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.

Cognitive performance, psychiatric comorbidities, and quality of life in pediatric patients with juvenile idiopathic arthritis: a comparative analysis with healthy controls.

This study aimed to assess the extent of cognitive impairment in children and adolescents with Juvenile Idiopathic Arthritis (JIA). While cognitive deficits are recognized in other systemic rheumatic diseases, exploration within the pediatric JIA population remains limited. The investigation utilized a comprehensive approach to examine neuropsychological test performance.

How do gene mutation diversity and disease severity scoring affect physical capacity and quality of life in children/adolescents with Familial Mediterranean Fever?

Objectives: The aim of this study is to examine how gene mutation diversity and disease severity affect physical capacity and quality of life in children/adolescents with Familial Mediterranean Fever (FMF).

Methods: Eighty children/adolescents (42 female, 38 male) diagnosed with FMF according to Tell-Hashomer diagnostic criteria were included in this study. Disease severity score (PRAS), running speed and agility and strength subtests of Bruininks-Oseretsky Test of Motor Proficiency Second Edition Short Form (BOT-2 SF), Physical Activity Questionnaire, Pediatric Quality of Life Inventory 3.

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 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.

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.

Inflammatory comorbidities ın the largest pediatric Familial Mediterranean fever cohort: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG).

Aim: The aim of this study was to investigate the frequency and type of FMF-associated inflammatory diseases in a large FMF pediatric patients and to compare them to those FMF patients without concomitant inflammatory diseases.

Materials And Methods: Familial Mediterranean fever patients enrolled in the Pediatric Rheumatology Academy (PeRA)-Research Group (RG) were included. The patients were divided into two groups according to concomitant inflammatory disease as FMF patients who had a concomitant inflammatory disease (group 1) and FMF patients who did not have a concomitant inflammatory disease (group 1).

Do the features of juvenile psoriatic arthritis change according to age? A comprehensive evaluation of the PeRA Research Group Registry.

Objectives: To describe the clinical features and treatment outcomes of children with juvenile psoriatic arthritis (JPsA) and compare the distinct patterns of the disease between early-onset and late-onset age groups.

Methods: Patients with JPsA followed regularly for at least 6 months between 2010 and 2020 in seven paediatric rheumatology centres in Turkey were included in the study. The demographic features, clinical manifestations, treatment strategies and outcomes of the patients were evaluated retrospectively.

How common is remission in rheumatoid factor-positive juvenile idiopathic arthritis patients? The multicenter Pediatric Rheumatology Academy (PeRA) research group experience.

Objective: Rheumatoid factor (RF)-positive polyarthritis is the least common type of juvenile idiopathic arthritis (JIA). Functional disability in RF-positive polyarthritis patients is much more severe than in patients with other subtypes; but data on this subtype alone is limited. This study aimed to analyze clinical features, long-term follow-up, treatment response, and remission status in a large multicenter cohort of RF-positive polyarthritis patients.

Initial manifestations and risk factors for calcinosis in juvenile dermatomyositis: A retrospective multicenter study.

Objective: This study aimed to look for the initial manifestations of juvenile dermatomyositis (JDM), give follow-up results, and search for risk factors for the development of calcinosis.

Methods: The files of children with JDM diagnosed between 2005 and 2020 were reviewed retrospectively.

Results: The study included 48 children, 33 girls and 15 boys.

A pediatric case of Takayasu's arteritis with anti-neutrophil cytoplasmic antibody-associated vasculitis triggered by COVID-19 infection.

Takayasu's arteritis (TA) is a rare chronic granulomatous vasculitis characterized by large-vessel involvement. The aorta and its main branches are most commonly involved. Although pulmonary artery involvement is common, hemoptysis or respiratory findings are rarely seen.

Remission rates and risk factors for relapse in pediatric morphea: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG).

Aim: Morphea, also known as localized scleroderma, is an immune-mediated disease and the most common form of scleroderma in children. It is a localized sclerosing disease of the skin, but can also involve such adjacent tissues as the fascia, muscle, bone, and underlying tissues. This multicenter study aimed to evaluate Turkish pediatric morphea patients, regarding demographics, treatments, and response to treatment.

A clinical overview of paediatric sarcoidosis: Multicentre experience from Turkey.

Objectives: We aimed to outline the demographic data, clinical spectrum, and treatment approach of sarcoidosis in a large group of patients and sought to figure out the variations of early-onset (EOS) and late-onset paediatric sarcoidosis (LOS).

Methods: The study followed a retrospective-descriptive design, with the analysis of medical records of cases diagnosed as paediatric sarcoidosis.

Results: Fifty-two patients were included in the study.

Clinical characteristics and predictors for recurrence in chronic nonbacterial osteomyelitis: a retrospective multicenter analysis.

Background/aim: Chronic nonbacterial osteomyelitis (CNO) is a rare disease of unknown etiology and most commonly occurs during childhood or adolescence. The purpose of this study is to collect data on the clinical features, outcomes, and management of the disease and to identify the factors affecting recurrence.

Materials And Methods: This is a retrospective multicenter cross-sectional study of pediatric patients diagnosed with CNO.

Withdrawal of biologic therapy in juvenile idiopathic arthritis due to remission: predictors of flare and outcomes.

Objectives: To investigate patients who flared after discontinuation of biological disease-modifying anti-rheumatic agents (bDMARDs) and identify risk factors associated with flare.

Methods: A multicenter study evaluating systemic and non-systemic juvenile idiopathic arthritis (sJIA and non-sJIA) patients whose bDMARDs were ceased after remission.

Results: A total of 101 patients whose bDMARDs were ceased after remission was evaluated.

PREDICT-crFMF score: A novel model for predicting colchicine resistance in children with familial Mediterranean fever.

Objectives: To develop a novel scoring system to predict colchicine resistance in Familial Mediterranean fever (FMF) based on the initial features of the patients.

Methods: The medical records of patients were analyzed prior to the initiation of colchicine. After generating a predictive score in the initial cohort, it was applied to an independent cohort for external validation of effectiveness and reliability.

Does having MEFV gene sequence variants affect the clinical course and colchicine response in children with PFAPA syndrome?

Unlabelled: The primary aim of this study was to document the treatment modalities used in periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and look for the efficacy and safety of colchicine in the treatment of PFAPA patients. The secondary aim was to search for whether having MEFV (Mediterranean fever) gene sequence variants affect the clinical course and response to colchicine. The study was conducted in 2 pediatric rheumatology centers.

Anakinra treatment in multisystemic inflammatory syndrome in children (MIS-C) associated with COVID-19.

Objective: The study aimed to report the efficacy and safety of anakinra treatment in patients with the refractory multisystemic inflammatory syndrome in children (MIS-C).

Methods: This is a cross-sectional retrospective study consisting of pediatric patients diagnosed with MIS-C who were treated with anakinra.

Results: Among the 378 patients diagnosed with MIS-C, 82 patients (21.

Corneal and lenticular clarity in children with inflammatory disease as assessed by Scheimpflug imaging.

Background: Corneal and lenticular optical properties are not well-documented in pediatric patients with inflammatory diseases. Here we aimed to evaluate corneal and lenticular optical density as well as corneal morphology in children with ocular and/or systemic inflammation by Scheimpflug imaging.

Methods: Fifty-five eyes of 29 children with non-infectious uveitis, 56 eyes of 28 children with systemic inflammation without uveitis and 60 eyes of 31 healthy children were included.

Cluster analysis of paediatric Behçet's disease: Data from The Pediatric Rheumatology Academy-Research Group.

Objectives: Behçet's disease (BD) is a systemic vasculitis affecting many organ systems, with the involvement of all-sized arteries and veins. The study aims to determine the main characteristics of paediatric BD patients and also analyse the clustering phenotypes.

Methods: Demographic data, clinical manifestations, laboratory features, treatment schedules, and disease outcomes were achieved from patients' charts retrospectively.

The Multifaceted Presentation of the Multisystem Inflammatory Syndrome in Children: Data from a Cluster Analysis.

Background: The aim of this study was to evaluate the outcomes of patients with the multisystem inflammatory syndrome in children (MIS-C) according to phenotypes of disease and define the prognostic factors for the severe course.

Methods: This cross-sectional study included 293 patients with MIS-C from seven pediatric rheumatology centers. A two-step cluster analysis was performed to define the spectrum of disease and their outcomes were compared between each group.

Is it all about age? Clinical characteristics of Kawasaki disease in the extremely young: PeRA research group experience.

Objectives: In the evaluation of children with Kawasaki disease (KD), the age of onset is important and complications may occur if the distinctive features are not assessed accordingly. The objective of the study is to define the clinical and laboratory presentations and treatment outcomes of KD in infants ≤6 months of age compared to those >6 months multicentrically.

Methods: This retrospective study reviewed the medical records of the patients diagnosed with KD and followed up between January 2009 and January 2019.