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A tale of two regions: comparing clinical features and outcomes in pediatric enthesitis-related arthritis
Fatma Gül Demirkan1, Vafa Guliyeva1, Özlem Akgün1
1Department of Pediatric Rheumatology, İstanbul School of Medicine, İstanbul University, Fatih, İstanbul, Türkiye.
Insights
Enthesitis-related arthritis (ERA) presents differently in Turkish and Tunisian children, with distinct symptoms and outcomes. Ethnic and regional factors significantly influence ERA presentation and management strategies.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Genetics
Background:
- Enthesitis-related arthritis (ERA) may present with varying clinical characteristics based on ethnic origin.
- A collaborative study was initiated between Turkish and Tunisian pediatric rheumatology teams to investigate ERA in children.
- The European Society of Pediatric Rheumatology (PReS) supported this research through its Second Sister Hospital Initiative.
Purpose of the Study:
- To investigate and compare the clinical characteristics and disease outcomes of children diagnosed with ERA in Turkey and Tunisia.
- To identify potential ethnic and regional influences on the presentation and progression of ERA.
- To analyze differences in human leukocyte antigen B27 (HLA-B27) positivity and treatment approaches between the two patient cohorts.
Main Methods:
- Retrospective review of medical records for patients diagnosed with ERA.
- Assessment of disease activity using the Juvenile Spondyloarthritis Disease Activity Index (JSpADA).
- Comparison of clinical findings, laboratory results, treatment modalities, and disease outcomes between Tunisian and Turkish children.
Main Results:
- Turkish children showed higher rates of heel pain and enthesitis, while Tunisian children had more sacroiliac tenderness and axial disease.
- HLA-B27 positivity was significantly higher in Tunisian children (60%) compared to Turkish children (32%), but was associated with a longer diagnostic delay.
- Turkish patients received biologics more frequently and had significantly lower JSpADA scores at the last visit compared to Tunisian patients.
Conclusions:
- Significant differences exist in ERA clinical features and outcomes between Turkish and Tunisian pediatric populations.
- Ethnic and regional factors appear to play a crucial role in shaping ERA presentation and disease course.
- Tailored management strategies are essential for ERA, considering variations in HLA-B27 positivity and treatment responses across diverse ethnic groups.
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.
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