Zehra Kızıldağ

Background/Aims: To compare the clinical characteristics of enthesitis/ spondylitis-related juvenile idiopathic arthritis (E/SpRJIA), a relatively prevalent JIA subtype in Türkiye and India, and to explore potential populationspecific differences in disease expression. Materials and Methods: This multicenter, binational, retrospective cohort study included children fulfilling the provisional PRINTO classification criteria for E/SpRJIA from 12 pediatric rheumatology centers in Türkiye and India. Demographic characteristics, clinical manifestations, human leukocyte antigen B27 (HLA-B27) status, laboratory findings, disease course, and treatment-related data were collected. A multivariable logistic regression analysis was performed to identify factors independently associated with sacroiliitis at diagnosis. Results: A total of 498 patients were included (391 Turkish and 107 Indian). At disease onset, peripheral polyarticular involvement was more common in Indian patients (40.2% vs 5.6%), whereas axial involvement was more frequent in Turkish patients (45.8% vs 18.7%). HLA-B27 positivity was higher in Indian patients than in Turkish patients (79.5% vs 56.4%). During follow-up, sacroiliitis, enthesitis, and tarsitis were more common among Turkish patients, while peripheral arthritis and dactylitis were more frequent among Indian patients. NSAIDs were used more frequently in India, whereas conventional and biologic DMARDs were more commonly used in Türkiye. In multivariable analysis, country of origin remained independently associated with sacroiliitis at diagnosis, whereas HLA-B27 positivity did not. Conclusion: E/SpRJIA demonstrates substantial phenotypic heterogeneity between Turkish and Indian populations. Turkish patients exhibited a predominantly axial disease pattern, whereas Indian patients more commonly showed peripheral polyarticular involvement despite higher HLA-B27 positivity. These findings suggest that factors beyond HLA-B27, including broader genetic, environmental, and population-specific influences, may contribute to differences in disease expression. Further prospective and genetically informed studies are needed to clarify the determinants of this heterogeneity. Cite this article as: İşgüder R, Sharma A, Kızıldağ Z, et al. Comparison of the Clinical Expression of Patients with Enthesitis/Spondylitis-Related Juvenile Idiopathic Arthritis (E/SpRJIA) from Türkiye and India. ArchRheumatol. Published online September 1, 2026. doi: 10.5152/ArchRheumatol.2026.25122.

Objectives: Behçet’s disease (BD) is a multisystemic inflammatory disease, and its neurologic involvement represents serious manifestations. This study aimed to elucidate the neurologic manifestations of BD in a large paediatric population and to describe its outcomes and prognosis. Methods: This retrospective multicentre study included 75 paediatric patients diagnosed with neuro-Behçet’s disease (NBD). Demographic data, clinical features, neuroimaging findings, treatments and disability conditions were reviewed. Results: Neurologic manifestations were observed in 52 patients (69.3%) at initial presentation and in 23 patients (30.7%) at a median of 14 months (8–30) after diagnosis. The distribution of NBD subgroups was as follows: 56 patients (74.7%) with nonparenchymal, 15 patients (20%) with parenchymal and 4 patients (5.3%) with mixed forms. Headache, observed in 70 patients (93.3%), was the most common neurological symptom, being significantly more frequent in the non-parenchymal form (P = 0.028). Paresis, sensory loss and mood/mental health problems were significantly more frequent in the parenchymal form (P = 0.034, P = 0.016, P = 0.005, respectively). Neuroimaging revealed parenchymal lesions in 19 patients (25.3%), and vascular lesions were in 55 patients (73.3%). Azathioprine was the most commonly used immunosuppressor therapy (78.7%). The median follow-up duration of patients was 30 months (17.5–52.5), during which significant clinical improvement was observed, with no deaths reported. Conclusion: Paediatric NBD remains a significant issue in paediatric rheumatology. The non-parenchymal subgroup is the predominant form in children. Paediatric NBD should be confirmed and classified by MRI. The early aggressive immunosuppressive therapy provides clinical improvement. Activity and disability indexes are valuable for treatment and follow-up.

Nailfold Capillaroscopy: A Non-invasive Tool for Early Detection of Microvascular Alterations in Children with Type 1 Diabetes Mellitus - Journal of Clinical Research in Pediatric Endocrinology

Objectives: This study aimed to investigate coronavirus disease 2019 (COVID-19) vaccination rates and factors affecting vaccination in children with rheumatic diseases. Patients and methods: This multicenter cross-sectional survey-based study was conducted between July 2022 and September 2022. Four hundred seventy-four patients (256 females, 218 males; median age: 15 years; interquartile range, 13 to 16 years) were included in the patient group, and 211 healthy children (124 females, 87 males; median age: 15 years; interquartile range, 13 to 16 years) were included in the control group. A questionnaire was administered to the parents face-to-face during routine outpatient visits. Results: Of the patients, 220 were followed up with the diagnosis of autoinflammatory disease, 174 with juvenile idiopathic arthritis, 48 with connective tissue disease, 23 with vasculitis, eight with uveitis, and one with sarcoidosis. In the study group, 256 (54%) patients and 115 (54.5%) healthy children received at least one dose of COVID-19 vaccine. Parents' concern regarding potential side effects of the vaccine was the most common reason for COVID-19 vaccination hesitancy in both groups. The median patient age, follow-up period, colchicine treatment rates, childhood vaccination and influenza vaccination rates, median parental age, parental vaccination rate, and parental education level were higher in vaccinated patients (p

Objectives: The study aimed to determine the factors that increase the risk of disease flare in patients with juvenile idiopathic arthritis who stopped methotrexate (MTX) monotherapy following inactive disease (ID). Patients and methods: In the retrospective study, files of all juvenile idiopathic arthritis cases between April 1992 and June 2022 were examined. Patients who stopped MTX monotherapy following ID were evaluated. Patients with disease flare and persistent ID were compared. Juvenile idiopathic arthritis subgroup, age of symptom onset, autoantibodies, acute phase reactants, MTX method of use, and withdrawal strategy were recorded. Systemic juvenile idiopathic arthritis patients were excluded from the study due to different clinical symptoms, diagnosis, and treatment methods. Results: Files of 1,036 patients were evaluated, and 107 patients (88 females, 19 males; mean age: 5.9±4.2 years; range, 0.8-16.5 years) were included in the study. The median age at symptom onset was 4.8 (interquartile range [IQR]: 2-7.6) years. In terms of juvenile idiopathic arthritis subgroups, 52 (48.6%) had oligoarticular juvenile idiopathic arthritis, 43 (40.2%) had polyarticular juvenile idiopathic arthritis, and 12 (11.2%) had juvenile psoriatic arthritis. The patients reached ID in nine (IQR: 4.8-17.7) months after starting MTX, and MTX treatment was discontinued after one (IQR: 0.7-1.3) year following ID. The disease flare developed in 59 (55%) of the cases. The ID continued in 48 (45%) patients. In multivariate analysis, the risk of flare was associated with younger symptom onset (odds ratio [OR]=2.2, p=0.006), antinuclear antibody positivity (OR=1.6, p=0.03), higher erythrocyte sedimentation rate (OR=1.01, p=0.04), and C-reactive protein (OR=1, p=0.02) at the MTX onset. No difference was observed between the two groups regarding MTX dose, route of administration, prior and concomitant treatments, time to reach ID, and time and method of MTX discontinuation. Conclusion: In this study, the risk of flare was associated with patient’s characteristics, rather than the administration and discontinuation method of MTX.

Aim Morphea, also known as localized scleroderma, is an immune-mediated disease and the most common form of scleroderma in children. It is a localized sclerosing disease of the skin, but can also involve such adjacent tissues as the fascia, muscle, bone, and underlying tissues. This multicenter study aimed to evaluate Turkish pediatric morphea patients, regarding demographics, treatments, and response to treatment. Materials and methods The study was performed by the Pediatric Rheumatology Academy and included pediatric morphea patients from 6 Turkish pediatric rheumatology centers who were followed up for ≥6 months. Demographic, clinical, and laboratory findings and treatment modalities were analyzed. The patients were divided into 3 groups according to treatment response, as follows: group 1: topical treatment response, group 2: methotrexate response, and group 3: methotrexate resistance. Clinical findings were compared between the 3 groups. Results The study included 76 patients, of which 53 (69.7%) were female. Mean age at diagnosis of morphea was 9.7 ± 4.3 years and mean duration of follow-up was 3.2 ± 2.9 years. Linear morphea was the most common form, accounting for 43.4% (n = 33) of the patients. Extracutaneous features were noted in 17 patients (22.4%) and anti-nuclear antibody positivity was noted in 32 (42.1%). In all, 14.4% of the patients received topical treatment only, whereas 86.6% received both topical and systemic treatment. The methotrexate response rate was 76.9% in the patients that received systemic immunosuppressive therapy. The overall relapse rate while under treatment was 19.7%. Conclusion In this study, most of the pediatric morphea patients responded well to methotrexate. Bilateral lesions were more common in the methotrexate-resistant group. Multiple involvement, and bilateral lesions, were more common in relapsed patients than in non-relapsed patients. Key points • Most of the pediatric morphea patients respond well to MTX. • Multiple involvement, and bilateral involvement, were more common in relapsed patients than in non-relapsed patients. • Presence of extracutaneous findings in patients increased relapse rate 5.7 times.

Pediatric mixed connective tissue disease (MCTD) is a subgroup of overlap syndromes. We aimed to compare the characteristics and outcomes in children with MCTD and other overlap syndromes. All MCTD patients met either Kasukawa or Alarcon-Segovia and Villareal criteria. The patients with other overlap syndromes had the features of ≥ 2 autoimmune rheumatic diseases but did not meet MCTD diagnostic criteria. Thirty MCTD (F/M = 28/2) and thirty (F/M = 29/1) overlap patients were included (disease onset < 18 years). The most prominent phenotype at disease onset and the last visit was systemic lupus erythematosus (SLE) in the MCTD group; juvenile idiopathic arthritis and dermatomyositis/polymyositis, respectively, in the overlap group. At the last visit, systemic sclerosis (SSc) phenotype was more frequent among MCTD than overlap patients (60% vs. 33.3%; p = 0.038). The frequency of the predominant SLE phenotype had decreased (60% to 36.7%), while predominant SSc phenotype had increased (13.3% to 33.3%) during follow-up in MCTD patients. Weight loss (36.7% vs. 13.3%), digital ulcers (20% vs. 0), swollen hands (60% vs. 20%), Raynaud phenomenon (86.7% vs. 46.7%), hematologic involvement (70% vs. 26.7%), and anti-Sm positivity (29% vs. 3.3%) were more common, while Gottron papules (16.7% vs. 40%) were less frequent among MCTD than overlap patients (p < 0.05). A higher percentage of overlap patients achieved complete remission than MCTD patients (51.7% vs. 24.1%; p = 0.047). The disease phenotype and outcome differ between pediatric MCTD and other overlap syndromes where MCTD may be regarded as a more severe disease. Analyzing these patients could pave the way for early and effective treatment.