Cyclophosphamide treatment with a comparison in both pediatric rheumatology and pediatric nephrology practices.

Background: Cyclophosphamide (CYC) is an inactive alkylating agent that transforms the alkyl radicals into other molecules and is used in combination with systemic corticosteroids in the treatment of many childhood rheumatic diseases, such as systemic lupus erythematosus (SLE), and ANCA-associated vasculitis (AAV). In recent years, rituximab (RTX), a B-cell-targeting anti-CD20 monoclonal antibody, has emerged as a new alternative treatment modality over CYC for induction therapy of childhood-onset rheumatic diseases. Clinicians adopt different practices for using CYC particularly in relation to indications, posology, pre-treatment laboratory work-up, post-treatment follow-up, and screening pre- and post-treatment vaccination status.

Assessment of sacroiliitis using zero echo time magnetic resonance imaging: a comprehensive evaluation.

Background: Enthesitis-related arthritis (ERA), a subset of juvenile idiopathic arthritis (JIA), is characterized by frequent involvement of the sacroiliac (SI) joints.

Objective: The aim of this study was to assess the effectiveness of zero echo time (ZTE) magnetic resonance imaging (MRI) in identifying structural lesions in patients with ERA. Conventional MRI pulse sequences often struggle to adequately visualize osseous and calcified tissues.

Corticosteroid use in PFAPA syndrome: clinical practice data from the JIR-CliPS Survey Study and a comprehensive literature review.

Objectives: Corticosteroids are used to abort disease flares in PFAPA syndrome. We aimed to obtain a global overview of physicians' corticosteroid usage strategies and analyze the data in the literature regarding corticosteroid use in PFAPA syndrome.

Methods: The JIR-CliPS PFAPA questionnaire included nine questions on corticosteroid use in addition to the demographic data questions.

Assessment of Health Literacy in Parents of Children With Familial Mediterranean Fever Using the Turkish Health Literacy Scale.

Background Health literacy (HL) refers to the ability of individuals to find, understand, and use information and resources to make informed health-related decisions and actions for themselves and others. Managing chronic diseases in children and adolescents requires active family involvement. The primary objective of the study is to evaluate the HL levels of parents of children diagnosed with familial Mediterranean fever (FMF).

The first proteomics analysis of tonsils in patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA).

Background: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a recurrent fever syndrome. The exact etiopathogenesis of PFAPA syndrome remains unknown. Biological fluids or tissues may provide disease-specific biomarkers that may help clinicians to find new pathogenic pathways.

The journey of MEFV heterozygous children: with or without colchicine.

To investigate the rate of colchicine use in the longitudinal follow-up of familial Mediterranean fever (FMF) carriers and identify variables that could predict the necessity of colchicine treatment in this group. The study was conducted in 9 pediatric rheumatology centers. The files of children with MEFV gene carriers were retrospectively reviewed between February 2014 and May 2024.

Comparison of low-dose CT and MRI in enthesitis-related arthritis patients with sacroiliitis.

Objective: This study investigated the utility of low-dose CT (ldCT) compared with MRI in diagnosing sacroiliitis in enthesitis-related arthritis (ERA) patients.

Methods: Thirty patients diagnosed with ERA were evaluated, with a median follow-up of 1.47 years.

A rare disease with many faces: A multicenter registry of IgG4-related disease in children.

Objectives: We aimed to report the characteristics of pediatric IgG4-related disease (IgG4-RD) through a multicentre registry, to assess disease clusters, and to evaluate the performances of the 2019 American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classification criteria and the 2020 revised comprehensive diagnostic (RCD) criteria in this cohort.

Methods: Data of IgG4-RD patients in 13 pediatric rheumatology centers were recorded to a web-based registration system. The diagnosis of IgG4-RD was made according to the 2011 comprehensive diagnostic criteria.

Impact of perception of illness on quality of life in juvenile systemic lupus erythematosus.

Objective: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organs, notably the skin, joints, and kidneys. The primary goal in managing SLE is to enhance patients' quality of life (QoL). Illness perception can influence QoL in patients with chronic disease.

Exploring gastrointestinal manifestations in childhood onset systemic lupus erythematosus - Insights from a multicenter study.

Objective: Systemic lupus erythematosus (SLE) constitutes an autoimmune disorder with potential involvement of the gastrointestinal system (GIS). Our objective was to assess the gastrointestinal (GI) manifestations in patients diagnosed with childhood onset SLE.

Methods: The study cohort consisted of 123 patients with childhood onset-SLE and GIS involvement from 16 referral departments of pediatric rheumatology.

Exploring factors for predicting colchicine responsiveness in children with PFAPA.

Unlabelled: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) are the most common autoinflammatory syndromes in children. This study aimed to evaluate the clinical and laboratory parameters that may predict colchicine responsiveness.This retrospective, multicenter, cross-sectional study involved nine pediatric rheumatology centers from our country.

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.

Cardiac evaluation of patients with juvenile dermatomyositis.

Background: The present study aims to evaluate possible cardiac involvement in juvenile dermatomyositis (JDM) patients by conventional methods and cardiac magnetic resonance imaging (MRI) along with a systematic review of the literature on cardiac features in JDM.

Methods: The study group consisted of JDM patients who underwent cardiac MRI. We conducted a systematic review of the published literature involving JDM patients with cardiac involvement.

Retinal vessel density and choroidal flow changes in oligoarticular juvenile idiopathic arthritis with and without uveitis.

Purpose: This cross-sectional optical coherence tomography angiography (OCTA) study aimed to assess the macular and optic nerve head (ONH) vascular density, foveal avascular zone, and outer retina and choriocapillaris flow in oligoarticular juvenile idiopathic arthritis (oJIA).

Study Design: Prospective.

Methods: Twenty-two eyes of 22 oJIA patients with uveitis (oJIA-U), 20 eyes of 20 oJIA patients without uveitis (isolated oJIA), and 26 healthy volunteers of similar ages and sexes were investigated.

Pediatric Primary Raynaud's Phenomenon: A Comprehensive Cardiovascular Analysis.

Our aim in this study is to evaluate the cardiovascular findings of pediatric patients with primary Raynaud's phenomenon (RP) and to determine if there are any pathological findings. Our study included 42 pediatric patients aged between 7 and 18 who were diagnosed with primary RP and did not have any additional underlying structural vascular disease or secondary rheumatological conditions. The control group consisted of 30 healthy volunteers aged 7-18 years, matched by age and sex, without any additional diseases.

The relationship between Behçet's disease and the quality of life for pediatric patients and their parents.

Objectives: Chronic diseases impact people's quality of life (QoL). Behçet's disease (BD) is a multisystemic chronic disease characterized by vasculitis of various vessels. We aimed to assess QoL in paediatric BD (PEDBD) patients and their parents.

Evaluation of childhood malignancies presenting with musculoskeletal manifestations from two different divisions: a multicenter study.

Background: The aim of the study was to evaluate the approaches of pediatric rheumatologists and pediatric hematologists to patients with similar musculoskeletal (MSK) complaints and to highlight the differences that general pediatricians should consider when referring patients to these specialties.

Methods: This is a cross-sectional study involving the patients who applied to pediatric rheumatology centers with MSK complaints and were diagnosed with malignancy, as well as patients who were followed up in pediatric hematology centers with a malignancy diagnosis, and had MSK complaints at the time of admission.

Results: A total of 142 patients were enrolled in the study.

Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis on anti-interleukin-1 or -6 therapy.

Objectives: The aim of this study is to investigate the effect of anti-interleukin (IL)-1/-6 biologics on systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS).

Methods: Demographic, clinical and laboratory data of patients followed up with a diagnosis of sJIA-associated MAS assessed from sixteen paediatric rheumatology centres across the country. The clinical and laboratory features of MAS developing while on biological drugs were compared with those without this treatment.

Towards a standardized program of transitional care for adolescents with juvenile idiopathic arthritis for Turkey: a national survey study.

Background: Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored.

Is it possible to extend the dose interval of canakinumab treatment in children with familial Mediterranean fever? PeRA group experience.

Background: There is no clear data on the optimal duration of treatment with anti-interleukin-1 drugs in colchicine-resistant familial Mediterranean fever patients, as well as on the dose interval. This study aimed to assess patients whose canakinumab dose interval was adjusted according to a specific protocol, with the objective of evaluating the effectiveness of implementing this protocol for the patient care.

Methods: The files of 45 patients whose canakinumab treatment interval was opened with a standard protocol previously determined by the Delphi method were retrospectively reviewed.

The safety of canakinumab in systemic juvenile idiopathic arthritis and autoinflammatory diseases in pediatric patients: a multicenter study.

Objective: To evaluate the safety of canakinumab using real-world data in patients with systemic juvenile idiopathic arthritis (sJIA) and autoinflammatory diseases (AID).

Research Design And Methods: This was a cross-sectional observational, multicenter study. Patients diagnosed with AID and sJIA treated with canakinumab were included in the study.