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The indications, dosage, and duration of systemic corticosteroid treatment may vary among pediatric rheumatologists (PRs) and adult rheumatologists (ARs) in daily clinical practice. This study aims to evaluate and compare practices, perceptions, and attitudes of PRs and ARs regarding the use of systemic corticosteroids. An online survey was administered to rheumatologists in Türkiye to assess their use of systemic corticosteroids in the management of rheumatic diseases. The questionnaire addressed preferred corticosteroid formulations, dosing and tapering strategies, monitoring and preventive measures for adverse effects, and vaccination practices. Sixty PRs and 40 ARs participated in the study. Prednisolone was most commonly preferred by PRs while almost all ARs used methylprednisolone. Weight-based dosing was favored by PRs whereas ARs preferred fixed-dose regimens. PRs more frequently preferred longer pulse corticosteroid therapy, whereas ARs most commonly used a 3-day pulse regimen. In contrast, ARs reported significantly longer durations of bridging and maintenance therapy. Delirium was reported significantly more often by ARs as a complication (p = 0.026). In pre-treatment evaluation, PRs more commonly assessed peripheral blood smear (p < 0.001) whereas ARs more frequently screened for hepatitis serology (p = 0.014). As preventive strategies, PRs were significantly more likely to request ophthalmologic examination (p = 0.005). Immunization assessment for meningococcal, measles-mumps-rubella, and varicella vaccines was significantly more common among PRs (p > 0.005, for all). Significant heterogeneity exists between PRs and ARs in systemic corticosteroids practices, highlighting the need for age-specific standardized protocols for dosing, preventive strategies, and complication monitoring. Graphical abstract available for this article.

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

Objective: Cutaneous manifestations can indicate various systemic diseases, including rheumatological, metabolic, endocrinological, infectious, gastrointestinal, and oncological disorders. The study aimed to examine the demographic, clinical, and laboratory characteristics as well as the final diagnoses of pediatric patients who presented with a rash complaint at a rheumatology clinic. Materials and Methods: This was a retrospective, cross-sectional, and descriptive study conducted at the pediatric rheumatology department of the center. The medical records of patients aged 0-18 years who presented with a rash as one of the initial complaints at the pediatric rheumatology clinic between January 2017 and December 2023 were reviewed. Data on demographics, clinical presentation, laboratory results, and final diagnosis were analyzed. Results: A total of 307 patients were included, including 166 women (54%). Of these, 255 (83%) were diagnosed with rheumatic disease. The most common rash type was petechiae/purpura (63.5%), followed by maculopapular rash (10%), urticarial rash (10.7%), and others. The most frequent rheumatic diagnosis was immunoglobulin A vasculitis. Other diagnoses include juvenile dermatomyositis, systemic lupus erythematosus, Kawasaki disease, and multisystem inflammatory syndrome in children. Conclusion: Skin manifestations often present as the initial complaint in pediatric rheumatic diseases. A thorough cutaneous examination is crucial for identifying the underlying systemic conditions and guiding appropriate management.

Objectives: Exertional leg pain (ELP) is an underrecognized manifestation of FMF with unclear pathophysiology. This study performed a comprehensive ultrasonographic evaluation of lower-extremity joints, tendons and entheses in paediatric FMF patients with ELP, comparing findings with non-ELP patients and matched healthy controls. Methods: Paediatric FMF patients carrying homozygous or compound heterozygous pathogenic MEFV variants were enrolled in this prospective study. Demographic, anthropometric and clinical data were collected, while a blinded operator performed standardized ultrasonographic evaluations. Results: A total of 50 patients and 25 heathy controls were included (25 with ELP, 25 without). No differences were observed between the groups in demographic, anthropometric or FMF-related factors, including MEFV mutations and ISSF scores. Among the evaluated parameters, Achilles tendon thickness was significantly increased in children with ELP, independent of demographic or anthropometric factors, compared with both healthy and non-ELP group (P < 0.01). Nine patients exhibited grade 1 knee synovial effusions, predominantly in the ELP cohort, most with subclinical inflammation. One patient with ELP demonstrated enthesitis and was diagnosed with HLA-B27-negative ERA. Patellar tendon and plantar fascia thickness were comparable. Conclusion: Paediatric FMF patients with ELP demonstrated increased achilles tendon thickness. Although no standardized cut-off exists for tendinopathy, this may reflect tendon structural changes related to ELP. Moreover, FMF patients, particularly those with ELP, exhibited inter-attack knee effusions which may serve as a marker for subclinical inflammation. Larger longitudinal cohorts are needed to define ELP, elucidate its underlying pathways and clarify the clinical significance of Achilles tendon thickening.

Objectives: This study aims to evaluate the frequency and predictors of immunoglobulin A vasculitis (IgAV) late relapses based on clinical and laboratory features in children. Methods: We retrospectively reviewed the medical records of IgAV patients. To assess the severity of the disease, the Paediatric Vasculitis Assessment Score (PVAS) was calculated for each patient during the initial visit. Late recurrence of IgAV was defined as the reappearance of skin lesions and/or systemic involvement after a symptom-free period of at least 6 months. Patients were divided into two subgroups: Group 1 consisted of IgAV patients who experienced at least one recurrence, while Group 2 included those with no recurrence. Results: A total of 40 (7.3%) patients were in Group 1. Male gender, mean age at diagnosis, gastrointestinal and testicular involvement, white blood cell count, creatinine level, and paediatric vasculitis, the PVAS at the first visit were significantly higher in Group 1 than in Group 2 (P = .033, P = .015, P = .047, P = .003, P = .012, P = .012, and P = .000, respectively). Male sex, diarrhoea, testicular involvement, white blood cell value, and PVAS were found to be independent risk factors to predict IgAV late recurrence (P = .033, P = .015, P = .003, P = .025, and P = .005, respectively). Conclusions: Close follow-up is essential, particularly in male patients and in the presence of testicular involvement, diarrhoea, or a high PVAS at the initial visit, given the potential for late disease recurrence in the long term.

Introduction: Juvenile spondyloarthropathies (JSpA) are a group of chronic inflammatory diseases that differ in their clinical features and course from adult spondyloarthropathies and other subtypes of juvenile idiopathic arthritis (JIA). Therefore, defining disease inactivity in JSpA requires specific criteria. This Delphi study aimed to establish a national consensus on its core clinical, laboratory, and radiological domains. Methods: A total of 27 pediatric rheumatologists participated in the Delphi survey, conducted in two rounds. Participants were asked multiple‐choice and Likert‐type questions regarding their preferences for using domains including anamnesis, laboratory findings, imaging methods, and predefined disease activity scores for assessing inactivity. At the end of each round, the study coordinators determined the “strong consensus” items based on a power analysis of these parameters. Results: The absence of “pain or tenderness in the peripheral joints, lower back and entheseal regions” in anamnesis domain and “tenderness in the peripheral joints, entheseal areas, and hip examination”; “swelling in the peripheral joints; tenderness on sacroiliac compression testing”; and “no reduction in hip RoM examination” in physical examination domain received the highest scores and were accepted as strong consensus. Furthermore, normalization of MRI findings of SIJ and hip/peripheral joint and “physician global score = 0” reached the specified thresholds, resulting in strong consensus following the second round. Conclusions: This Delphi study highlights the need for a multidimensional approach that integrates clinical, radiological, and physician assessments to define disease inactivity in patients with JSpA. The resulting consensus provides a more specific assessment on inactivity defining JSpA patients and reflects a national consensus.

Aim: Evaluating the walking capacity of patients with juvenile idiopathic arthritis (JIA) is important to understand disease progression and to determine treatment efficacy. This study aims to investigate the walking capacity of JIA patients and to explore potential factors affecting their performance.Material and Method: A total of 30 JIA patients were enrolled between January 2023 and March 2023; as one patient could not complete the 6-minute walk test due to leg pain, analyses were performed on 29 patients. Before and after the test, the patients' pulse, oxygen saturation, and blood pressure values were recorded.Results: Among the patients, 16 (55.2%) were female. The median age was 14.7 years (range: 8-19 years), and the median age of disease onset was 9.3 years (range: 1-17 years). The distribution of patients by JIA subtype was as follows: 22 (75.9%) had oligoarticular JIA, 5 (17.2%) had enthesitis-related arthritis, 1 (3.4%) had RF-positive polyarticular JIA, and 1 (3.4%) had psoriatic arthritis. The mean walking distance achieved by the patients was 536.8±80.8 meters. The patients walked 73.7- 102.5% of the walking distances predicted in the literature, and a significant decrease in walking capacity was found.Conclusion: This study suggests reduced walking capacity among JIA patients relative to published reference values. A lower walking capacity was observed in patients receiving treatment compared with those in remission, although this finding may be influenced by confounding by indication and should be interpreted with caution. These preliminary findings support further investigation into the potential value of physical activity as an adjunct to medical treatment in JIA.