OBJECTIVESTo compare the severity at diagnosis and disease activity during follow-up between juvenile (jDM) and adult-onset dermatomyositis (aDM).METHODSPatients with DM fulfilling the 2017 ACR/EULAR criteria and tested for myositis-specific antibodies were included. Overall severity at diagnosis was defined by at least one of the following: severe muscle impairment, symptomatic interstitial lung disease (ILD), gastrointestinal (GI) vasculitis, myocarditis, severe skin ulceration, or admission to an intensive care unit. Clinically inactive disease and remission were defined according to PRINTO group criteria and IMACS guidelines.RESULTS201 patients were included: 123 with aDM (≥ 18 years) and 78 with jDM (< 18 years). Female predominance was less pronounced and GI involvement was more frequent in jDM. Anti-NXP2 antibodies (Abs) were more frequent in jDM. Severe disease at diagnosis was present in 44% of cases, with symptomatic ILD more common in adults and GI vasculitis more common in children. No difference was observed in severe muscle disease. In jDM, anti-NXP2 Abs (OR = 6.29 [2.00-23.07], p= 0.003) and joint involvement (OR = 3.44 [1.33-11.47], p= 0.03) were associated with severe disease at diagnosis, while in aDM, anti-Mi2 Abs were associated with a lower likelihood of severe disease (OR = 0.12 [0.02-0.42], p= 0.003). No significant difference was observed in clinically inactive disease or remission at the last follow-up. Severe infections occurred in 25% of patients.CONCLUSIONSeverity profiles differ by age of DM onset. Autoantibodies are key indicators of severity at diagnosis in both juvenile DM and adult DM.
{"title":"Comparison of disease severity and activity between juvenile- and adult-onset dermatomyositis: a multicenter cohort study.","authors":"Agathe Sinnaeve,Brigitte Bader-Meunier,Anne Mirguet,Thomas Moulinet,Irène Lemelle,Anne-Claire Bursztejn,Joëlle Terzic,Ariane Zaloszyc,Alain Meyer,Thierry Martin,Claire Ballot-Schmit,Julien Campagne,Loïs Bolko,Bernard Bonnotte,Roland Jaussaud,Olivier Benveniste,Paul Decker","doi":"10.1093/rheumatology/keag032","DOIUrl":"https://doi.org/10.1093/rheumatology/keag032","url":null,"abstract":"OBJECTIVESTo compare the severity at diagnosis and disease activity during follow-up between juvenile (jDM) and adult-onset dermatomyositis (aDM).METHODSPatients with DM fulfilling the 2017 ACR/EULAR criteria and tested for myositis-specific antibodies were included. Overall severity at diagnosis was defined by at least one of the following: severe muscle impairment, symptomatic interstitial lung disease (ILD), gastrointestinal (GI) vasculitis, myocarditis, severe skin ulceration, or admission to an intensive care unit. Clinically inactive disease and remission were defined according to PRINTO group criteria and IMACS guidelines.RESULTS201 patients were included: 123 with aDM (≥ 18 years) and 78 with jDM (< 18 years). Female predominance was less pronounced and GI involvement was more frequent in jDM. Anti-NXP2 antibodies (Abs) were more frequent in jDM. Severe disease at diagnosis was present in 44% of cases, with symptomatic ILD more common in adults and GI vasculitis more common in children. No difference was observed in severe muscle disease. In jDM, anti-NXP2 Abs (OR = 6.29 [2.00-23.07], p= 0.003) and joint involvement (OR = 3.44 [1.33-11.47], p= 0.03) were associated with severe disease at diagnosis, while in aDM, anti-Mi2 Abs were associated with a lower likelihood of severe disease (OR = 0.12 [0.02-0.42], p= 0.003). No significant difference was observed in clinically inactive disease or remission at the last follow-up. Severe infections occurred in 25% of patients.CONCLUSIONSeverity profiles differ by age of DM onset. Autoantibodies are key indicators of severity at diagnosis in both juvenile DM and adult DM.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"56 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146033786","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}
Pub Date : 2026-01-24DOI: 10.1093/rheumatology/keag043
Rajiv Ark,Victoria Allen,Katie Bechman,Zijing Yang,Deepak Nagra,Mark D Russell,James Galloway
There are several pressing reasons to re-examine shingles and strategies for its prevention through vaccination. Firstly, the UK has recently recommended the introduction of a national childhood varicella vaccination programme, and this may impact the epidemiology of shingles. Secondly, treatments for immune-mediated inflammatory diseases are increasingly targeting immunological pathways essential for antiviral defence. Thirdly, we now have access to a highly efficacious shingles vaccine. This review article will explore each of these developments in a way that is globally relevant, incorporating international recommendations.
{"title":"Shingles vaccination in immune-mediated inflammatory diseases: a narrative review.","authors":"Rajiv Ark,Victoria Allen,Katie Bechman,Zijing Yang,Deepak Nagra,Mark D Russell,James Galloway","doi":"10.1093/rheumatology/keag043","DOIUrl":"https://doi.org/10.1093/rheumatology/keag043","url":null,"abstract":"There are several pressing reasons to re-examine shingles and strategies for its prevention through vaccination. Firstly, the UK has recently recommended the introduction of a national childhood varicella vaccination programme, and this may impact the epidemiology of shingles. Secondly, treatments for immune-mediated inflammatory diseases are increasingly targeting immunological pathways essential for antiviral defence. Thirdly, we now have access to a highly efficacious shingles vaccine. This review article will explore each of these developments in a way that is globally relevant, incorporating international recommendations.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"284 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146033785","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}
Pub Date : 2026-01-24DOI: 10.1093/rheumatology/keag036
Fuensanta Gómez-Bernal,Juan C Quevedo Abeledo,Cristina Almeida-Santiago,Yolanda Fernández-Cladera,Enrique García-Barrera,Luisa M Villar,Raquel Largo,J Gonzalo Ocejo-Vinyals,Miguel Á González-Gay,Iván Ferraz-Amaro
OBJECTIVEInterferons (IFNs) are implicated in the pathogenesis of rheumatoid arthritis (RA). However, the relationship between serum levels of IFN-α and IFN-γ and the clinical manifestations of RA, particularly regarding cardiovascular comorbidity, remains poorly established. In this study, we aimed to investigate the associations between serum concentrations of IFN-α and IFN-γ and the spectrum of disease manifestations in RA, with special attention to cardiovascular involvement.METHODSA total of 216 RA patients were recruited. They underwent comprehensive evaluations, including disease-related characteristics and disease activity indices. Moreover, complete lipid profile, insulin resistance indices, metabolic syndrome criteria, and carotid ultrasound for carotid stiffness, intima-media thickness and carotid plaque detection were assessed. IFN-α and IFN-γ serum levels were measured using Simoa (Single Molecule Array) technique. A multivariable linear regression analysis was performed to examine the associations between the disease characteristics and IFN-α and IFN-γ.RESULTSSerum levels of IFN-α and IFN-γ were significantly correlated with each other and were also associated with circulating levels of interleukin 2, 6, and 8. After multivariable adjustment, higher serum IFN-α levels were positively associated with rheumatoid factor and anti-citrullinated protein antibodies positivity, as well as with increased disease activity scores. In contrast, IFN-γ levels showed no significant associations with most clinical manifestations of RA. Furthermore, neither IFN-α nor IFN-γ levels were related to cardiovascular comorbidities, including lipid profiles, insulin resistance indices, or carotid ultrasound findings.CONCLUSIONSerum levels of IFN-α, but not IFN-γ, are associated with disease activity in patients with RA. However, neither IFN is correlated with the cardiovascular comorbidities commonly observed in this population.
{"title":"Clinical and serological associations of interferon-α and interferon-γ in rheumatoid arthritis patients.","authors":"Fuensanta Gómez-Bernal,Juan C Quevedo Abeledo,Cristina Almeida-Santiago,Yolanda Fernández-Cladera,Enrique García-Barrera,Luisa M Villar,Raquel Largo,J Gonzalo Ocejo-Vinyals,Miguel Á González-Gay,Iván Ferraz-Amaro","doi":"10.1093/rheumatology/keag036","DOIUrl":"https://doi.org/10.1093/rheumatology/keag036","url":null,"abstract":"OBJECTIVEInterferons (IFNs) are implicated in the pathogenesis of rheumatoid arthritis (RA). However, the relationship between serum levels of IFN-α and IFN-γ and the clinical manifestations of RA, particularly regarding cardiovascular comorbidity, remains poorly established. In this study, we aimed to investigate the associations between serum concentrations of IFN-α and IFN-γ and the spectrum of disease manifestations in RA, with special attention to cardiovascular involvement.METHODSA total of 216 RA patients were recruited. They underwent comprehensive evaluations, including disease-related characteristics and disease activity indices. Moreover, complete lipid profile, insulin resistance indices, metabolic syndrome criteria, and carotid ultrasound for carotid stiffness, intima-media thickness and carotid plaque detection were assessed. IFN-α and IFN-γ serum levels were measured using Simoa (Single Molecule Array) technique. A multivariable linear regression analysis was performed to examine the associations between the disease characteristics and IFN-α and IFN-γ.RESULTSSerum levels of IFN-α and IFN-γ were significantly correlated with each other and were also associated with circulating levels of interleukin 2, 6, and 8. After multivariable adjustment, higher serum IFN-α levels were positively associated with rheumatoid factor and anti-citrullinated protein antibodies positivity, as well as with increased disease activity scores. In contrast, IFN-γ levels showed no significant associations with most clinical manifestations of RA. Furthermore, neither IFN-α nor IFN-γ levels were related to cardiovascular comorbidities, including lipid profiles, insulin resistance indices, or carotid ultrasound findings.CONCLUSIONSerum levels of IFN-α, but not IFN-γ, are associated with disease activity in patients with RA. However, neither IFN is correlated with the cardiovascular comorbidities commonly observed in this population.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"117 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146033781","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}
OBJECTIVETo evaluate the diagnostic yield of whole exome sequencing (WES) vs targeted gene panel (TGP) testing in patients evaluated for autoinflammation at Great Ormond Street Hospital Autoinflammation Centre of Excellence.METHODSWe retrospectively analysed 476 patients who underwent TGP testing between 2015-2022; and 210 patients who underwent WES between 2022-2025. Analysis of WES data combined: 1. a virtual gene panel of genes (germline and somatic) associated with inflammation; 2. agnostic filtering according to ClinVar classification of pathogenicity; 3. copy number variant analysis using ExomeDepth; and 4. phenotype-driven prioritisation using Exomiser.RESULTSTGP testing identified molecular diagnoses in 71/476 patients (14.9%). WES increased the molecular diagnostic yield to 41/210 patients (19.5%). WES also enabled the discovery of novel genotype-phenotype associations. Significant incidental findings were identified in 29/210 (13.8%) of cases, including variants predisposing to cancer or cardiomyopathy.CONCLUSIONIn patients with suspected autoinflammation, WES increased diagnostic yield compared with TGP testing while providing additional clinical value through novel gene discovery and capacity for future systematic reanalysis of unsolved cases. Incidental findings required careful and explicit a priori patient counselling and informed consent before offering WES. While there is an inevitable shift to whole genome sequencing, significant challenges remain, including costly large-data handling and storage, and uncertainty about the interpretation of variants in non-exonic regions. Our findings, therefore, demonstrate significant clinical impact of WES for the workup of autoinflammation, with pragmatic utility for timely return of results.
{"title":"Clinical impact of whole exome sequencing: ten years of the Great Ormond Street Hospital Autoinflammation Centre of Excellence experience.","authors":"Fiona Price-Kuehne,Alice Burleigh,Ebun Omoyinmi,Dara McCreary,Kirsty McLellan,Sana Ibrahim,Ying Hong,Katia Nazmutdinova,Despina Eleftheriou,Paul Brogan","doi":"10.1093/rheumatology/keag046","DOIUrl":"https://doi.org/10.1093/rheumatology/keag046","url":null,"abstract":"OBJECTIVETo evaluate the diagnostic yield of whole exome sequencing (WES) vs targeted gene panel (TGP) testing in patients evaluated for autoinflammation at Great Ormond Street Hospital Autoinflammation Centre of Excellence.METHODSWe retrospectively analysed 476 patients who underwent TGP testing between 2015-2022; and 210 patients who underwent WES between 2022-2025. Analysis of WES data combined: 1. a virtual gene panel of genes (germline and somatic) associated with inflammation; 2. agnostic filtering according to ClinVar classification of pathogenicity; 3. copy number variant analysis using ExomeDepth; and 4. phenotype-driven prioritisation using Exomiser.RESULTSTGP testing identified molecular diagnoses in 71/476 patients (14.9%). WES increased the molecular diagnostic yield to 41/210 patients (19.5%). WES also enabled the discovery of novel genotype-phenotype associations. Significant incidental findings were identified in 29/210 (13.8%) of cases, including variants predisposing to cancer or cardiomyopathy.CONCLUSIONIn patients with suspected autoinflammation, WES increased diagnostic yield compared with TGP testing while providing additional clinical value through novel gene discovery and capacity for future systematic reanalysis of unsolved cases. Incidental findings required careful and explicit a priori patient counselling and informed consent before offering WES. While there is an inevitable shift to whole genome sequencing, significant challenges remain, including costly large-data handling and storage, and uncertainty about the interpretation of variants in non-exonic regions. Our findings, therefore, demonstrate significant clinical impact of WES for the workup of autoinflammation, with pragmatic utility for timely return of results.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"42 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146033783","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}
Pub Date : 2026-01-24DOI: 10.1093/rheumatology/keag037
Thomas Khoo,Syed Ali,Athena Chin
{"title":"Is air pollution a modifiable risk factor for autoantibody genesis?","authors":"Thomas Khoo,Syed Ali,Athena Chin","doi":"10.1093/rheumatology/keag037","DOIUrl":"https://doi.org/10.1093/rheumatology/keag037","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"66 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146033784","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}
Pub Date : 2026-01-24DOI: 10.1093/rheumatology/keag045
Konrad Hüther,Nicolai Leuchten,Martin Aringer
OBJECTIVESTo compare the proportion of patients in remission and low disease activity (LDA) between rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE).METHODSOutpatient record data of 100 patients with SLE and of 100 matched patients with seropositive RA, with at least 3 consecutive outpatient visits each between August, 2021, and January, 2023, were compared. Documentation at every visit included SLE Disease Activity Index 2000 (SLEDAI-2K) for SLE, Simplified Disease Activity Index (SDAI) for RA, and visual analogue scales (VAS). Remission and LDA were defined by SDAI cut-offs in RA and Definition of Remission in SLE (DORIS) remission and Lupus Low Disease Activity State (LLDAS) in SLE.RESULTSRemission or LDA were present in 347 (88.5%) of 392 visits of SLE and 354 (91.0%) of 389 visits of RA patients (p= 0.2885). Remission was more common in SLE (79.3%) than RA (69.4%) data points (p= 0.0018). Physician global assessment (PhGA) was not significantly different between SLE and RA patients in low disease activity, and only slightly higher in SLE in remission (p= 0.0002). C-reactive protein (CRP) was increased (p= 0.0107), but the erythrocyte sedimentation rate was similar, in active RA vs active SLE.CONCLUSIONTreat-to-target (T2T) goals were similarly met for SLE and RA patients on current treatment. Despite differences between the diseases, and according differences in definitions, these data suggest that the SLE definitions for remission and LDA were successfully modelled on those of RA. The rates with which favorable states were noted supports the T2T approach in SLE.
{"title":"Treating-to-target: remission and low disease activity in systemic lupus erythematosus versus rheumatoid arthritis.","authors":"Konrad Hüther,Nicolai Leuchten,Martin Aringer","doi":"10.1093/rheumatology/keag045","DOIUrl":"https://doi.org/10.1093/rheumatology/keag045","url":null,"abstract":"OBJECTIVESTo compare the proportion of patients in remission and low disease activity (LDA) between rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE).METHODSOutpatient record data of 100 patients with SLE and of 100 matched patients with seropositive RA, with at least 3 consecutive outpatient visits each between August, 2021, and January, 2023, were compared. Documentation at every visit included SLE Disease Activity Index 2000 (SLEDAI-2K) for SLE, Simplified Disease Activity Index (SDAI) for RA, and visual analogue scales (VAS). Remission and LDA were defined by SDAI cut-offs in RA and Definition of Remission in SLE (DORIS) remission and Lupus Low Disease Activity State (LLDAS) in SLE.RESULTSRemission or LDA were present in 347 (88.5%) of 392 visits of SLE and 354 (91.0%) of 389 visits of RA patients (p= 0.2885). Remission was more common in SLE (79.3%) than RA (69.4%) data points (p= 0.0018). Physician global assessment (PhGA) was not significantly different between SLE and RA patients in low disease activity, and only slightly higher in SLE in remission (p= 0.0002). C-reactive protein (CRP) was increased (p= 0.0107), but the erythrocyte sedimentation rate was similar, in active RA vs active SLE.CONCLUSIONTreat-to-target (T2T) goals were similarly met for SLE and RA patients on current treatment. Despite differences between the diseases, and according differences in definitions, these data suggest that the SLE definitions for remission and LDA were successfully modelled on those of RA. The rates with which favorable states were noted supports the T2T approach in SLE.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"273 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146033788","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}
Pub Date : 2026-01-21DOI: 10.1093/rheumatology/keag022
Grégoire Martin de Frémont,Lisa Rudolph,Rui da Silva Rodrigues,Malin Hedlund,Tilen Tršelič,Sven Wegner,Ashley Orillion,Jennifer Painter,Rebecca Zaha,Håkan Eliasson,Felicia Nordenstam,Gunnar Bergman,Sven-Erik Sonesson,Marie Wahren-Herlenius
OBJECTIVESTo investigate the relationship between maternal disease, anti-rheumatic treatment, autoantibody levels and fetal atrioventricular (AV) conduction.METHODSA total of 324 pregnancies from two groups of anti-Ro/La autoantibody positive women were included. The Surveillance group consisted of pregnancies with known anti-Ro positivity followed from early pregnancy; n = 302, including 9 with AV block (AVB), and the Bradycardia group with no prior knowledge of anti-Ro/La autoantibodies and referred upon detection of fetal bradycardia, with a subsequent AVB II-III diagnosis (n = 22). Fetal AV conduction and routine anti-Ro52, anti-Ro60 and anti-La autoantibody levels were analysed.RESULTSWomen with primary Sjögren disease (SjD) had longer fetal AV time intervals (130.5 ± 11.9 vs 125.8 ± 8.7 ms, p= 0.002) compared with autoantibody-positive women with neither SjD nor SLE. Notably, azathioprine, mostly used in women with SLE, was associated with shorter fetal AV time intervals compared with autoantibody-positive pregnancies without azathioprine treatment (122.5 ± 8.9 vs 128.7 ± 11.0 ms, p= 0.038). For antibody-based prediction, anti-Ro52, anti-Ro60 and anti-La antibody levels obtained from routine assays could identify nearly 20% of pregnancies as low risk, and high levels (≥8 U/ml) of anti-La antibodies were associated with an increased risk of AVB I-III (OR = 6.0, 1.26-28.5). However, the positive predictive value for AVB II-III of the three autoantibodies did not reach 5% for a sensitivity of 100%.CONCLUSIONThis report, based on over 20 years of surveillance of anti-Ro positive pregnancies, identifies associations between maternal disease, their treatments, and fetal AV conduction. Routine detection assays for anti-Ro/La antibodies had low performance in predicting AVB among an anti-Ro/La positive population.
目的探讨母体疾病、抗风湿治疗、自身抗体水平与胎儿房室传导的关系。方法收集两组抗ro /La自身抗体阳性孕妇324例。监测组包括已知抗- ro阳性的妊娠，从妊娠早期开始；n = 302，包括9例AV阻滞（AVB），以及心动过缓组，先前不知道抗ro /La自身抗体，在检测到胎儿心动过缓后，随后诊断为AVB II-III （n = 22）。分析胎儿AV传导及常规抗ro52、抗ro60、抗la自身抗体水平。结果原发性Sjögren疾病（SjD）女性与自身抗体阳性的SjD和SLE女性相比，胎儿AV时间间隔（130.5±11.9 ms vs 125.8±8.7 ms, p= 0.002）更长。值得注意的是，硫唑嘌呤主要用于SLE女性，与未使用硫唑嘌呤治疗的自身抗体阳性妊娠相比，胎儿AV时间间隔更短（122.5±8.9 ms vs 128.7±11.0 ms, p= 0.038）。对于基于抗体的预测，常规检测获得的抗ro52、抗ro60和抗la抗体水平可识别近20%的妊娠为低风险妊娠，高水平（≥8 U/ml）的抗la抗体与AVB I-III风险增加相关（OR = 6.0, 1.26-28.5）。然而，三种自身抗体对AVB II-III的阳性预测值未达到5%，敏感性为100%。本报告基于对抗ro阳性妊娠的20多年监测，确定了母体疾病及其治疗与胎儿房室传导之间的关系。常规抗ro /La抗体检测方法在预测抗ro /La阳性人群中AVB的效果较差。
{"title":"Prediction of fetal atrioventricular conduction using maternal disease, treatment, and anti-Ro/La autoantibody levels.","authors":"Grégoire Martin de Frémont,Lisa Rudolph,Rui da Silva Rodrigues,Malin Hedlund,Tilen Tršelič,Sven Wegner,Ashley Orillion,Jennifer Painter,Rebecca Zaha,Håkan Eliasson,Felicia Nordenstam,Gunnar Bergman,Sven-Erik Sonesson,Marie Wahren-Herlenius","doi":"10.1093/rheumatology/keag022","DOIUrl":"https://doi.org/10.1093/rheumatology/keag022","url":null,"abstract":"OBJECTIVESTo investigate the relationship between maternal disease, anti-rheumatic treatment, autoantibody levels and fetal atrioventricular (AV) conduction.METHODSA total of 324 pregnancies from two groups of anti-Ro/La autoantibody positive women were included. The Surveillance group consisted of pregnancies with known anti-Ro positivity followed from early pregnancy; n = 302, including 9 with AV block (AVB), and the Bradycardia group with no prior knowledge of anti-Ro/La autoantibodies and referred upon detection of fetal bradycardia, with a subsequent AVB II-III diagnosis (n = 22). Fetal AV conduction and routine anti-Ro52, anti-Ro60 and anti-La autoantibody levels were analysed.RESULTSWomen with primary Sjögren disease (SjD) had longer fetal AV time intervals (130.5 ± 11.9 vs 125.8 ± 8.7 ms, p= 0.002) compared with autoantibody-positive women with neither SjD nor SLE. Notably, azathioprine, mostly used in women with SLE, was associated with shorter fetal AV time intervals compared with autoantibody-positive pregnancies without azathioprine treatment (122.5 ± 8.9 vs 128.7 ± 11.0 ms, p= 0.038). For antibody-based prediction, anti-Ro52, anti-Ro60 and anti-La antibody levels obtained from routine assays could identify nearly 20% of pregnancies as low risk, and high levels (≥8 U/ml) of anti-La antibodies were associated with an increased risk of AVB I-III (OR = 6.0, 1.26-28.5). However, the positive predictive value for AVB II-III of the three autoantibodies did not reach 5% for a sensitivity of 100%.CONCLUSIONThis report, based on over 20 years of surveillance of anti-Ro positive pregnancies, identifies associations between maternal disease, their treatments, and fetal AV conduction. Routine detection assays for anti-Ro/La antibodies had low performance in predicting AVB among an anti-Ro/La positive population.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"81 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146015160","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}
Pub Date : 2026-01-21DOI: 10.1093/rheumatology/keag028
Jean-Pascal Grenier,Alexander Thiel
OBJECTIVESDespite growing evidence supporting the benefits of physical activity in patients with rheumatic and musculoskeletal diseases (RMDs), both clinicians and patients often remain cautious-particularly regarding higher intensities. Although exercise is universally recommended in international guidelines for these populations, it is frequently accompanied by warnings to engage only in moderate-intensity training. The primary objective of this systematic review was to examine the effectiveness and safety of high-intensity, land-based exercise in patients with inflammatory rheumatic conditions, compared with usual care, no intervention, or low-intensity exercise. We aimed to determine whether high-intensity exercise leads to an increase in disease activity, pain intensity, or impairment of function.METHODSWe conducted a systematic review through a systematic literature search in the electronic databases MEDLINE (via PubMed), the Cochrane Library, PEDro, and ClinicalTrials.gov.RESULTSWe identified 28 studies (15 original randomized controlled trials and 13 secondary analyses or follow-up studies). Study quality assessed using the JBI checklist was generally adequate, with main limitations in patient and clinician blinding. High-intensity exercise-whether aerobic, resistance-based, or in the form of high-intensity interval training (HIIT)-is safe for patients with inflammatory rheumatic diseases. We found moderate certainty evidence that high-intensity exercise does not increase disease activity, and may reduce pain and may improve function when compared with control interventions.CONCLUSIONWhile patients can certainly benefit from low- to moderate-intensity exercise, current evidence does not suggest harm from high-intensity exercise. Exercise selection should follow an individualized, person-centered approach.
{"title":"Effectiveness and safety of high-intensity exercise in rheumatic diseases friend or foe? a systematic review.","authors":"Jean-Pascal Grenier,Alexander Thiel","doi":"10.1093/rheumatology/keag028","DOIUrl":"https://doi.org/10.1093/rheumatology/keag028","url":null,"abstract":"OBJECTIVESDespite growing evidence supporting the benefits of physical activity in patients with rheumatic and musculoskeletal diseases (RMDs), both clinicians and patients often remain cautious-particularly regarding higher intensities. Although exercise is universally recommended in international guidelines for these populations, it is frequently accompanied by warnings to engage only in moderate-intensity training. The primary objective of this systematic review was to examine the effectiveness and safety of high-intensity, land-based exercise in patients with inflammatory rheumatic conditions, compared with usual care, no intervention, or low-intensity exercise. We aimed to determine whether high-intensity exercise leads to an increase in disease activity, pain intensity, or impairment of function.METHODSWe conducted a systematic review through a systematic literature search in the electronic databases MEDLINE (via PubMed), the Cochrane Library, PEDro, and ClinicalTrials.gov.RESULTSWe identified 28 studies (15 original randomized controlled trials and 13 secondary analyses or follow-up studies). Study quality assessed using the JBI checklist was generally adequate, with main limitations in patient and clinician blinding. High-intensity exercise-whether aerobic, resistance-based, or in the form of high-intensity interval training (HIIT)-is safe for patients with inflammatory rheumatic diseases. We found moderate certainty evidence that high-intensity exercise does not increase disease activity, and may reduce pain and may improve function when compared with control interventions.CONCLUSIONWhile patients can certainly benefit from low- to moderate-intensity exercise, current evidence does not suggest harm from high-intensity exercise. Exercise selection should follow an individualized, person-centered approach.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"52 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146015213","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}
Pub Date : 2026-01-20DOI: 10.1093/rheumatology/keag015
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 M D Smith
OBJECTIVESTo conduct data-driven sensitivity analyses to evaluate whether refined definitions of childhood-onset systemic lupus erythematosus (cSLE) treat-to-target goals provide better protection against moderate-severe flares and new damage, compared with original consensus-derived targets.METHODSThe UK JSLE Cohort Study was utilised. Childhood-SLE target attainment was determined at each visit. Removal or transformation of cSLE target criteria ("variations") were investigated, for Childhood Lupus Low Disease Activity State (cLLDAS), cSLE Clinical Remission on Steroids (cCR) and cSLE Clinical Remission off Steroids (cCR-0). The impact of such variations on the hazards of subsequent moderate-severe flare and new damage was assessed, using Prentice-Williams-Peterson (PWP) models. Two-sided t-tests compared the hazard ratios (HRs) obtained from the PWP gap-time models for the original and varied cSLE target definitions.RESULTSTwo variations of cLLDAS demonstrated significantly better protection against moderate-severe flare, including transformation of SLEDAI-2K cut-off to ≤ 3 (HR 0.13 [0.09, 0.19], p < 0.001); and transformation of PGA cut-off to ≤ 0.25 (HR 0.14 [0.10, 0.20], p < 0.001). These variations in cLLDAS did not impact on the hazards of new damage. No variations of cCR and cCR-0 led to a significant improvement in hazards of moderate-severe flare/new damage (all p > 0.05). A modified version of cLLDAS, combining these two transformations was also assessed, demonstrating further improvement in protection against moderate-severe flare (HR 0.12 [0.08, 0.17], p < 0.001).CONCLUSIONSRefining the cLLDAS definition by lowering the SLEDAI-2K cut-off to ≤ 3 and PGA to ≤ 0.25 may enhance protection against moderate-severe flare, but not new damage. No variations of cCR or cCR-0 showed significant improvement.
目的进行数据驱动的敏感性分析，以评估儿童期发病系统性红斑狼疮（cSLE）治疗目标的改进定义是否比原始共识衍生的目标提供更好的保护，以防止中重度耀斑和新损伤。方法采用英国JSLE队列研究。在每次访问时确定儿童sle目标实现情况。研究了儿童狼疮低疾病活动状态（cLLDAS）、cSLE类固醇临床缓解（cCR）和cSLE类固醇临床缓解（cCR-0）的cSLE目标标准的去除或转化（“变异”）。使用Prentice-Williams-Peterson （PWP）模型评估这些变化对随后中重度耀斑和新损害的危害的影响。双侧t检验比较了原始和不同cSLE目标定义的PWP间隙时间模型获得的风险比（hr）。结果cLLDAS的两种变异对中重度耀斑的保护作用显著增强，SLEDAI-2K截止值变为≤3 （HR 0.13 [0.09, 0.19], p 0.05）。对cLLDAS的改进版本，结合这两种转化也进行了评估，显示出对中重度耀斑的保护进一步改善（HR 0.12 [0.08, 0.17], p < 0.001）。结论通过将SLEDAI-2K截止值降至≤3和PGA降至≤0.25来完善cLLDAS的定义可以增强对中重度耀斑的保护，但不会产生新的损伤。cCR和cCR-0无明显改善。
{"title":"Validating treat-to-target endpoints in childhood lupus: data-driven sensitivity analyses from the UK JSLE cohort study.","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 M D Smith","doi":"10.1093/rheumatology/keag015","DOIUrl":"https://doi.org/10.1093/rheumatology/keag015","url":null,"abstract":"OBJECTIVESTo conduct data-driven sensitivity analyses to evaluate whether refined definitions of childhood-onset systemic lupus erythematosus (cSLE) treat-to-target goals provide better protection against moderate-severe flares and new damage, compared with original consensus-derived targets.METHODSThe UK JSLE Cohort Study was utilised. Childhood-SLE target attainment was determined at each visit. Removal or transformation of cSLE target criteria (\"variations\") were investigated, for Childhood Lupus Low Disease Activity State (cLLDAS), cSLE Clinical Remission on Steroids (cCR) and cSLE Clinical Remission off Steroids (cCR-0). The impact of such variations on the hazards of subsequent moderate-severe flare and new damage was assessed, using Prentice-Williams-Peterson (PWP) models. Two-sided t-tests compared the hazard ratios (HRs) obtained from the PWP gap-time models for the original and varied cSLE target definitions.RESULTSTwo variations of cLLDAS demonstrated significantly better protection against moderate-severe flare, including transformation of SLEDAI-2K cut-off to ≤ 3 (HR 0.13 [0.09, 0.19], p < 0.001); and transformation of PGA cut-off to ≤ 0.25 (HR 0.14 [0.10, 0.20], p < 0.001). These variations in cLLDAS did not impact on the hazards of new damage. No variations of cCR and cCR-0 led to a significant improvement in hazards of moderate-severe flare/new damage (all p > 0.05). A modified version of cLLDAS, combining these two transformations was also assessed, demonstrating further improvement in protection against moderate-severe flare (HR 0.12 [0.08, 0.17], p < 0.001).CONCLUSIONSRefining the cLLDAS definition by lowering the SLEDAI-2K cut-off to ≤ 3 and PGA to ≤ 0.25 may enhance protection against moderate-severe flare, but not new damage. No variations of cCR or cCR-0 showed significant improvement.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"63 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146005469","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}
Pub Date : 2026-01-18DOI: 10.1093/rheumatology/keag025
Ioannis Arkoumanis,Maria Pappa,Kyriaki Keramiotou,Petros P Sfikakis,Maria G Tektonidou
OBJECTIVEPatients with Systemic Lupus Erythematosus (SLE) experience poor health-related quality of life (HRQoL) that is associated with disease characteristics, comorbidities and reduced physical activity. We aimed to investigate the relationship between upper limb function and HRQoL in patients with SLE.METHODS240 patients with SLE and 122 age-/sex-matched healthy controls were assessed for upper limb function using grip and pinch strength, as well as the Purdue pegboard test for dexterity. Disability in activities of daily living (ADLs) was assessed using the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire. HRQoL was evaluated with the LupusQoL questionnaire. Associations of upper limb function and DASH scores with all LupusQoL domains were examined using linear regression analyses.RESULTSPatients with SLE demonstrated significant impairment in grip strength, pinch strength and dexterity compared with healthy controls (all p< 0.05). In univariate analysis, lower grip strength, pinch strength and Purdue pegboard scores, as well as higher DASH scores, were significantly associated with diminished LupusQoL across all domains (all p< 0.05). In multivariate models, all upper limb function parameters were independently associated with all LupusQoL domains (p< 0.05) after adjustments for disease activity, disease damage, medications, fibromyalgia and visual analogue score, except the Body image domain in relation to the Purdue test. DASH scores were inversely related to all LupusQoL domain scores (all p< 0.001).CONCLUSIONImpaired upper limb function and ADL performance are independently associated with poor LupusQoL across all domains in SLE, highlighting the importance of incorporating upper limb function assessment and management into clinical practice.
{"title":"Upper limb function impairment is independently associated with all Lupus Quality of Life (LupusQoL) domains in systemic lupus erythematosus.","authors":"Ioannis Arkoumanis,Maria Pappa,Kyriaki Keramiotou,Petros P Sfikakis,Maria G Tektonidou","doi":"10.1093/rheumatology/keag025","DOIUrl":"https://doi.org/10.1093/rheumatology/keag025","url":null,"abstract":"OBJECTIVEPatients with Systemic Lupus Erythematosus (SLE) experience poor health-related quality of life (HRQoL) that is associated with disease characteristics, comorbidities and reduced physical activity. We aimed to investigate the relationship between upper limb function and HRQoL in patients with SLE.METHODS240 patients with SLE and 122 age-/sex-matched healthy controls were assessed for upper limb function using grip and pinch strength, as well as the Purdue pegboard test for dexterity. Disability in activities of daily living (ADLs) was assessed using the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire. HRQoL was evaluated with the LupusQoL questionnaire. Associations of upper limb function and DASH scores with all LupusQoL domains were examined using linear regression analyses.RESULTSPatients with SLE demonstrated significant impairment in grip strength, pinch strength and dexterity compared with healthy controls (all p< 0.05). In univariate analysis, lower grip strength, pinch strength and Purdue pegboard scores, as well as higher DASH scores, were significantly associated with diminished LupusQoL across all domains (all p< 0.05). In multivariate models, all upper limb function parameters were independently associated with all LupusQoL domains (p< 0.05) after adjustments for disease activity, disease damage, medications, fibromyalgia and visual analogue score, except the Body image domain in relation to the Purdue test. DASH scores were inversely related to all LupusQoL domain scores (all p< 0.001).CONCLUSIONImpaired upper limb function and ADL performance are independently associated with poor LupusQoL across all domains in SLE, highlighting the importance of incorporating upper limb function assessment and management into clinical practice.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"22 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145994975","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}
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