kone-Paut, Marc Fabre, Marie Durel, Antoine Dossier, Sébastien Abad, Marcella Visentini, Adrien Bigot, Halil Yildiz, Olivier Fain, Maxime Samson, Guillaume Gondran, Vered Abitbol, Benjamin Terrier","doi":"10.1093/rheumatology/keaf030","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf030","url":null,"abstract":"<p><strong>Objectives: </strong>To describe the characteristics and outcome of patients with the association of large vessel vasculitis (LVV, Takayasu arteritis [TA] or giant cell arteritis [GCA]) and inflammatory bowel disease (IBD).</p><p><strong>Methods: </strong>An observational, multicentre, retrospective case-control study. Cases were LVV-IBD patients from European countries, whereas controls had isolated LVV (iLVV).</p><p><strong>Results: </strong>39 TA-IBD and 12 GCA-IBD cases were enrolled, compared with 52 isolated GCA (iGCA) and 93 isolated TA (iTA) controls. LVV occurred after IBD in 56% in TA-IBD and 75% in GCA-IBD, with a median interval of 1 year (IQR 1-7) in TA-IBD and 8.6 years (IQR 1-17.7) in GCA-IBD. Crohn's disease was more common in TA-IBD (67%), whereas ulcerative colitis was more common in GCA-IBD (58%). Compared with iTA, TA-IBD were significantly younger at diagnosis of TA (median age 27 vs 37 years, p< 0.001) and had more upper limb claudication (36% vs 12%, p= 0.006). GCA-IBD patients had more frequent arterial thickening or stenosis than controls (75% vs 30%, respectively, p= 0.044), and tended to more frequently involve gastrointestinal arteries (20% vs 0%, respectively, p= 0.06). LVV occurred in IBD patients despite treatment with glucocorticoids (36%), azathioprine (25%), or TNF-alpha blockers (29%). The presence of the IBD was not associated with a higher LVV relapse rate in multivariate analysis (adjusted hazard ratio [aHR] 0.62 [0.13-2.83] for GCA and aHR 0.92 [0.44-1.89] for TA).</p><p><strong>Conclusion: </strong>This study identifies specific clinical and imaging characteristics of LVV-IBD patients, in particular a more severe vascular presentation of GCA-IBD patients compared with iGCA patients.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143079873","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Teledidactic peer-tutored musculoskeletal ultrasound training for medical students-the TELMUS study.","authors":"Ricarda Neubauer, Florian Recker, Claus-Juergen Bauer, Simon Petzinna, Pantelis Karakostas, Charlotte Behning, Valentin Sebastian Schäfer","doi":"10.1093/rheumatology/keae709","DOIUrl":"https://doi.org/10.1093/rheumatology/keae709","url":null,"abstract":"<p><strong>Objectives: </strong>Despite growing interest in musculoskeletal ultrasound (MSUS), training opportunities are often limited due to staff shortages and disbalances of expertise between rural and urban areas. Teledidactic approaches have the potential to expand access to training opportunities. This study aims to compare the effectiveness of teledidactic peer-tutored MSUS training to a conventional approach.</p><p><strong>Methods: </strong>A teledidactic course was held by a student tutor following a validated MSUS curriculum. An on-campus MSUS training taught by physician lecturers served as a control. Students were randomly assigned to one of both study groups. Objective structured clinical examinations (OSCE) were conducted before and after the training to objectively measure the learning outcome of the participants. Handheld ultrasound devices (ButterflyIQ®) and iPads (Apple Inc., 8th generation) were provided to the students for the MSUS course.</p><p><strong>Results: </strong>Thirty medical students participated in the study. Prior to the course, baseline OSCE scores were recorded as 13.03/63 (SD ± 4.20) for the on-campus cohort and 13.00/63 (SD ± 6.04) for the teledidactic group. In the post-training OSCE evaluation, the on-campus cohort attained an average score of 56.80/63 (SD ± 4.22), while the TELMUS group averagely achieved 58.53/63 points (SD ± 3.52). While all students' skills increased over time, there was no significant difference between the two cohorts either before or after the course.</p><p><strong>Conclusion: </strong>Peer-tutored, teledidactic MSUS training showed to be non-inferior to the conventional approach and is a promising approach to reduce local and global disparities in educational opportunities regarding MSUS.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080741","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Predictors of lack of response to methotrexate in juvenile idiopathic arthritis associated uveitis.","authors":"Chiara Mapelli, Elisabetta Miserocchi, Marco Nassisi, Gisella B Beretta, Luca Marelli, Gaia Leone, Achille Marino, Cecilia Chighizola, Gilberto Cincinelli, Teresa Giani, Paolo Nucci, Francesco Viola, Giovanni Filocamo, Francesca Minoia","doi":"10.1093/rheumatology/keae079","DOIUrl":"10.1093/rheumatology/keae079","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate clinical features associated with lack of response to MTX in juvenile idiopathic arthritis associated uveitis (JIA-U).</p><p><strong>Methods: </strong>Clinical records of JIA-U patients were retrospectively reviewed. Differences among variables were assessed by Mann-Whitney and χ2 or Fisher's exact tests as appropriate. Association between predictors and requirement of a biological disease-modifying antirheumatic drug (bDMARD) was evaluated by univariate Cox regression analysis and Kaplan-Meier curves. A multivariable logistic model was applied to estimate strength of association, adjusting for potential confounders.</p><p><strong>Results: </strong>Data from 99 JIA-U patients treated with MTX were analysed (82.8% female), with a mean follow up of 9.2 years and a mean age at uveitis onset of 5.7 years. In 65 patients (65.7%) at least one bDMARD to control uveitis was required. Children requiring a bDMARD for uveitis had lower age at JIA and uveitis onset, more frequent polyarticular course, higher frequency of bilateral uveitis at onset and higher prevalence of systemic steroids' use. Despite similar frequency of ocular damage at onset, MTX non-responders showed a higher percentage of ocular damage at last visit. Younger age at JIA onset, polyarticular course and a history of systemic steroids' use resulted independent factors associated to lack of response to MTX at Cox regression analysis. Kaplan-Meier curves and the multivariate model confirm the independent role of both polyarticular course and systemic steroids' use.</p><p><strong>Conclusions: </strong>Younger age at JIA onset, polyarticular course and a history of systemic steroids' use are predictors of a worse response to MTX in JIA-U.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"798-804"},"PeriodicalIF":4.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11781584/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139707765","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction to: Heliotrope-like rash in a febrile child: think of Kawasaki disease.","authors":"","doi":"10.1093/rheumatology/keae299","DOIUrl":"10.1093/rheumatology/keae299","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"887"},"PeriodicalIF":4.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141180580","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessment of damage in Takayasu's arteritis.","authors":"Tanaz A Kermani, Antoine G Sreih, David Cuthbertson, Nader A Khalidi, Curry L Koening, Carol A Langford, Carol A McAlear, Paul A Monach, Larry Moreland, Christian Pagnoux, Rennie L Rhee, Philip Seo, Kenneth J Warrington, Peter A Merkel","doi":"10.1093/rheumatology/keae333","DOIUrl":"10.1093/rheumatology/keae333","url":null,"abstract":"<p><strong>Objectives: </strong>To evaluate damage and clinical characteristics associated with damage in Takayasu's arteritis (TAK).</p><p><strong>Methods: </strong>Patients with TAK enrolled in a multicentre, prospective, observational study underwent standardized damage assessment every 6 months using the Vasculitis Damage Index (VDI) and the Large-Vessel Vasculitis Index of Damage (LVVID).</p><p><strong>Results: </strong>The study included 236 patients with TAK: 92% female, 81% Caucasian; median (25th, 75th percentile) disease duration = 2.6 (0.12, 6.9) years. Eighty-four percent had follow-up: median (25th, 75th) duration 4.1 (1.9, 7.5) years. Items of damage were present in 89% on VDI, 87% on LVVID, in the peripheral vascular (76% VDI, 74% LVVID) and cardiac (40% VDI, 45% LVVID) systems. During follow-up, 42% patients had new damage, including major vessel stenosis/arterial occlusion (8%), limb claudication (6%), hypertension (7%), aortic aneurysm (4%) and bypass surgery (4%). Disease-specific damage accounted for >90% of new items. Older age, relapse and longer duration of follow-up were associated with new damage items; a higher proportion of patients without new damage were on MTX (P <0.05). Among 48 patients diagnosed with TAK within 180 days of enrolment, new damage occurred in 31% on VDI and 52% on LVVID. History of relapse was associated with new damage in the entire cohort while in patients with a recent diagnosis, older age at diagnosis was associated with new damage.</p><p><strong>Conclusion: </strong>Damage is present in >80% of patients with TAK even with recent diagnosis and >40% of patients accrue new, mainly disease-specific damage. Therapies for TAK that better control disease activity and prevent damage should be prioritized.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"682-689"},"PeriodicalIF":4.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141420668","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}