{"title":"两个地区的故事:比较儿科麻醉相关关节炎的临床特征和结果。","authors":"Fatma Gül Demirkan, Vafa Guliyeva, Özlem Akgün, Hanene Lassoued Ferjani, Dorra Ben Nessib, Kawther Maatallah, Dhia Kaffel, Wafa Hamdi, Nuray Aktay Ayaz","doi":"10.1186/s12969-025-01095-2","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Enthesitis-related arthritis (ERA) may exhibit a distinct disease spectrum on the basis of ethnic origin. The pediatric rheumatology teams from the Istanbul Medical Faculty and Tunisia Kassab Institute engaged in collaboration via the Second Sister Hospital Initiative of the European Society of Pediatric Rheumatology (PReS) to investigate the clinical characteristics and outcomes of children with ERA.</p><p><strong>Methods: </strong>The medical records of patients with the diagnosis of ERA were reviewed retrospectively. The Juvenile Spondyloarthritis Disease Activity Index (JSpADA) was the tool for assessing disease activity. In addition to clinical and laboratory findings, treatments and disease outcomes were compared.</p><p><strong>Results: </strong>A total of 94 children with ERA were enrolled (45 Tunisian, 49 Turkish). Sex and age at disease onset were similar between the groups. Heel pain (8.8% vs. 61.2% for Tunisia vs. Türkiye, p = 0.03) and enthesitis (40% vs. 69.3% for Tunisia vs. Türkiye, p = 0.03, p = 0.8) were more common in Turkish children. Conversely, the rates of sacroiliac tenderness, suggesting clinical sacroiliitis (91.1% vs. 55.1% for Tunisia vs. Türkiye), and axial disease (97.8% vs. 55.1% for Tunisia vs. Türkiye) were significantly greater in Tunisian children (p = 0.002 and p < 0.001, respectively). Overall, 45.7% of the cohort was HLA-B27 positive, including 32% of Turkish patients and 60% of Tunisian patients (p < 0.001). HLA-B27 positivity did not influence age at disease onset (p = 0.45) but was associated with a longer diagnostic delay of the disease (p < 0.001). Nearly half of the Turkish children received biologics during the disease course, whereas only 8.9% of the Tunisian children did. While the median JSpADA scores at disease onset were similar between the groups, Turkish patients had significantly lower scores at the last visit than Tunisian patients did (p < 0.001).</p><p><strong>Conclusions: </strong>This study highlights notable differences in the clinical features and outcomes of ERA among Turkish and Tunisian children, emphasizing the potential influence of ethnic and regional factors on disease presentation and management. Variations in HLA-B27 positivity and treatment approaches, including the use of biologics, further underscore the need for tailored strategies in managing ERA across diverse populations.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"82"},"PeriodicalIF":2.3000,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12309154/pdf/","citationCount":"0","resultStr":"{\"title\":\"A tale of two regions: comparing clinical features and outcomes in pediatric enthesitis-related arthritis.\",\"authors\":\"Fatma Gül Demirkan, Vafa Guliyeva, Özlem Akgün, Hanene Lassoued Ferjani, Dorra Ben Nessib, Kawther Maatallah, Dhia Kaffel, Wafa Hamdi, Nuray Aktay Ayaz\",\"doi\":\"10.1186/s12969-025-01095-2\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Enthesitis-related arthritis (ERA) may exhibit a distinct disease spectrum on the basis of ethnic origin. The pediatric rheumatology teams from the Istanbul Medical Faculty and Tunisia Kassab Institute engaged in collaboration via the Second Sister Hospital Initiative of the European Society of Pediatric Rheumatology (PReS) to investigate the clinical characteristics and outcomes of children with ERA.</p><p><strong>Methods: </strong>The medical records of patients with the diagnosis of ERA were reviewed retrospectively. The Juvenile Spondyloarthritis Disease Activity Index (JSpADA) was the tool for assessing disease activity. In addition to clinical and laboratory findings, treatments and disease outcomes were compared.</p><p><strong>Results: </strong>A total of 94 children with ERA were enrolled (45 Tunisian, 49 Turkish). Sex and age at disease onset were similar between the groups. Heel pain (8.8% vs. 61.2% for Tunisia vs. Türkiye, p = 0.03) and enthesitis (40% vs. 69.3% for Tunisia vs. Türkiye, p = 0.03, p = 0.8) were more common in Turkish children. Conversely, the rates of sacroiliac tenderness, suggesting clinical sacroiliitis (91.1% vs. 55.1% for Tunisia vs. Türkiye), and axial disease (97.8% vs. 55.1% for Tunisia vs. Türkiye) were significantly greater in Tunisian children (p = 0.002 and p < 0.001, respectively). Overall, 45.7% of the cohort was HLA-B27 positive, including 32% of Turkish patients and 60% of Tunisian patients (p < 0.001). HLA-B27 positivity did not influence age at disease onset (p = 0.45) but was associated with a longer diagnostic delay of the disease (p < 0.001). Nearly half of the Turkish children received biologics during the disease course, whereas only 8.9% of the Tunisian children did. While the median JSpADA scores at disease onset were similar between the groups, Turkish patients had significantly lower scores at the last visit than Tunisian patients did (p < 0.001).</p><p><strong>Conclusions: </strong>This study highlights notable differences in the clinical features and outcomes of ERA among Turkish and Tunisian children, emphasizing the potential influence of ethnic and regional factors on disease presentation and management. Variations in HLA-B27 positivity and treatment approaches, including the use of biologics, further underscore the need for tailored strategies in managing ERA across diverse populations.</p>\",\"PeriodicalId\":54630,\"journal\":{\"name\":\"Pediatric Rheumatology\",\"volume\":\"23 1\",\"pages\":\"82\"},\"PeriodicalIF\":2.3000,\"publicationDate\":\"2025-07-29\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12309154/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Pediatric Rheumatology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1186/s12969-025-01095-2\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"PEDIATRICS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric Rheumatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1186/s12969-025-01095-2","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"PEDIATRICS","Score":null,"Total":0}
引用次数: 0
摘要
背景:膝炎相关关节炎(ERA)可能表现出不同的疾病谱系,基于种族起源。伊斯坦布尔医学院和突尼斯Kassab研究所的儿科风湿病学团队通过欧洲儿科风湿病学会(PReS)的第二姐妹医院倡议开展合作,调查ERA患儿的临床特征和预后。方法:回顾性分析诊断为ERA的患者的病历资料。青少年脊椎关节炎疾病活动性指数(JSpADA)是评估疾病活动性的工具。除了临床和实验室结果外,还比较了治疗和疾病结果。结果:共纳入94例ERA患儿(突尼斯45例,土耳其49例)。两组患者的性别和发病年龄相似。土耳其儿童的足跟疼痛(突尼斯与 rkiye分别为8.8%和61.2%,p = 0.03)和结肠炎(突尼斯与 rkiye分别为40%和69.3%,p = 0.03, p = 0.8)更为常见。相反,突尼斯儿童的骶髂压痛率,提示临床骶髂炎(91.1% vs. 55.1%)和轴性疾病(97.8% vs. 55.1%)在突尼斯儿童中明显更高(p = 0.002和p)。结论:本研究强调了土耳其和突尼斯儿童中ERA的临床特征和结局的显著差异,强调了种族和地区因素对疾病表现和管理的潜在影响。HLA-B27阳性和治疗方法的差异,包括生物制剂的使用,进一步强调了在不同人群中管理ERA的量身定制策略的必要性。
A tale of two regions: comparing clinical features and outcomes in pediatric enthesitis-related arthritis.
Background: Enthesitis-related arthritis (ERA) may exhibit a distinct disease spectrum on the basis of ethnic origin. The pediatric rheumatology teams from the Istanbul Medical Faculty and Tunisia Kassab Institute engaged in collaboration via the Second Sister Hospital Initiative of the European Society of Pediatric Rheumatology (PReS) to investigate the clinical characteristics and outcomes of children with ERA.
Methods: The medical records of patients with the diagnosis of ERA were reviewed retrospectively. The Juvenile Spondyloarthritis Disease Activity Index (JSpADA) was the tool for assessing disease activity. In addition to clinical and laboratory findings, treatments and disease outcomes were compared.
Results: A total of 94 children with ERA were enrolled (45 Tunisian, 49 Turkish). Sex and age at disease onset were similar between the groups. Heel pain (8.8% vs. 61.2% for Tunisia vs. Türkiye, p = 0.03) and enthesitis (40% vs. 69.3% for Tunisia vs. Türkiye, p = 0.03, p = 0.8) were more common in Turkish children. Conversely, the rates of sacroiliac tenderness, suggesting clinical sacroiliitis (91.1% vs. 55.1% for Tunisia vs. Türkiye), and axial disease (97.8% vs. 55.1% for Tunisia vs. Türkiye) were significantly greater in Tunisian children (p = 0.002 and p < 0.001, respectively). Overall, 45.7% of the cohort was HLA-B27 positive, including 32% of Turkish patients and 60% of Tunisian patients (p < 0.001). HLA-B27 positivity did not influence age at disease onset (p = 0.45) but was associated with a longer diagnostic delay of the disease (p < 0.001). Nearly half of the Turkish children received biologics during the disease course, whereas only 8.9% of the Tunisian children did. While the median JSpADA scores at disease onset were similar between the groups, Turkish patients had significantly lower scores at the last visit than Tunisian patients did (p < 0.001).
Conclusions: This study highlights notable differences in the clinical features and outcomes of ERA among Turkish and Tunisian children, emphasizing the potential influence of ethnic and regional factors on disease presentation and management. Variations in HLA-B27 positivity and treatment approaches, including the use of biologics, further underscore the need for tailored strategies in managing ERA across diverse populations.
期刊介绍:
Pediatric Rheumatology is an open access, peer-reviewed, online journal encompassing all aspects of clinical and basic research related to pediatric rheumatology and allied subjects.
The journal’s scope of diseases and syndromes include musculoskeletal pain syndromes, rheumatic fever and post-streptococcal syndromes, juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, local and systemic scleroderma, Kawasaki disease, Henoch-Schonlein purpura and other vasculitides, sarcoidosis, inherited musculoskeletal syndromes, autoinflammatory syndromes, and others.