to investigate the impact of cutaneous, vascular, and musculoskeletal (MSK) involvements on hand function-related patient-reported outcomes (PROs) by assessing clinical, radiographic, and ultrasonographic (US) features in a real-world cohort of Systemic Sclerosis (SSc) patients.
Methods
patients were enrolled in a multicentre cross-sectional study. PROs included Cochin Hand Function Scale (CHFS). Medical history and clinical examination, hands and wrists’ ultrasonography and radiography were collected. Analogue data were obtained from an external validation cohort. Separate and pooled cohorts’ analyses were conducted.
Results
140 consecutive patients were enrolled from 3 centres. In both univariate and multivariate analyses, CHFS scores showed a significant correlation with modified Rodnan Skin Score (mRSS) (p<0.001) and with US-detected tenosynovitis (p<0.01). A trend was observed for the presence of US-detected tenosynovitis with a sclerosing pattern and for the presence of calcinosis, with statistically significant correlations in univariate analysis (p=0.01 and 0.02, respectively) but not in multivariate analysis (p=0.06 and 0.055, respectively).
Conclusion
our findings support the multifactorial origin of hand function impairment in SSc patients, with mostly contributions from cutaneous and MSK involvement. Skin fibrosis and US-detected tenosynovitis were the most significant variables explaining hand disability as measured by CHFS. Ultrasonography may represent a valuable complementary tool, assessing MSK disease severity and activity.
What was already known
cutaneous, vascular and musculoskeletal involvement contribute to hand function impairment in SSc patients
What was learned from this study
● Skin fibrosis and US-detected tenosynovitis are the most relevant features. ● Ultrasonography may provide complementary information in the routine care of SSc patients.
{"title":"Hand function impairment in Systemic Sclerosis: determinants through investigations of clinical, radiographic and ultrasonographic features","authors":"Giulia Franchi , Elena Marazzi , Alain Lescoat , Sophie Hecquet , Sandrine Carvès , Guillaume Coiffier , Marine Tas , Veronica Codullo , Carlomaurizio Montecucco , Jérôme Avouac , Yannick Allanore","doi":"10.1016/j.semarthrit.2025.152901","DOIUrl":"10.1016/j.semarthrit.2025.152901","url":null,"abstract":"<div><h3>Objectives</h3><div>to investigate the impact of cutaneous, vascular, and musculoskeletal (MSK) involvements on hand function-related patient-reported outcomes (PROs) by assessing clinical, radiographic, and ultrasonographic (US) features in a real-world cohort of Systemic Sclerosis (SSc) patients.</div></div><div><h3>Methods</h3><div>patients were enrolled in a multicentre cross-sectional study. PROs included Cochin Hand Function Scale (CHFS). Medical history and clinical examination, hands and wrists’ ultrasonography and radiography were collected. Analogue data were obtained from an external validation cohort. Separate and pooled cohorts’ analyses were conducted.</div></div><div><h3>Results</h3><div>140 consecutive patients were enrolled from 3 centres. In both univariate and multivariate analyses, CHFS scores showed a significant correlation with modified Rodnan Skin Score (mRSS) (<em>p</em> <em><</em> <em>0.001</em>) and with US-detected tenosynovitis (<em>p</em> <em><</em> <em>0.01</em>). A trend was observed for the presence of US-detected tenosynovitis with a sclerosing pattern and for the presence of calcinosis, with statistically significant correlations in univariate analysis (<em>p</em> <em>=</em> <em>0.01 and 0.02, respectively</em>) but not in multivariate analysis <em>(p</em> <em>=</em> <em>0.06 and 0.055, respectively)</em>.</div></div><div><h3>Conclusion</h3><div>our findings support the multifactorial origin of hand function impairment in SSc patients, with mostly contributions from cutaneous and MSK involvement. Skin fibrosis and US-detected tenosynovitis were the most significant variables explaining hand disability as measured by CHFS. Ultrasonography may represent a valuable complementary tool, assessing MSK disease severity and activity.</div></div><div><h3>What was already known</h3><div>cutaneous, vascular and musculoskeletal involvement contribute to hand function impairment in SSc patients</div></div><div><h3>What was learned from this study</h3><div>● Skin fibrosis and US-detected tenosynovitis are the most relevant features. ● Ultrasonography may provide complementary information in the routine care of SSc patients.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"76 ","pages":"Article 152901"},"PeriodicalIF":4.4,"publicationDate":"2025-12-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145841199","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 : 2025-12-17DOI: 10.1016/j.semarthrit.2025.152898
Athena Chin , Lekshmi Minikumari Rahulan , Devansh Lalwani , Ryan Moore , Jessica A Day , Samuel Katsuyuki Shinjo , Tamaraudubamo Agunbiade , Aman Kumar , Tsz Ho Luk , Yan Ki Tang , Marco Fornaro , Anamika Anuja , Ho So , Vidya Sadanand Limaye , Vikas Agarwal , Latika Gupta
Introduction and Aims
Cardiovascular disease (CVD) is a leading contributor of morbidity and mortality in patients with idiopathic inflammatory myopathies (IIM). The British Society for Rheumatology (BSR) 2022 guidelines recommend that IIM patients should undergo a regular cardiovascular risk assessment; however, many tools underestimate scores in IIM due to their sole focus on traditional risk factors. Cardiovascular Risk Score (QRISK) incorporates risk factors that are more relevant in IIM, such as corticosteroid use. The National Institute for Health and Care Excellence (NICE) guidelines recommend offering statin therapy for the primary prevention of CVD in patients with an estimated 10-year CVD risk of ≥10 %. This study aimed to undertake an audit of cardiovascular risk assessment in a multinational cohort of patients with IIM, based on BSR and NICE recommendations.
Methods
A multi-centre, international retrospective chart review was conducted in IIM cohorts from centres in India, Hong Kong, Brazil, Italy, Australia, and the United Kingdom, of patients who attended clinics between 2020 and 2023. Data were collected from medical records to evaluate CVD risk using both WHO cardiovascular risk charts and QRISK3 score. Based on their risk of cardiovascular events in the next 10 years, patients were defined as high-risk (>20 %), moderate-risk (10–20 %), or low-risk (<10 %). Adherence to the BSR and NICE guidelines was evaluated.
Results
A total of 336 patients were included. All centres, except one, did not routinely assess CVD risk. More than a third of patients were deemed moderate-high risk for CVD as per QRISK3, although only 34 % in this group were prescribed a statin as per NICE guidelines. Disease subtype immune-mediated necrotizing myopathy was strongly associated with moderate-high risk scores (OR = 4.64; 95 %CI = 1.88–11.45, p < 0.001), as was active steroid use [OR = 1.76 (95 % CI = 1.06–2.92), p = 0.03] and dyslipidaemia [OR = 1.22 (95 % CI = 1.03–1.45), p = 0.02]. There was a moderate level of agreement between WHO and QRISK3 scores.
Conclusion
Over a third of patients with IIM are at moderate-high-risk of cardiovascular events in 10 years. Steroid use and dyslipidaemia were modifiable risk factors that were statistically significant predictors of risk. Despite this, there is a lack of adherence to current guidelines advising regular CVD risk assessment and use of statins for primary prevention in this moderate-high risk group.
简介和目的：心血管疾病（CVD）是特发性炎症性肌病（IIM）患者发病率和死亡率的主要原因。英国风湿病学会（BSR） 2022指南建议IIM患者应定期进行心血管风险评估；然而，许多工具低估了IIM的得分，因为它们只关注传统的风险因素。心血管风险评分（QRISK）纳入了与IIM更相关的风险因素，如皮质类固醇的使用。国家健康与护理卓越研究所（NICE）指南建议，对于估计10年心血管疾病风险≥10%的患者，他汀类药物治疗可用于心血管疾病的一级预防。本研究旨在根据BSR和NICE的建议，对IIM患者的跨国队列进行心血管风险评估审计。方法：对2020年至2023年间就诊的印度、香港、巴西、意大利、澳大利亚和英国中心的IIM队列进行了多中心、国际回顾性图表回顾。从医疗记录中收集数据，使用WHO心血管风险图表和QRISK3评分评估CVD风险。根据患者在未来10年内发生心血管事件的风险，将患者分为高危（10- 20%）、中危（10- 20%）和低危(结果：共纳入336例患者。除一家中心外，所有中心均未常规评估心血管疾病风险。根据QRISK3，超过三分之一的患者被认为是心血管疾病的中高风险，尽管根据NICE指南，该组中只有34%的患者服用了他汀类药物。疾病亚型免疫介导的坏死性肌病与中-高风险评分（OR = 4.64; 95% CI = 1.88-11.45, p < 0.001）、活跃类固醇使用[OR = 1.76 (95% CI = 1.06-2.92), p = 0.03]和血脂异常[OR = 1.22 (95% CI = 1.03-1.45), p = 0.02]密切相关。世卫组织和QRISK3评分之间存在中等程度的一致性。结论：超过三分之一的IIM患者在10年内处于心血管事件的中高危状态。类固醇使用和血脂异常是可改变的危险因素，是有统计学意义的危险预测因子。尽管如此，目前的指南建议定期进行心血管疾病风险评估，并建议在这一中高风险人群中使用他汀类药物进行一级预防，但缺乏对指南的遵守。
{"title":"Cardiovascular risk assessment in idiopathic inflammatory myopathies: a multicentre international study by the myositis audit and research collaborative group","authors":"Athena Chin , Lekshmi Minikumari Rahulan , Devansh Lalwani , Ryan Moore , Jessica A Day , Samuel Katsuyuki Shinjo , Tamaraudubamo Agunbiade , Aman Kumar , Tsz Ho Luk , Yan Ki Tang , Marco Fornaro , Anamika Anuja , Ho So , Vidya Sadanand Limaye , Vikas Agarwal , Latika Gupta","doi":"10.1016/j.semarthrit.2025.152898","DOIUrl":"10.1016/j.semarthrit.2025.152898","url":null,"abstract":"<div><h3>Introduction and Aims</h3><div>Cardiovascular disease (CVD) is a leading contributor of morbidity and mortality in patients with idiopathic inflammatory myopathies (IIM). The British Society for Rheumatology (BSR) 2022 guidelines recommend that IIM patients should undergo a regular cardiovascular risk assessment; however, many tools underestimate scores in IIM due to their sole focus on traditional risk factors. Cardiovascular Risk Score (QRISK) incorporates risk factors that are more relevant in IIM, such as corticosteroid use. The National Institute for Health and Care Excellence (NICE) guidelines recommend offering statin therapy for the primary prevention of CVD in patients with an estimated 10-year CVD risk of ≥10 %. This study aimed to undertake an audit of cardiovascular risk assessment in a multinational cohort of patients with IIM, based on BSR and NICE recommendations.</div></div><div><h3>Methods</h3><div>A multi-centre, international retrospective chart review was conducted in IIM cohorts from centres in India, Hong Kong, Brazil, Italy, Australia, and the United Kingdom, of patients who attended clinics between 2020 and 2023. Data were collected from medical records to evaluate CVD risk using both WHO cardiovascular risk charts and QRISK3 score. Based on their risk of cardiovascular events in the next 10 years, patients were defined as high-risk (>20 %), moderate-risk (10–20 %), or low-risk (<10 %). Adherence to the BSR and NICE guidelines was evaluated.</div></div><div><h3>Results</h3><div>A total of 336 patients were included. All centres, except one, did not routinely assess CVD risk. More than a third of patients were deemed moderate-high risk for CVD as per QRISK3, although only 34 % in this group were prescribed a statin as per NICE guidelines. Disease subtype immune-mediated necrotizing myopathy was strongly associated with moderate-high risk scores (OR = 4.64; 95 %CI = 1.88–11.45, <em>p</em> < 0.001), as was active steroid use [OR = 1.76 (95 % CI = 1.06–2.92), <em>p</em> = 0.03] and dyslipidaemia [OR = 1.22 (95 % CI = 1.03–1.45), <em>p</em> = 0.02]. There was a moderate level of agreement between WHO and QRISK3 scores.</div></div><div><h3>Conclusion</h3><div>Over a third of patients with IIM are at moderate-high-risk of cardiovascular events in 10 years. Steroid use and dyslipidaemia were modifiable risk factors that were statistically significant predictors of risk. Despite this, there is a lack of adherence to current guidelines advising regular CVD risk assessment and use of statins for primary prevention in this moderate-high risk group.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"76 ","pages":"Article 152898"},"PeriodicalIF":4.4,"publicationDate":"2025-12-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145865392","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}
Systemic lupus erythematosus (SLE) still lacks highly specific biomarkers; high-throughput metabolomics offers a route to elucidate disease-defining metabolic perturbations.
Objective
This systematic review aims to identify common metabolite changes related to SLE.
Methods
PubMed, Web of Science, Scopus, and the Cochrane Library were searched through November 2024 for human observational studies comparing the metabolomic profiles of adult SLE patients with those of healthy controls. Random-effects meta-analyses used the ratio of means (RoM); heterogeneity was assessed using the I² statistic.
Results
Forty-six studies comprising 2,238 SLE patients and 1,761 healthy controls (total n = 3,999) were included. Ten metabolites, each reported in ≥2 of the five eligible studies, were quantitatively synthesized. Compared with controls, isoleucine (RoM = 0.73, 95 % CI = 0.72–0.74, I² = 0 %), leucine (RoM = 0.81, 95 % CI = 0.80–0.81, I² = 0 %), and tryptophan (RoM = 0.73, 95 % CI = 0.64–0.84, I² = 75 %) were significantly lower in SLE, whereas methionine was significantly higher (RoM = 1.54, 95 % CI = 1.26–1.88, I² = 88 %). Lipid remodeling included elevated oleic acid (RoM = 1.42, 95 % CI = 1.19–1.69, I² = 0 %) and reduced capric acid (RoM = 0.80, 95 % CI = 0.67–0.95, I² = 31 %). Qualitative synthesis revealed consistent reduction of tricarboxylic acid intermediates, accumulation of acylcarnitines, and an oxidized-lipid signature (e.g., 9-hydroxyoctadecadienoic acid, leukotriene B4), implying mitochondrial stress and redox imbalance.
Conclusions
Metabolomic profiling identifies a reproducible SLE signature: relative to controls, branched-chain (isoleucine, leucine) and aromatic (tryptophan) amino acids are lower, methionine is higher, and lipid profiles show higher oleic and lower capric acids, patterns consistent with impaired mitochondrial energetics and altered one-carbon flux.
背景：系统性红斑狼疮（SLE）仍然缺乏高度特异性的生物标志物；高通量代谢组学为阐明疾病定义代谢扰动提供了一条途径。目的：本系统综述旨在确定与SLE相关的常见代谢物变化。方法：到2024年11月，PubMed、Web of Science、Scopus和Cochrane图书馆检索了成人SLE患者与健康对照者的代谢组学特征比较的人类观察性研究。随机效应荟萃分析使用均值比（RoM）；采用I²统计量评估异质性。结果：纳入46项研究，包括2238例SLE患者和1761例健康对照（总n = 3999）。定量合成了10种代谢物，每种代谢物均在5项符合条件的研究中≥2项报道。与对照组相比，异亮氨酸（RoM = 0.73, 95% CI = 0.72-0.74, I²= 0%）、亮氨酸（RoM = 0.81, 95% CI = 0.80-0.81, I²= 0%）和色氨酸（RoM = 0.73, 95% CI = 0.64-0.84, I²= 75%）在SLE中显著降低，而蛋氨酸显著升高（RoM = 1.54, 95% CI = 1.26-1.88, I²= 88%）。脂质重塑包括油酸升高（RoM = 1.42, 95% CI = 1.19-1.69, I²= 0%）和癸酸降低（RoM = 0.80, 95% CI = 0.67-0.95, I²= 31%）。定性合成显示三羧酸中间体一致减少，酰基肉碱积累，氧化脂质特征（如9-羟基十八烯二烯酸，白三烯B4），暗示线粒体应激和氧化还原失衡。结论：代谢组学分析确定了可重复的SLE特征：相对于对照组，支链（异亮氨酸、亮氨酸）和芳香（色氨酸）氨基酸含量较低，蛋氨酸含量较高，脂质谱显示出较高的油酸和较低的己酸，与线粒体能量受损和单碳通量改变的模式一致。
{"title":"Metabolomics in systemic lupus erythematosus: A systematic review and meta-analysis","authors":"Susana Barrera-Hernández , Claudia Mendoza-Pinto , Pamela Munguía-Realpozo , Ivet Etchegaray-Morales , Blanca Guadalupe Baez-Duarte , Irma Zamora-Ginez , Edith Ramírez-Lara , Álvaro José Montiel-Jarquin , Máximo Alejandro García-Flores , Socorro Méndez-Martínez","doi":"10.1016/j.semarthrit.2025.152893","DOIUrl":"10.1016/j.semarthrit.2025.152893","url":null,"abstract":"<div><h3>Background</h3><div>Systemic lupus erythematosus (SLE) still lacks highly specific biomarkers; high-throughput metabolomics offers a route to elucidate disease-defining metabolic perturbations.</div></div><div><h3>Objective</h3><div>This systematic review aims to identify common metabolite changes related to SLE.</div></div><div><h3>Methods</h3><div>PubMed, Web of Science, Scopus, and the Cochrane Library were searched through November 2024 for human observational studies comparing the metabolomic profiles of adult SLE patients with those of healthy controls. Random-effects meta-analyses used the ratio of means (RoM); heterogeneity was assessed using the I² statistic.</div></div><div><h3>Results</h3><div>Forty-six studies comprising 2,238 SLE patients and 1,761 healthy controls (total n = 3,999) were included. Ten metabolites, each reported in ≥2 of the five eligible studies, were quantitatively synthesized. Compared with controls, isoleucine (RoM = 0.73, 95 % CI = 0.72–0.74, I² = 0 %), leucine (RoM = 0.81, 95 % CI = 0.80–0.81, I² = 0 %), and tryptophan (RoM = 0.73, 95 % CI = 0.64–0.84, I² = 75 %) were significantly lower in SLE, whereas methionine was significantly higher (RoM = 1.54, 95 % CI = 1.26–1.88, I² = 88 %). Lipid remodeling included elevated oleic acid (RoM = 1.42, 95 % CI = 1.19–1.69, I² = 0 %) and reduced capric acid (RoM = 0.80, 95 % CI = 0.67–0.95, I² = 31 %). Qualitative synthesis revealed consistent reduction of tricarboxylic acid intermediates, accumulation of acylcarnitines, and an oxidized-lipid signature (e.g., 9-hydroxyoctadecadienoic acid, leukotriene B4), implying mitochondrial stress and redox imbalance.</div></div><div><h3>Conclusions</h3><div>Metabolomic profiling identifies a reproducible SLE signature: relative to controls, branched-chain (isoleucine, leucine) and aromatic (tryptophan) amino acids are lower, methionine is higher, and lipid profiles show higher oleic and lower capric acids, patterns consistent with impaired mitochondrial energetics and altered one-carbon flux.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"76 ","pages":"Article 152893"},"PeriodicalIF":4.4,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145820675","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 : 2025-12-13DOI: 10.1016/j.semarthrit.2025.152896
Bingxin Ma , Yan Kan , Jie Lv , Jianyu Sun , Juan Kang , Xinyi Dong , Junwei Ma , Shixiang Chen , Jin Yang , Yue Zhao , Qi Lu
Aims
To identify the factors associated with frailty in Chinese participants with rheumatoid arthritis (RA), guided by the health ecology theory, and to provide a culturally adapted and psychometrically validated Chinese version of the Comprehensive Rheumatologic Assessment of Frailty (CRAF) to support context-specific assessments in clinical practice.
Methods
This cross-sectional study involved the translation and cultural adaptation of the CRAF using a modified Brislin method. Psychometric properties were evaluated, and factors associated with frailty were identified through multivariate ordered logistic regression, guided by the health ecology theory.
Results
A total of 1288 RA participants were recruited. The Cronbach’s alpha for the Chinese CRAF was 0.75, and the intraclass correlation coefficient was 0.92. The Item-content validity index (CVI) ranged from 0.80 to 1.00, with an average CVI of 0.92. A significant positive correlation between the CRAF and the FRAIL Scale was found (P < 0.001), with the CRAF demonstrating better discriminatory power than the FRAIL scale (P = 0.041). Factors significantly associated with frailty included disease duration, pain intensity, limitations in physical function, the use of methotrexate, non-steroidal anti-inflammatory drugs, glucocorticoids, and biologic disease-modifying antirheumatic drugs, as well as comorbidities such as hypertension, diabetes, cardiovascular disease, stroke/transient ischemic attack, pulmonary disease, diminished grip strength, smoking, depression, and lack of social participation.
Conclusion
The Chinese version of the CRAF is a reliable and valid tool for assessing frailty in RA participants. Targeted interventions addressing the identified risk factors may help prevent or delay frailty progression, ultimately improving prognosis and quality of life.
{"title":"Exploring factors contributing to frailty in Chinese patients with rheumatoid arthritis: Validation of a tailored assessment tool","authors":"Bingxin Ma , Yan Kan , Jie Lv , Jianyu Sun , Juan Kang , Xinyi Dong , Junwei Ma , Shixiang Chen , Jin Yang , Yue Zhao , Qi Lu","doi":"10.1016/j.semarthrit.2025.152896","DOIUrl":"10.1016/j.semarthrit.2025.152896","url":null,"abstract":"<div><h3>Aims</h3><div>To identify the factors associated with frailty in Chinese participants with rheumatoid arthritis (RA), guided by the health ecology theory, and to provide a culturally adapted and psychometrically validated Chinese version of the Comprehensive Rheumatologic Assessment of Frailty (CRAF) to support context-specific assessments in clinical practice.</div></div><div><h3>Methods</h3><div>This cross-sectional study involved the translation and cultural adaptation of the CRAF using a modified Brislin method. Psychometric properties were evaluated, and factors associated with frailty were identified through multivariate ordered logistic regression, guided by the health ecology theory.</div></div><div><h3>Results</h3><div>A total of 1288 RA participants were recruited. The Cronbach’s alpha for the Chinese CRAF was 0.75, and the intraclass correlation coefficient was 0.92. The Item-content validity index (CVI) ranged from 0.80 to 1.00, with an average CVI of 0.92. A significant positive correlation between the CRAF and the FRAIL Scale was found (<em>P</em> < 0.001), with the CRAF demonstrating better discriminatory power than the FRAIL scale (<em>P</em> = 0.041). Factors significantly associated with frailty included disease duration, pain intensity, limitations in physical function, the use of methotrexate, non-steroidal anti-inflammatory drugs, glucocorticoids, and biologic disease-modifying antirheumatic drugs, as well as comorbidities such as hypertension, diabetes, cardiovascular disease, stroke/transient ischemic attack, pulmonary disease, diminished grip strength, smoking, depression, and lack of social participation.</div></div><div><h3>Conclusion</h3><div>The Chinese version of the CRAF is a reliable and valid tool for assessing frailty in RA participants. Targeted interventions addressing the identified risk factors may help prevent or delay frailty progression, ultimately improving prognosis and quality of life.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"76 ","pages":"Article 152896"},"PeriodicalIF":4.4,"publicationDate":"2025-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145799065","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}
Although musculoskeletal symptoms are common in patients with inflammatory bowel disease (IBD), spondyloarthritis (SpA) remains underdiagnosed. This systematic review aimed at identifying and comparing self-administered screening questionnaires for SpA in IBD patients.
Methods
We conducted a systematic review according to PRISMA guidelines using PubMed, Cochrane, and ACR/EULAR congress databases, with the last search on April 21, 2024. Studies focusing on self-administered screening questionnaires for SpA in IBD patients were included. We extracted questionnaire characteristics and their psychometric data. The content validity of each questionnaire was assessed using the COSMIN method.
Results
A total of 1491 articles were screened. Eight were included and six screening questionnaires were identified. Two questionnaires assessed axial, one peripheral and three both axial and peripheral symptoms of SpA. The IBIS-Q questionnaire showed the highest sensitivity (92.7 %), while the Queiro’s axial questionnaire had the highest specificity (89.8 %). The DETAIL questionnaire gave the highest post-test probability to have a SpA with a positive likelihood ratio of at least 14, using a threshold of three affirmative responses. None of these questionnaires met all criteria of the COSMIN methodology to evaluate their quality. The content validity of the questionnaires was judged to be sufficient or inconsistent by reviewers with moderate qualities of evidence at best.
Conclusions
Six self-administered questionnaires have been developed to screen for SpA in IBD patients. Limited methodological transparency hinders direct comparison. Although none of the available questionnaires fully meets validation standards, the DETAIL questionnaire appears to offer the greatest clinical potential, despite only moderate-quality evidence.
虽然肌肉骨骼症状在炎症性肠病（IBD）患者中很常见，但脊椎关节炎（SpA）仍未得到充分诊断。本系统综述旨在确定和比较IBD患者自我管理的SpA筛查问卷。方法根据PRISMA指南，使用PubMed、Cochrane和ACR/ ular congress数据库进行系统评价，最后一次检索时间为2024年4月21日。研究集中在IBD患者自我管理的SpA筛查问卷。我们提取了问卷特征及其心理测量数据。采用COSMIN方法评估各问卷的内容效度。结果共筛选文献1491篇。共纳入8份，确定了6份筛选问卷。两份问卷评估SpA的轴向症状、一份外周症状和三份轴向和外周症状。IBIS-Q问卷灵敏度最高（92.7%），Queiro轴向问卷特异性最高（89.8%）。DETAIL问卷给出了测试后SpA的最高概率，其阳性似然比至少为14，使用三个肯定回答的阈值。这些问卷都不符合COSMIN方法评估其质量的所有标准。调查问卷的内容效度由具有中等证据质量的审稿人判断为充分或不一致。结论已编制了6份自填问卷，用于筛查IBD患者的SpA。有限的方法透明度妨碍了直接比较。尽管现有的问卷都不完全符合验证标准，DETAIL问卷似乎提供了最大的临床潜力，尽管只有中等质量的证据。
{"title":"Self-administered screening questionnaires for spondyloarthritis in inflammatory bowel disease: A systematic review","authors":"Romain Schotkosky , Tiphaine Dujardin , Marianne Hupé , Athan Baillet , Xavier Romand","doi":"10.1016/j.semarthrit.2025.152890","DOIUrl":"10.1016/j.semarthrit.2025.152890","url":null,"abstract":"<div><h3>Objective</h3><div>Although musculoskeletal symptoms are common in patients with inflammatory bowel disease (IBD), spondyloarthritis (SpA) remains underdiagnosed. This systematic review aimed at identifying and comparing self-administered screening questionnaires for SpA in IBD patients.</div></div><div><h3>Methods</h3><div>We conducted a systematic review according to PRISMA guidelines using PubMed, Cochrane, and ACR/EULAR congress databases, with the last search on April 21, 2024. Studies focusing on self-administered screening questionnaires for SpA in IBD patients were included. We extracted questionnaire characteristics and their psychometric data. The content validity of each questionnaire was assessed using the COSMIN method.</div></div><div><h3>Results</h3><div>A total of 1491 articles were screened. Eight were included and six screening questionnaires were identified. Two questionnaires assessed axial, one peripheral and three both axial and peripheral symptoms of SpA. The IBIS-Q questionnaire showed the highest sensitivity (92.7 %), while the Queiro’s axial questionnaire had the highest specificity (89.8 %). The DETAIL questionnaire gave the highest post-test probability to have a SpA with a positive likelihood ratio of at least 14, using a threshold of three affirmative responses. None of these questionnaires met all criteria of the COSMIN methodology to evaluate their quality. The content validity of the questionnaires was judged to be sufficient or inconsistent by reviewers with moderate qualities of evidence at best.</div></div><div><h3>Conclusions</h3><div>Six self-administered questionnaires have been developed to screen for SpA in IBD patients. Limited methodological transparency hinders direct comparison. Although none of the available questionnaires fully meets validation standards, the DETAIL questionnaire appears to offer the greatest clinical potential, despite only moderate-quality evidence.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"76 ","pages":"Article 152890"},"PeriodicalIF":4.4,"publicationDate":"2025-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145799056","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}
To identify associated and protective factors of rapid spinal radiographic progression in axial spondyloarthritis (axSpA) using artificial intelligence (AI).
Methods
We conducted a hospital-based retrospective cohort study involving 242 axSpA patients taken ≥2 lateral spine radiographs between 2002 and 2024. Spinal damage was assessed with modified Stoke Ankylosing Spondylitis Spine Score (mSASSS) by a deep learning model. Each pair of consecutive radiographs defined an observational interval (total 379 intervals); annual mSASSS progression rate was calculated for each interval. Demographics, clinical features, baseline mSASSS, activity indices, cumulative dosage of prescriptions, and laboratory recordings were collected. Time-dependent generalized estimating equations (GEE) were applied to identify associated or protective factors of rapid spinal radiographic progression (ΔmSASSS/year >1), accounting for within-patient correlation.
Results
For recorded intervals, mean mSASSS progression was 0.5/year; 26.7% of intervals showed progression >1/year. For enrolled patients, mean mSASSS progression was 0.6/year; 27.3% of intervals showed progression >1/year. Conditional multivariable GEE analysis revealed age at baseline mSASSS, especially ≥40 years, was independently associated with rapid mSASSS progression [adjusted odds ratio (aOR), 1.03; 95% confidence interval (CI), 1.003–1.06]. Higher cumulative dosage of non-steroidal anti-inflammatory drugs (NSAIDs) during the intervals was negatively associated with rapid mSASSS progression (aOR, 0.38; 95% CI, 0.19–0.75). Cumulative dosage of tumor necrosis factor inhibitors and secukinumab during the intervals was independent of rapid mSASSS progression.
Conclusions
Using AI-assisted mSASSS scoring, this retrospective cohort study identified older age at assessment as an associated factor and full-dose NSAIDs use as protective factor for rapid spinal radiographic progression.
{"title":"Factors associated with rapid spinal radiographic progression in patients with axial spondyloarthritis: A hospital-based retrospective cohort study with mSASSS scoring using deep learning model","authors":"Chung-Mao Kao , Yi-Hsing Chen , Wen-Nan Huang , Tsu-Yi Hsieh , Chia-Wei Hsieh , Kuo-Lung Lai , Ching-Tsai Lin , Yi-Ming Chen , Wei-Ting Hung , Yin-Yi Chou , Kuo-Tung Tang , Chih-Wei Tseng , Yi-Da Wu , Yen-Ju Chen , Yu-Wan Liao , Yun-Wen Chen , Tsai-Hung Yen , Heh-Shiang Sheu , Hsin-Hua Chen","doi":"10.1016/j.semarthrit.2025.152888","DOIUrl":"10.1016/j.semarthrit.2025.152888","url":null,"abstract":"<div><h3>Objective</h3><div>To identify associated and protective factors of rapid spinal radiographic progression in axial spondyloarthritis (axSpA) using artificial intelligence (AI).</div></div><div><h3>Methods</h3><div>We conducted a hospital-based retrospective cohort study involving 242 axSpA patients taken ≥2 lateral spine radiographs between 2002 and 2024. Spinal damage was assessed with modified Stoke Ankylosing Spondylitis Spine Score (mSASSS) by a deep learning model. Each pair of consecutive radiographs defined an observational interval (total 379 intervals); annual mSASSS progression rate was calculated for each interval. Demographics, clinical features, baseline mSASSS, activity indices, cumulative dosage of prescriptions, and laboratory recordings were collected. Time-dependent generalized estimating equations (GEE) were applied to identify associated or protective factors of rapid spinal radiographic progression (ΔmSASSS/year >1), accounting for within-patient correlation.</div></div><div><h3>Results</h3><div>For recorded intervals, mean mSASSS progression was 0.5/year; 26.7% of intervals showed progression >1/year. For enrolled patients, mean mSASSS progression was 0.6/year; 27.3% of intervals showed progression >1/year. Conditional multivariable GEE analysis revealed age at baseline mSASSS, especially ≥40 years, was independently associated with rapid mSASSS progression [adjusted odds ratio (aOR), 1.03; 95% confidence interval (CI), 1.003–1.06]. Higher cumulative dosage of non-steroidal anti-inflammatory drugs (NSAIDs) during the intervals was negatively associated with rapid mSASSS progression (aOR, 0.38; 95% CI, 0.19–0.75). Cumulative dosage of tumor necrosis factor inhibitors and secukinumab during the intervals was independent of rapid mSASSS progression.</div></div><div><h3>Conclusions</h3><div>Using AI-assisted mSASSS scoring, this retrospective cohort study identified older age at assessment as an associated factor and full-dose NSAIDs use as protective factor for rapid spinal radiographic progression.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"76 ","pages":"Article 152888"},"PeriodicalIF":4.4,"publicationDate":"2025-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145795064","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 : 2025-12-11DOI: 10.1016/j.semarthrit.2025.152891
Koji Suzuki, Mitsuhiro Akiyama, Kanako Shimanuki, Hiroyuki Fukui, Yuko Kaneko
Objective
To clarify the real-world effectiveness of nintedanib continuation beyond 12 months on pulmonary function in progressive pulmonary fibrosis (PPF) associated with systemic autoimmune rheumatic disease-related interstitial lung disease (SARD-ILD).
Methods
We conducted a review of all consecutive SARD-ILD patients from the KEIO-SARD-ILD cohort who received nintedanib for PPF between 2015 and 2025. The primary outcome was the 12-month change in percent predicted forced vital capacity (%FVC) between patients who continued nintedanib for 12 months and those who discontinued treatment earlier. Secondary outcomes included 12-month changes in Krebs von den Lungen-6 (KL-6) levels and mortality between the two groups.
Results
Among the 65 patients, systemic sclerosis was the most common underlying condition (n = 16), followed by idiopathic inflammatory myopathies (n = 15), and rheumatoid arthritis (n = 13). The overall 12-month retention rate of nintedanib was 69.2 %. Both the continuation and discontinuation groups exhibited a comparable decline in %FVC in 12 months prior to nintedanib initiation (-3.6 % vs. -3.8 %, p = 0.58), but after 12 months of treatment, the continuation group demonstrated a significantly greater improvement in %FVC (1.8 % vs. -3.5 %, p = 0.01) and KL-6 (-175 vs 71 U/mL, p = 0.04) compared to the discontinuation group. The ILD-related survival rates were better in the continuation group compared to the discontinuation group (p = 0.02).
Conclusions
Continuation of nintedanib beyond 12 months, compared with discontinuation within 12 months, is associated with significant improvements in pulmonary function, biomarkers, and survival in patients with SARD-ILD-related PPF, suggesting that long-term nintedanib therapy plays a critical role in stabilizing disease progression.
目的：明确尼达尼布持续治疗12个月以上对进行性肺纤维化（PPF）伴系统性自身免疫性风湿性疾病相关间质性肺疾病（SARD-ILD）患者肺功能的实际有效性。方法：我们对2015年至2025年间接受尼达尼布治疗PPF的KEIO-SARD-ILD队列中所有连续的SARD-ILD患者进行了回顾。主要结局是持续尼达尼布12个月的患者和早期停止治疗的患者之间12个月预测用力肺活量（%FVC）百分比的变化。次要结局包括两组间12个月Krebs von den Lungen-6 （KL-6）水平和死亡率的变化。结果在65例患者中，系统性硬化症是最常见的基础疾病（n = 16），其次是特发性炎症性肌病（n = 15）和类风湿性关节炎（n = 13）。尼达尼布12个月的总体保留率为69.2%。在尼达尼布开始治疗前的12个月内，继续组和停药组的%FVC都有相当的下降（- 3.6%对- 3.8%,p = 0.58），但在治疗12个月后，与停药组相比，继续组的%FVC（1.8%对- 3.5%,p = 0.01）和KL-6（-175对71 U/mL， p = 0.04）有更大的改善。与停药组相比，继续治疗组的ild相关生存率更高（p = 0.02）。结论：与停药12个月相比，持续使用尼达尼布12个月以上与sard - ild相关PPF患者肺功能、生物标志物和生存率的显著改善相关，表明长期使用尼达尼布治疗在稳定疾病进展中起关键作用。
{"title":"Benefits of nintedanib continuation in systemic autoimmune rheumatic disease-related progressive pulmonary fibrosis: KEIO-SARD-ILD-cohort","authors":"Koji Suzuki, Mitsuhiro Akiyama, Kanako Shimanuki, Hiroyuki Fukui, Yuko Kaneko","doi":"10.1016/j.semarthrit.2025.152891","DOIUrl":"10.1016/j.semarthrit.2025.152891","url":null,"abstract":"<div><h3>Objective</h3><div>To clarify the real-world effectiveness of nintedanib continuation beyond 12 months on pulmonary function in progressive pulmonary fibrosis (PPF) associated with systemic autoimmune rheumatic disease-related interstitial lung disease (SARD-ILD).</div></div><div><h3>Methods</h3><div>We conducted a review of all consecutive SARD-ILD patients from the KEIO-SARD-ILD cohort who received nintedanib for PPF between 2015 and 2025. The primary outcome was the 12-month change in percent predicted forced vital capacity (%FVC) between patients who continued nintedanib for 12 months and those who discontinued treatment earlier. Secondary outcomes included 12-month changes in Krebs von den Lungen-6 (KL-6) levels and mortality between the two groups.</div></div><div><h3>Results</h3><div>Among the 65 patients, systemic sclerosis was the most common underlying condition (<em>n</em> = 16), followed by idiopathic inflammatory myopathies (<em>n</em> = 15), and rheumatoid arthritis (<em>n</em> = 13). The overall 12-month retention rate of nintedanib was 69.2 %. Both the continuation and discontinuation groups exhibited a comparable decline in %FVC in 12 months prior to nintedanib initiation (-3.6 % vs. -3.8 %, <em>p</em> = 0.58), but after 12 months of treatment, the continuation group demonstrated a significantly greater improvement in %FVC (1.8 % vs. -3.5 %, <em>p</em> = 0.01) and KL-6 (-175 vs 71 U/mL, <em>p</em> = 0.04) compared to the discontinuation group. The ILD-related survival rates were better in the continuation group compared to the discontinuation group (<em>p</em> = 0.02).</div></div><div><h3>Conclusions</h3><div>Continuation of nintedanib beyond 12 months, compared with discontinuation within 12 months, is associated with significant improvements in pulmonary function, biomarkers, and survival in patients with SARD-ILD-related PPF, suggesting that long-term nintedanib therapy plays a critical role in stabilizing disease progression.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"76 ","pages":"Article 152891"},"PeriodicalIF":4.4,"publicationDate":"2025-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145750220","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 : 2025-12-11DOI: 10.1016/j.semarthrit.2025.152894
S.E. (Sabine) Kloprogge, J.J. (Jacoline) van den Driest, L. (Laura) Struik, S.M.A. (Sita) Bierma-Zeinstra, M. (Marienke) van Middelkoop
Objective
Osteoarthritis (OA) of the first metatarsophalangeal (MTP) joint is accompanied by pain and stiffness and associated with reduced health-related quality of life. Although prevalence of radiographic 1st MTP joint OA is high, the incidence of clinical 1st MTP joint OA is unknown. Therefore, we aimed to determine the incidence and management of general practice (GP) consultations for symptomatic 1st MTP joint OA.
Methods
A retrospective cohort study was conducted using electronic health records of GPs. An algorithm was defined to identify 1st MTP joint OA patients based on free text and codified data between 2013 and 2022. First MTP joint OA incidence rate, comorbidities and management strategies were assessed.
Results
The overall 1st MTP joint OA incidence was 0.74/1000 person-years in patients ≥35 years. The most initiated management by GPs was explanation/reassurance (360/672 (53.6 %)), followed by referral to podiatry (171/672 (25.4 %)) and orthopedic surgeon consultation (162/672 (24.1 %)). Of the 823 patients consulting their GP with foot/toe problems in the year before diagnosis, 491 (47.1 %) were referred to radiology, and 271 (26 %) for orthopedic surgeon consultation.
Conclusion
The incidence of 1st MTP joint OA has been estimated for the first time in general practice. Most patients are diagnosed after referral to radiology or orthopedic surgeon consultation. From diagnosis, half of 1st MTP joint OA patients are referred, mostly for orthopedic surgeon consultation and podiatry. As evidence for these diagnostic and management strategies is lacking, research into their effectiveness for 1st MTP joint OA in general practice is needed.
{"title":"Incidence and management of first metatarsophalangeal joint osteoarthritis in Dutch general practice estimates from the Rijnmond Primary Care Database","authors":"S.E. (Sabine) Kloprogge, J.J. (Jacoline) van den Driest, L. (Laura) Struik, S.M.A. (Sita) Bierma-Zeinstra, M. (Marienke) van Middelkoop","doi":"10.1016/j.semarthrit.2025.152894","DOIUrl":"10.1016/j.semarthrit.2025.152894","url":null,"abstract":"<div><h3>Objective</h3><div>Osteoarthritis (OA) of the first metatarsophalangeal (MTP) joint is accompanied by pain and stiffness and associated with reduced health-related quality of life. Although prevalence of radiographic 1st MTP joint OA is high, the incidence of clinical 1st MTP joint OA is unknown. Therefore, we aimed to determine the incidence and management of general practice (GP) consultations for symptomatic 1st MTP joint OA.</div></div><div><h3>Methods</h3><div>A retrospective cohort study was conducted using electronic health records of GPs. An algorithm was defined to identify 1st MTP joint OA patients based on free text and codified data between 2013 and 2022. First MTP joint OA incidence rate, comorbidities and management strategies were assessed.</div></div><div><h3>Results</h3><div>The overall 1st MTP joint OA incidence was 0.74/1000 person-years in patients ≥35 years. The most initiated management by GPs was explanation/reassurance (360/672 (53.6 %)), followed by referral to podiatry (171/672 (25.4 %)) and orthopedic surgeon consultation (162/672 (24.1 %)). Of the 823 patients consulting their GP with foot/toe problems in the year before diagnosis, 491 (47.1 %) were referred to radiology, and 271 (26 %) for orthopedic surgeon consultation.</div></div><div><h3>Conclusion</h3><div>The incidence of 1st MTP joint OA has been estimated for the first time in general practice. Most patients are diagnosed after referral to radiology or orthopedic surgeon consultation. From diagnosis, half of 1st MTP joint OA patients are referred, mostly for orthopedic surgeon consultation and podiatry. As evidence for these diagnostic and management strategies is lacking, research into their effectiveness for 1st MTP joint OA in general practice is needed.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"76 ","pages":"Article 152894"},"PeriodicalIF":4.4,"publicationDate":"2025-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145782587","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 : 2025-12-11DOI: 10.1016/j.semarthrit.2025.152889
Kevin Chevalier , Brigitte Bader-Meunier , Isabelle Kone-Paut , Benjamin Thoreau , Marc Michel , Bertrand Godeau , Christian Agard , Thomas Papo , Karim Sacre , Raphaèle Seror , Xavier Mariette , Patrice Cacoub , Ygal Benhamou , Mathilde Leclercq , Cécile Goujard , Olivier Lambotte , Bernard Bonnotte , Maxime Samson , Félix Ackermann , Jean Schmidt , Benjamin Chaigne
Objectives
Juvenile-onset mixed connective tissue disease (jMCTD) accounts for 7–23 % of MCTD cases but remains poorly described. We aimed to characterize clinical features, treatments, and outcomes of patients with jMCTD, and compare them to adult-onset MCTD (aMCTD) patients.
Methods
We conducted a multicenter, retrospective, case-control study within the French MCTD cohort. Each jMCTD patient was compared to 3 matched aMCTD patients.
Results
Forty-seven jMCTD patients (93.6 % girls; median age at onset 14 [11–16] years) were included. Forty-four (93.6 %) jMCTD patients fulfilled either Sharp or Kasukawa diagnostic criteria. None of them met other diagnostic criteria without fulfilling Sharp or Kasukawa criteria. At diagnosis, jMCTD patients’ main manifestations were Raynaud’s phenomenon, arthralgia, and myalgia. jMCTD patients had less frequently puffy fingers than aMCTD (p < 0.0001). Cumulatively, jMCTD patients mainly received glucocorticoids (80.9 %), hydroxychloroquine (95.7 %) and immunosuppressants (93.6 %). They received a higher initial dose of glucocorticoids (30 [20–60] mg/day vs. 15 [10–35] mg/day, p = 0.02), and significantly more frequently methotrexate (Methotrexate) and rituximab (p = 0.01) over time compared to aMCTD. After a median follow-up of 9.8 [6.6–16.2] years, 29 (61.7 %) jMCTD patients were in remission (vs. 62 (44.0 %) aMCTD; p < 0.05), 36 % had progressed to another CTD (vs. 30.5 % aMCTD; p = 0.5), mainly systemic lupus erythematosus, 11 (23.4 %) had developed interstitial lung disease, 2 (4.3 %) pulmonary arterial hypertension, and 1 (2.1 %) died.
Conclusions
jMCTD share the same clinical characteristics as aMCTD patients, but less frequently have puffy fingers. Outcomes appear more favorable in jMCTD than aMCTD, with higher remission rates, albeit at the cost of more intensive treatment.
{"title":"Juvenile-onset mixed connective tissue disease: A multicenter retrospective cohort study","authors":"Kevin Chevalier , Brigitte Bader-Meunier , Isabelle Kone-Paut , Benjamin Thoreau , Marc Michel , Bertrand Godeau , Christian Agard , Thomas Papo , Karim Sacre , Raphaèle Seror , Xavier Mariette , Patrice Cacoub , Ygal Benhamou , Mathilde Leclercq , Cécile Goujard , Olivier Lambotte , Bernard Bonnotte , Maxime Samson , Félix Ackermann , Jean Schmidt , Benjamin Chaigne","doi":"10.1016/j.semarthrit.2025.152889","DOIUrl":"10.1016/j.semarthrit.2025.152889","url":null,"abstract":"<div><h3>Objectives</h3><div>Juvenile-onset mixed connective tissue disease (jMCTD) accounts for 7–23 % of MCTD cases but remains poorly described. We aimed to characterize clinical features, treatments, and outcomes of patients with jMCTD, and compare them to adult-onset MCTD (aMCTD) patients.</div></div><div><h3>Methods</h3><div>We conducted a multicenter, retrospective, case-control study within the French MCTD cohort. Each jMCTD patient was compared to 3 matched aMCTD patients.</div></div><div><h3>Results</h3><div>Forty-seven jMCTD patients (93.6 % girls; median age at onset 14 [11–16] years) were included. Forty-four (93.6 %) jMCTD patients fulfilled either Sharp or Kasukawa diagnostic criteria. None of them met other diagnostic criteria without fulfilling Sharp or Kasukawa criteria. At diagnosis, jMCTD patients’ main manifestations were Raynaud’s phenomenon, arthralgia, and myalgia. jMCTD patients had less frequently puffy fingers than aMCTD (<em>p</em> < 0.0001). Cumulatively, jMCTD patients mainly received glucocorticoids (80.9 %), hydroxychloroquine (95.7 %) and immunosuppressants (93.6 %). They received a higher initial dose of glucocorticoids (30 [20–60] mg/day <em>vs.</em> 15 [10–35] mg/day, <em>p</em> = 0.02), and significantly more frequently methotrexate (Methotrexate) and rituximab (<em>p</em> = 0.01) over time compared to aMCTD. After a median follow-up of 9.8 [6.6–16.2] years, 29 (61.7 %) jMCTD patients were in remission (<em>vs.</em> 62 (44.0 %) aMCTD; <em>p</em> < 0.05), 36 % had progressed to another CTD (<em>vs</em>. 30.5 % aMCTD; <em>p</em> = 0.5), mainly systemic lupus erythematosus, 11 (23.4 %) had developed interstitial lung disease, 2 (4.3 %) pulmonary arterial hypertension, and 1 (2.1 %) died.</div></div><div><h3>Conclusions</h3><div>jMCTD share the same clinical characteristics as aMCTD patients, but less frequently have puffy fingers. Outcomes appear more favorable in jMCTD than aMCTD, with higher remission rates, albeit at the cost of more intensive treatment.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"76 ","pages":"Article 152889"},"PeriodicalIF":4.4,"publicationDate":"2025-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145782627","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 : 2025-12-11DOI: 10.1016/j.semarthrit.2025.152895
Katja Thiele , Katinka Albrecht , Carlo Veltri , Kirsten Karberg , Benjamin Köhler , Johanna Callhoff , Jutta G. Richter
Objective
To explore the development of fatigue over the past 17 years and its relationship to physician- and patient-reported outcomes and social factors in inflammatory rheumatic diseases.
Methodology
Data from ≈9300 patients per year from the German National Database (2007–2023) were included, considering arthritides, spondyloarthritides, connective tissue diseases and vasculitides. Fatigue was assessed on a numeric rating scale (0–10) with >2 defined as present and >6 as severe. Presence and severity were compared by diagnosis, gender and year. Fatigue clusters were identified based on trajectory patterns over three consecutive visits.
Results
Fatigue affected 55 % (adult onset Still disease) to 67 % (systemic sclerosis) of patients, with severe fatigue in up to 26 % (systemic sclerosis). Substantial proportions of women (47–61 %) and men (35–52 %) experienced moderate-to-severe fatigue. Despite marked improvements in inflammation-responsive outcomes (CRP -40 %, tender joints -50 %, physician disease activity -42 %) and employment (52 %→70 %), mean fatigue remained stable. Trajectory analysis identified 35 % with persistent low, 23 % persistent high, 24 % worsening, and 19 % improving fatigue. Tender joints and morning stiffness effectively discriminated between persistent high versus low fatigue clusters. Emotional well-being, physical functioning, coping, and sleep quality showed stronger associations with fatigue trajectories than inflammatory markers. Differences across fatigue clusters substantially exceeded those between diagnostic groups.
Conclusion
Fatigue affected a large proportion of both women and men across diagnoses. Fatigue trajectories reflect complex interplay of clinical and psychosocial factors. Management should incorporate multidimensional interventions addressing emotional well-being, physical function and social support beyond traditional inflammatory control.
{"title":"Trends in fatigue in inflammatory rheumatic diseases: Annual data and trajectory analysis of the German National Database 2007-2023","authors":"Katja Thiele , Katinka Albrecht , Carlo Veltri , Kirsten Karberg , Benjamin Köhler , Johanna Callhoff , Jutta G. Richter","doi":"10.1016/j.semarthrit.2025.152895","DOIUrl":"10.1016/j.semarthrit.2025.152895","url":null,"abstract":"<div><h3>Objective</h3><div>To explore the development of fatigue over the past 17 years and its relationship to physician- and patient-reported outcomes and social factors in inflammatory rheumatic diseases.</div></div><div><h3>Methodology</h3><div>Data from ≈9300 patients per year from the German National Database (2007–2023) were included, considering arthritides, spondyloarthritides, connective tissue diseases and vasculitides. Fatigue was assessed on a numeric rating scale (0–10) with >2 defined as present and >6 as severe. Presence and severity were compared by diagnosis, gender and year. Fatigue clusters were identified based on trajectory patterns over three consecutive visits.</div></div><div><h3>Results</h3><div>Fatigue affected 55 % (adult onset Still disease) to 67 % (systemic sclerosis) of patients, with severe fatigue in up to 26 % (systemic sclerosis). Substantial proportions of women (47–61 %) and men (35–52 %) experienced moderate-to-severe fatigue. Despite marked improvements in inflammation-responsive outcomes (CRP -40 %, tender joints -50 %, physician disease activity -42 %) and employment (52 %→70 %), mean fatigue remained stable. Trajectory analysis identified 35 % with persistent low, 23 % persistent high, 24 % worsening, and 19 % improving fatigue. Tender joints and morning stiffness effectively discriminated between persistent high versus low fatigue clusters. Emotional well-being, physical functioning, coping, and sleep quality showed stronger associations with fatigue trajectories than inflammatory markers. Differences across fatigue clusters substantially exceeded those between diagnostic groups.</div></div><div><h3>Conclusion</h3><div>Fatigue affected a large proportion of both women and men across diagnoses. Fatigue trajectories reflect complex interplay of clinical and psychosocial factors. Management should incorporate multidimensional interventions addressing emotional well-being, physical function and social support beyond traditional inflammatory control.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"76 ","pages":"Article 152895"},"PeriodicalIF":4.4,"publicationDate":"2025-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145782662","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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