Rüya Torun

The purpose of the study is to evaluate the demographic characteristics, clinical features, and management strategies of TNF inhibitor-related autoimmune d

Introduction: Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in childhood and exhibits a heterogeneous disease course. This study aimed to analyse and identify phenotypic clusters by examining the laboratory findings, nailfold capillaroscopy results, and myositis- specific autoantibodies (MSAs) in patients with JDM. Material and methods: This retrospective cohort study included data from patients with JDM treated at the Paediatric Rheumatology Departments of 14 advanced health centres in Turkey. A categorical principal component analysis (CATPCA)-based hierarchical cluster analysis method was employed for clustering. Results: A total of 176 JDM patients were enrolled, and 5 phenotypic clusters were identified using 23 categorical variables. These clusters were interpreted as follows: Cluster A with severe muscle weakness and oesophageal involvement requiring intensive immunosuppressive treatment; Cluster B with amyopathic/hypomyopathic patients; Cluster C with skin manifestations and lung involvement; Cluster D with complicated skin manifestations; and Cluster E with classic JDM. The clinical and laboratory findings and treatments of these 5 clusters were compared. Fatigue, myalgia, photosensiti­vity, Raynaud’s phenomenon, and the use of pulse glucocorticosteroids, intravenous immuno­globulin, and cyclophosphamide treatments differed between the groups (p < 0.001, p = 0.002, p = 0.015, p = 0.036, p = 0.002, p = 0.006, and p = 0.024, respectively). Myositis-specific autoantibodies results were available for 119 patients (65.3%). The most frequent MSAs were antinuclear matrix protein 2 (26.1%) and anti-transcription intermediary factor 1 (20.9%). However, no significant differences were found in MSAs or nailfold capillaroscopy findings. Conclusions: We identified 5 clusters based on patient symptoms and findings. The identification of these 5 clusters can guide more effective treatment strategies in clinical practice. Additionally, these approaches may contribute to improving patients’ quality of life and long-term outcomes by increasing the feasibility of individualised treatment.

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.