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两个地区的故事:比较儿童附着点炎相关关节炎的临床特征和结局

A tale of two regions: comparing clinical features and outcomes in pediatric enthesitis-related arthritis.

作者信息

Demirkan Fatma Gül, Guliyeva Vafa, Akgün Özlem, Ferjani Hanene Lassoued, Nessib Dorra Ben, Maatallah Kawther, Kaffel Dhia, Hamdi Wafa, Aktay Ayaz Nuray

机构信息

Department of Pediatric Rheumatology, İstanbul School of Medicine, İstanbul University, Fatih, İstanbul, Türkiye.

Department of Rheumatology, Kassab Orthopedics Institute, Ksar Saïd, Manouba, Tunisia.

出版信息

Pediatr Rheumatol Online J. 2025 Jul 29;23(1):82. doi: 10.1186/s12969-025-01095-2.

Abstract

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.

摘要

背景

附着点炎相关关节炎(ERA)可能因种族不同而呈现出不同的疾病谱。伊斯坦布尔医学院和突尼斯卡萨布研究所的儿科风湿病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,以调查ERA患儿的临床特征和预后。

方法

对诊断为ERA的患者病历进行回顾性分析。青少年脊柱关节炎疾病活动指数(JSpADA)是评估疾病活动的工具。除了临床和实验室检查结果外,还比较了治疗方法和疾病预后。

结果

共纳入94例ERA患儿(45例突尼斯患儿,49例土耳其患儿)。两组患儿的性别和发病年龄相似。足跟痛(突尼斯患儿为8.8%,土耳其患儿为61.2%,p = 0.03)和附着点炎(突尼斯患儿为40%,土耳其患儿为69.3%,p = 0.03,p = 0.8)在土耳其患儿中更为常见。相反,提示临床骶髂关节炎的骶髂关节压痛率(突尼斯患儿为91.1%,土耳其患儿为55.1%)和轴向疾病率(突尼斯患儿为97.8%,土耳其患儿为55.1%)在突尼斯患儿中显著更高(分别为p = 0.002和p < 0.001)。总体而言,该队列中45.7%的患儿HLA - B27呈阳性,其中土耳其患者为32%,突尼斯患者为60%(p < 0.001)。HLA - B27阳性不影响发病年龄(p = 0.45),但与疾病诊断延迟时间较长有关(p < 0.001)。近一半的土耳其患儿在病程中接受了生物制剂治疗,而突尼斯患儿只有8.9%接受了生物制剂治疗。虽然两组患儿发病时的JSpADA中位数评分相似,但土耳其患者在最后一次就诊时的评分显著低于突尼斯患者(p < 0.001)。

结论

本研究突出了土耳其和突尼斯患儿ERA临床特征和预后的显著差异,强调了种族和地区因素对疾病表现及管理的潜在影响。HLA - B27阳性率和治疗方法(包括生物制剂的使用)的差异进一步强调了针对不同人群管理ERA需要制定个性化策略。

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