Nuran Belder

Gastrointestinal (GI) involvement of IgA vasculitis (IgAV) has various clinical presentations from isolated abdominal pain to severe gastrointestinal bleeding. The aim of this study is to determine the incidence of GI involvement in children with IgAV and identify the predictors of severe GI involvement. Medical records of IgAV patients with GI involvement from 30 centers in Türkiye between 2014 and 2024 were retrospectively reviewed. Patients were classified into two groups according to the severity of GI presentations: Group 1 consisted of mild GI involvement, while Group 2 had severe GI involvement. Severe GI involvement was observed in 476 (42%) out of 1131 patients. A significant postpandemic (after March 2020) increase in GI involvement was observed (p < 0.001). Abdominal pain as an initial symptom, vomiting, purpuric rash on upper extremities and trunk, and kidney involvement were more frequent in Group 2 than in Group 1 (all p < 0.01). Leukocytosis, neutrophilia, thrombocytosis, increased C-reactive protein (CRP) level and neutrophil-to-lymphocyte ratio (NLR), and decreased serum total protein and albumin levels were detected more frequently in Group 2 (all p < 0.01). In multivariate regression analysis, thrombocytosis, elevated NLR and CRP, hypoalbuminemia, abdominal pain preceding rash, vomiting, and upper extremity rash were associated with severe GI involvement (all p < 0.05). ROC analyses identified the following optimal cut-off values for predicting severe GI involvement: WBC > 12.90 10^3/µL, ANS > 7.81 10³/µL, NLR > 3.09, PLT > 404 103/µL, CRP > 20 mg/L, plasma albumin ≤ 4 g/dL.Conclusion: Abdominal pain preceding rash, purpuric rash on upper extremities, vomiting, thrombocytosis, hypoalbuminemia, increased NLR, and high CRP may predict severe GI involvement in children with IgAV.

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.

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.

Oligoarticular juvenile idiopathic arthritis (oJIA) is the most common JIA subtype, with variable disease courses influencing long-term outcomes. Joint involvement patterns significantly affect disease activity, treatment response, and prognosis. This study evaluates the impact of joint involvement on disease activity, inflammatory markers, treatment needs, and disease extension in patients with oJIA. We retrospectively analysed 124 oJIA patients followed for a median of 62 months. Joint involvement, disease activity (Juvenile Arthritis Disease Activity Score [JADAS-27]), inflammatory markers (erythrocyte sedimentation rate [ESR], C-reactive protein [CRP]), and treatment modalities were assessed. Persistent and extended disease courses were compared. A total of 124 patients with oJIA (63.7% female) were included, with a median age at diagnosis of 8.2 years. The knee was the most frequently involved joint (87.1%), followed by the ankle (41.1%) and hip (22.6%). Patients with ankle, wrist, and elbow involvement had significantly higher JADAS-27 scores at baseline. Notably, ankle involvement was associated with sustained higher disease activity up to the first year and higher final CRP levels. Repeated measures ANOVA demonstrated a significant decline in JADAS-27 over time across all joint subgroups (p<0.001), with no joint-specific difference in trajectory. Ankle and small hand joint (MCP/PIP) involvement were significantly associated with increased use of cDMARDs and anti-TNF agents. Patients with extended oJIA had significantly higher JADAS-27 scores at all timepoints except the final visit and were more likely to have symmetric joint involvement and receive biologic therapy compared to those with persistent oJIA (anti-TNF use: 70% vs. 25%, p<0.001). Joint involvement patterns, particularly affecting the ankle, wrist, and small hand joints, are associated with higher disease activity and increased treatment requirements in oJIA. Symmetrical and extended disease forms further indicate a more severe course. Early identification of these high-risk features may guide personalized treatment and improve long-term outcomes.

Objectives: Artificial intelligence (AI) encompasses systems designed to perform tasks that require human cognitive abilities, such as reasoning, decision-making, and problem-solving. Open AI’s Generative Pre-Trained Transformer (GPT) model family, including ChatGPT, is widely recognized for its ability to generate human-like text and facilitate interactive discussions. ChatGPT has potential applications in diagnosis assistance and medical education in healthcare, yet its adoption raises concerns. Our study aims to evaluate ChatGPT’s diagnostic performance in identifying autoinflammatory diseases compared to clinicians, exploring its potential as an accessible tool for physicians and patients. Material and Methods: We evaluated the diagnostic performance of a publicly accessible AI model against two clinicians for identifying familial Mediterranean fever (FMF) and periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA). Clinical data from 50 patients were presented anonymously in structured format to both the AI model and the clinicians. Diagnoses were compared to confirmed clinical diagnoses. Results: A total of 50 patients were included in the study. The AI model suggested a rheumatologic diagnosis in 94% of cases but correctly diagnosed only 50% of them. In comparison, clinicians made accurate diagnoses in 76% and 70% of cases, respectively. Conclusion: The development of AI has attracted significant attention in healthcare, as it has in other fields. However, AIgenerated data may be incorrect, highlighting the importance of expert supervision. AI should complement, not replace physicians, enhancing their capabilities. Future research should evaluate AI performance across different fields and its impact on decision-making to ensure reliable use through standardized guidelines.

Juvenile idiopathic arthritis (JIA) presents with diverse phenotypes and can lead to significant morbidity. Obesity, affecting 5 to 23% of JIA patients, may exacerbate disease activity and complicate management. This study evaluates the impact of body mass index (BMI) on disease activity and the influence of joint involvement on BMI in JIA patients. Between January 2012 and June 2024, 225 JIA patients were reviewed, with 173 ultimately included based on specific inclusion and exclusion criteria. Treatments followed the American College of Rheumatology (ACR) recommendations. Data on demographics, BMI, JADAS-27 scores, joint involvement, and laboratory parameters were analyzed, with statistical significance defined as p < 0.05. Among 173 JIA patients, significant increases in weight and height SDS were observed (p < 0.05). Obese patients at baseline had higher JADAS-27 scores at 6 months (p < 0.05). A positive correlation was found between initial JADAS-27 and final BMI SDS (r = 0.170, p < 0.05). Patients with hip involvement had lower BMI at diagnosis and at the first-year follow-up (p < 0.0001, p = 0.049), while knee involvement was linked to lower height SDS at the second year (p = 0.041). Conclusion: Our study revealed that baseline obesity is a significant risk factor for poorer disease control at the 6-month follow-up in JIA patients. We also observed that effective treatment led to improvements in growth, particularly in patients with hip involvement, who initially had lower BMI values. These findings underscore the importance of monitoring BMI in JIA patients to optimize disease management and long-term outcomes. What is Known:• Overweight and obesity are prevalent in children with JIA, with reported rates ranging from 5 to 23% in various studies.• The relationship between BMI and disease activity in JIA remains controversial, with previous studies reporting conflicting results on whether BMI impacts disease remission or activity. What is New:• Baseline obesity in juvenile idiopathic arthritis (JIA) patients is associated with worse disease activity during follow-up, particularly evident at the 6-month visit.• Patients with hip involvement had significantly lower BMI values, indicating a potential correlation with more severe disease.• Improved linear growth was observed in JIA patients during follow-up, indicating that effective treatment positively impacts growth despite the chronic nature of the disease.