Biological therapies in pediatric Behçet's disease: results of an international collaborative study by the PRES Vasculitis Working Party.

Objective: This study aims to examine the indications for pediatric rheumatologists to use biologic therapies in childhood Behçet's disease (BD), as well as the efficacy, safety, and remission rates of such treatments. We also compare demographic and clinical characteristics of Turkish and European cohorts.

Methods: In this multicentric retrospective study, the data of 109 pediatric BD (pedBD) patients diagnosed before 18 years of age, obtained from Pediatric Rheumatology European Society (PRES) vasculitis study group, which involves 19 centers across six countries were analyzed.

Predicting homozygous M694V genotype in paediatric FMF: multicentre analysis and scoring system proposal.

Objectives: This study aimed to evaluate the predictability of the homozygous M694V genotype in paediatric FMF patients and to develop a clinical scoring system to enhance disease management strategies.

Methods: This nationwide, multicentre, cross-sectional study included 3981 paediatric FMF patients with biallelic pathogenic variants in exon 10 of the Mediterranean fever gene. Patients were divided into two groups: group 1 (homozygous M694V) and group 2 (other variants).

Characteristics of scrotal involvement in IgA vasculitis: Relationship with disease activity and inflammatory markers.

Unlabelled: Immunoglobulin A (IgA) vasculitis is a common systemic vasculitis in children, involving the skin, joint, gastrointestinal tract and kidneys. Scrotal involvement is a less common manifestation in the course of IgA vasculitis, which alters disease management. The purpose of this study was to present the characteristics of patients with IgA vasculitis with scrotal involvement and to compare patients with and without scrotal involvement.

How familial Mediterranean fever affects the characteristics of immunoglobulin a vasculitis in pediatric patients at the time of diagnosis?

Unlabelled: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease of childhood and is frequently associated with systemic inflammatory diseases such as vasculitis. The aim of this study is to evaluate the impact of FMF on clinical and laboratory findings and disease activity of immunoglobulin A vasculitis (IgAV) at presentation. Patients with IgAV who were followed up for more than 3 months were included in the study.

Anti-SRP myositis: a diagnostic and therapeutic challenge.

Background: Anti-signal recognition protein (anti-SRP) myopathy is a rare idiopathic inflammatory myopathy in children. Herein, a 3-year-old patient with severe anti-SRP myopathy showing a rapidly progressive disease course is presented in order to increase the awareness of pediatricians about idiopathic inflammatory myopathies.

Case Presentation: A previously healthy 3-year-old girl presented with progressive symmetrical proximal muscle weakness that caused difficulty in climbing stairs for two months prior to evaluation, and a marked elevation of the serum creatine kinase levels.

The effect of gene dosage and age at the disease onset on the severity of familial Mediterranean fever.

Objective: To compare the demographic and clinical characteristics of familial Mediterranean fever (FMF) patients according to age at disease onset and evaluate the dose effect of the number of pathogenic or likely pathogenic exon 10 mutations of the MEFV gene on disease severity.

Methods: This medical record review study was performed on 485 pediatric FMF patients with uni- or biallelic exon 10 mutations of the MEFV gene (M694V, M694I, M680I, V726A, R761H, T267I). Patients were grouped according to age at disease onset (Group 1:<6 years; Group 2:6-11 years; and Group 3:>11 years).

Age-Related Differences Between Juvenile and Adult Autoimmune Inflammatory Myopathies.

Background: Clinical features and prognosis of autoimmune inflammatory myopathies (AIMs) can vary depending on the age of disease onset. The aim of this study was to compare the demographic characteristics, clinical features, laboratory findings, and long-term prognosis of juvenile and adult AIMs.

Methods: Patients diagnosed with AIM between 2009 and 2023 in the pediatric rheumatology and rheumatology departments of our hospital were included in this medical records review study.

Characteristics of cutaneous manifestations in immunoglobulin a vasculitis and their relationships with system involvement and treatment needs.

Unlabelled: The aim of this study was to evaluate the cutaneous manifestations of immunoglobulin A vasculitis (IgAV) in terms of skin lesion type, distribution and persistence and to investigate the relationship between cutaneous manifestations and system involvement and treatment needs. This retrospective observational study was conducted with 489 IgAV patients who were followed-up for at least 6 months between 2013 and 2024. Demographic characteristics, detailed cutaneous manifestations, clinical findings and treatments were retrieved from electronic medical records.

Can the affected intestinal segment visualized on abdominal ultrasound predict gastrointestinal tract bleeding in immunoglobulin A vasculitis?

Objectives: The study aimed to evaluate immunoglobulin A vasculitis (IgAV) patients with gastrointestinal (GI) tract involvement and to reveal the relationship between the location and extent of the affected intestinal segment detected on the initial abdominal ultrasound and GI tract bleeding.

Methods: This medical record review study was conducted on 117 IgAV patients with GI tract involvement between January 2016 and June 2023. Patients were divided into two groups: those with (n = 28) and without (n = 89) GI tract bleeding.

Sacroiliitis in familial Mediterranean fever: A rare joint involvement of the disease.

Aim: Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease characterised by recurrent episodes of fever and polyserositis. Sacroiliac joint involvement is rare in FMF patients. The purpose of this study was to evaluate the demographic, clinical, laboratory and imaging findings of patients with FMF who developed sacroiliitis.

Evaluation of pediatric rheumatologists' approach to rituximab use: a questionnaire study.

Unlabelled: Rituximab (RTX) is a chimeric monoclonal antibody that targets the CD20 antigen on B cells and is used in various autoimmune disorders. In this study, we aimed to measure the awareness of pediatric rheumatologists about the use of RTX through a survey. Between February and March 2023, a 42-question survey was sent via email to pediatric rheumatology specialists in Turkey.

Predictors of disease course in systemic juvenile idiopathic arthritis.

Objectives: The aim of this study was to identify potential predictors of the disease course of systemic juvenile idiopathic arthritis (sJIA) at the time of diagnosis.

Methods: This retrospective observational study was conducted in patients diagnosed with sJIA in our hospital between April 2009 and October 2023. The relationship between the disease course of sJIA patients and demographic, clinical, laboratory findings and complications was analysed.

When should the use of biological agents be considered in persistent oligoarticular juvenile idiopathic arthritis patients?

Unlabelled: The purpose of this study was to compare the demographic and clinical characteristics of the groups with and without bDMARDs added to the treatment of persistent oligoarticular juvenile idiopathic arthritis (JIA) patients on methotrexate (MTX) and also to determine the predictors of adding bDMARDs to treatment. This study included 86 oligoarticular JIA patients on MTX. Patients were divided into two groups receiving MTX (n = 69) and MTX plus bDMARD (n = 17).

Adverse events of biological agents in pediatric rheumatologic diseases.

Objectives: To evaluate adverse events (AEs) in pediatric patients with rheumatologic diseases being treated with approved or off-label biologic agents (BAs).

Methods: This observational, retrospective, multicenter study was conducted from 2010 to 2022 in patients under 18 years of age with rheumatic diseases who were receiving interleukin-1 antibodies (Anti-IL1), interleukin-6 antibodies (Anti-IL6), and tumor necrosis factor alpha inhibitors (anti-TNF). Efficacy, AEs, and timing of AEs were collected from electronic medical records.

Serum interleukin-33 and soluble suppression of tumorigenicity 2 in pediatric leukemia with febrile neutropenia.

The purpose of this study was to evaluate the association between interleukin-33 (IL-33) and its receptor Soluble Suppression of Tumorigenicity-2 (sST2) levels and bacterial infections during febrile neutropenia (FN) in pediatric patients with acute lymphoblastic leukemia (ALL). In this prospective, case-control study, participants were divided into 3 groups: ALL patients with FN (Group A), ALL patients without neutropenia and fever (Group B), and healthy children without infection and chronic disease (Group C). There were 30 cases in each group.

Assessment of quality of life and physical activity in patients with oligoarticular juvenile idiopathic arthritis in remission.

The purpose of this study was to evaluate physical activity (PA) and health-related quality of life (HRQOL) in children with oligoarticular juvenile idiopathic arthritis (JIA) in remission in comparison with healthy peers and to determine the disease-related factors affecting PA levels. This study was conducted with 50 oligoarticular JIA patients in remission and 50 healthy peers between 9 and 14 years. Demographic and clinical characteristics, laboratory parameters, and treatments were noted from electronic medical records.

Does switching from coated colchicine to compressed colchicine improves treatment response in patients with familial Mediterranean fever?

Aim: To evaluate the treatment response to compressed colchicine tablets in familial Mediterranean fever (FMF) patients with resistance or intolerance to coated colchicine. The secondary aim was to determine the demographic and clinical characteristics of responders to compressed colchicine.

Methods: We retrospectively reviewed the medical records of 1574 pediatric patients with FMF treated at Ankara Bilkent City Hospital.

The effect of vitamin D supplementation on attacks in PFAPA syndrome patients with low vitamin D levels.

Background-aim: To evaluate the effect of vitamin D supplementation on the frequency and duration of attacks in patients of PFAPA syndrome with low vitamin D levels.

Methods: This retrospective study comprised PFAPA patients with vitamin D deficiency/insufficiency between 2018 and 2023. The frequency and duration of PFAPA attacks before and after vitamin D supplementation were noted.

The importance of small joint involvement in oligoarticular juvenile idiopathic arthritis.

Objectives: Our study aimed to evaluate the relationship of small joint involvement with demographic, clinical, and laboratory findings and to determine its possible effects on prognosis.

Methods: This retrospective observational study was conducted in patients diagnosed with oligoarticular juvenile idiopathic arthritis (oJIA) in the paediatric rheumatology department of Ankara City Hospital between April 2009 and September 2022. The relationship between small joint involvement and demographic, clinical, and laboratory findings and prognosis was investigated by statistical methods with the data recorded from the medical records of oJIA patients.

The role of indices in predicting disease severity and outcomes of multisystem inflammatory syndrome in children.

Background: The aim of this study was to evaluate the role of the systemic immune inflammation index (SII), C-reactive protein/albumin ratio (CAR), the monocyte/lymphocyte ratio (MLR), and the neutrophil/lymphocyte ratio (NLR) in predicting disease severity, treatment, and prognosis in multisystem inflammatory syndrome in children (MIS-C).

Methods: This medical record review retrospectively evaluated the clinical and laboratory findings of 191 MIS-C patients followed in the Department of Pediatric Rheumatology at Ankara City Hospital, Turkey. The patients were grouped by disease severity: mild, moderate, and severe.

Predictors of relapse in patients with oligoarticular juvenile idiopathic arthritis in remission off medication.

The aim of this study was to evaluate the predictors of relapse in patients with oligoarticular juvenile idiopathic arthritis (oJIA) who achieved clinical remission off medication. This retrospective observational study was conducted between June 2009 and July 2022 in 126 patients with oJIA who achieved remission off medication. The relationships between relapse status and demographic, clinical and laboratory findings, and treatment details were evaluated using electronic medical records.

The Juvenile Spondyloarthritis Disease Activity Index Is a Useful Tool in Enthesitis-Related Arthritis: Real-Life Data.

Objective: To compare enthesitis-related arthritis (ERA) patients with active and inactive disease at 6 months and define baseline predictors for disease inactivity. In addition, to evaluate the demographic, clinical, and laboratory characteristics of ERA patients and to identify the real-life impact of the Juvenile Spondyloarthritis Disease Activity Index (JSpADA) in predicting active disease in ERA.

Methods: This medical record review study was conducted with 56 patients who were diagnosed with ERA at our clinic between June 2009 and June 2022.

The Effect of Clinical and Genetic Variables of Familial Mediterranean Fever Patients: Real Life Data.

Background: The Eurofever/the Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for familial Mediterranean fever (FMF) include a combination of clinical symptoms and genotype. The pathogenicity of gene variants associated with FMF is categorized by the International Study Group for Systemic Autoinflammatory Diseases (INSAID) classification criteria.

Objective: The aim of this study was to evaluate the real-life impact and usefulness of the Eurofever/PRINTO classification criteria and the INSAID classification criteria in patients with FMF and their impact on treatment management.