The Effect of Secondary Prophylaxis Adherence on Rheumatic Heart Disease and Determinants of Poor Adherence in Children: A 10-Year Retrospective Cohort Study.

Rheumatic heart disease (RHD) remains the leading cause of acquired cardiovascular morbidity and mortality in children. Since secondary prophylaxis is the cornerstone of the management of acute rheumatic fever (ARF) and RHD, this study aimed to assess the impact of prophylaxis adherence on disease recurrence and cardiac outcomes and to identify causes of non-adherence with prophylaxis. We retrospectively reviewed 137 ARF cases diagnosed before the age of 18 years, with a minimum follow-up duration of ≥ 6 months.

Colchicine efficacy on oral ulcers caused by Behçet's spectrum disorders including idiopathic recurrent aphthous stomatitis, PFAPA, and Behçet's Disease.

Unlabelled: Recurrent aphthous stomatitis (RAS) is a common condition in pediatric age. Since there are significant genetic commonalities among idiopathic cases, periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA), and Behçet's disease (BD), they were proposed to be considered in one clinical spectrum termed Behçet's Spectrum Disorders (BSDs). This retrospective study aimed to evaluate the efficacy of colchicine on oral ulcers in children with BSD related RAS (BSD-RAS).

Reported Triggers and Self-reported Management Strategies in Children With Familial Mediterranean Fever.

Objectives: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disorder. Data regarding the reported triggers of this rare disease are scarce. This study aimed to analyze the demographic data, clinical findings during the attacks, reported triggering, and self-reported management strategies of pediatric patients with FMF with exon 10 MEFV mutations.

Utilization of disease activity scores and acute phase reactants in predicting colchicine response assessed by FMF50: a retrospective cohort study in children with Familial Mediterranean Fever.

Objectives: Monitoring disease activity in Familial Mediterranean Fever (FMF) patients might be challenging, mainly due to accompanying inflammatory conditions. This study assessed disease activity and colchicine response in children with FMF, using Auto-Inflammatory Diseases Activity Index (AIDAI), Pras, Mor, International Severity Score for FMF (ISSF), and FMF50 scores, and examined their acute phase reactant (APR) predictive value for FMF50 response.

Methods: FMF patients meeting Eurofever/PRINTO criteria and receiving colchicine for ≥ 6 months were included.

Colchicine treatment in PFAPA: how long should we wait for the clinical response?

Unlabelled: We aimed to evaluate the efficacy of prophylactic colchicine treatment in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) using the Auto-Inflammatory Diseases Activity Index (AIDAI) score measured before and during treatment. We also aimed to determine the optimal treatment duration for observing clinical response and to identify factors predicting response to treatment. Children with PFAPA receiving colchicine were included in the study.

The Efficacy and Safety of Hepatitis A Vaccine in Children and Young Adults With an Autoinflammatory Diseases on Canakinumab and Tocilizumab Treatments: A Prospective Observational Controlled Study.

Intro: In this prospective interventional study, we aimed to assess the efficacy and safety of the hepatitis A vaccine in patients with autoinflammatory diseases undergoing canakinumab and tocilizumab treatments.

Methods: A total of 24 patients with autoinflammatory diseases on canakinumab and tocilizumab treatments and 39 healthy children who were seronegative for hepatitis A were included in the study. All participants were vaccinated with 2 doses of inactivated hepatitis A vaccine at 6-month intervals.

Noninfectious Uveitis in Pediatric Rheumatology: Long-term Follow-up at Tertiary Centers.

Objectives: Our study aimed to identify potential predictors for additional systemic involvement in patients with noninfectious uveitis, specifically focusing on their demographic, etiological, clinical, and laboratory data features from the pediatric rheumatology perspective.

Methods: Patients with noninfectious uveitis before the age of 18 years and followed up for at least 3 months in 2 tertiary centers of pediatric rheumatology and ophthalmology departments were included in the study. Demographics, etiology, clinical features, laboratory data, and treatments administered were evaluated and compared based on the etiology (idiopathic and systemic disease-related uveitis [SD-U]) and the use of biologic disease-modifying antirheumatic drugs.

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.

The performance of the 2019 EULAR/ACR classification criteria in childhood-onset systemic lupus erythematosus.

ObjectiveClassification criteria for systemic lupus erythematosus (SLE) are required to ensure consistency in enrolling patients into clinical trials. We aimed to evaluate the performance of the 2019 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) criteria in patients with childhood-onset SLE (cSLE) by comparing its sensitivity and specificity with the 1997 ACR and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria.MethodsOur study included 111 cSLE patients (82% female) for sensitivity analysis.

Autoinflammatory Bone Diseases.

Autoinflammatory bone diseases (AIBDs) constitute a recently identified subset of autoinflammatory diseases. These conditions are characterized by an exaggerated inflammatory response in the bones without any apparent etiology. Inflammatory bone lesions associated with AIBDs exhibit chronic inflammation, are typically culture-negative, and do not exhibit discernible microorganisms on histopathological examination.

Spontaneously regressing mass-like soft tissue involvement in a child with chronic non-bacterial osteomyelitis: case-based review.

Chronic non-bacterial osteomyelitis (CNO) is an inflammatory bone disease, usually diagnosed in childhood. It is characterized by the presence of multifocal or unifocal osteolytic lesions that can cause bone pain and soft tissue swelling. CNO is known to have soft tissue involvement.

Measuring Transition Readiness of Patients After Transfer from Pediatric to Adult Care in Rheumatology.

Transitional care is essential to maintain the continuity of care in younger patients with rheumatic diseases. In this study, we aimed to assess the transition readiness of rheumatology patients who had already transferred from pediatric to adult care using a questionnaire. We included young adult rheumatology patients who had already transferred to adult rheumatology care.

Evaluation of thyroid dysfunction in childhood-onset systemic lupus erythematosus: Risk factors for Hashimoto's thyroiditis.

Objective: Increased frequency of autoimmune thyroid disease, particularly Hashimoto's thyroiditis (HT) was reported several studies in the literature, in individuals with childhood-onset systemic lupus erythematosus (cSLE). Our study aimed to investigate the prevalence and contributing factors of thyroid dysfunction and HT among cSLE patients.

Methods: Thyroid function tests were obtained cross-sectionally from cSLE patients.

The Impact of Different MEFV Genotypes on Clinical Phenotype of Patients with Familial Mediterranean Fever: Special Emphasis on Joint Involvement.

Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease worldwide. In this retrospective cohort study, we aimed to assess the effects of various MEFV genotypes on the clinical characteristics of the patients, with a special focus on the joint involvement. In total, 782 patients with FMF were categorized into 3 groups according to the MEFV mutation; Group 1: Patients homozygous for M694V; Group 2: Patients carrying other pathogenic MEFV variants in exon 10 in homozygous or compound heterozygous states; and Group 3: FMF patients with other variants or without mutations.

Long-term safety profile and secondary effectiveness of canakinumab in pediatric rheumatic diseases: a single-center experience.

Background: To demonstrate the long-term safety profile of canakinumab over a nine-year period by documenting adverse events in patients with various pediatric rheumatic diseases.

Research Design And Methods: This retrospective observational study was conducted at the Pediatric Rheumatology Department of Istanbul University Cerrahpasa between 2015 and 2023. The analysis concerned individuals who had been administered canakinumab treatment for at least six months.

COVID-19 vaccination rates and factors affecting vaccination in children with rheumatic disease.

Objectives: This study aimed to investigate coronavirus disease 2019 (COVID-19) vaccination rates and factors affecting vaccination in children with rheumatic diseases.

Patients And Methods: This multicenter cross-sectional survey-based study was conducted between July 2022 and September 2022. Four hundred seventy-four patients (256 females, 218 males; median age: 15 years; interquartile range, 13 to 16 years) were included in the patient group, and 211 healthy children (124 females, 87 males; median age: 15 years; interquartile range, 13 to 16 years) were included in the control group.

Health conditions of first-degree relatives of children with familial Mediterranean fever.

Background: Given the strong genetic background of familial Mediterranean fever (FMF), the frequently reported co-existing diseases in children with FMF should also be investigated in other family members. Therefore, we aimed to examine the medical conditions of first-degree relatives (FDRs) of our pediatric patients with FMF in the present study.

Methods: Chronic diseases of FDRs of pediatric 449 FMF, 147 juvenile idiopathic arthritis (JIA) patients and 93 healthy controls (HC) were questioned during their routine clinical visits for 9 consecutive months.

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.

Temporary Thyroid Dysfunction and Catecholamine Excess Due to Mercury Poisoning in 6 Cases.

Objective: Mercury poisoning is a condition with multiple-organ dysfunction that has effects on the central nervous system, gastrointestinal system, cardiovascular system, skin, lungs, and kidneys. It can be fatal or may result in sequelae such as neurological disturbances, if treated late or left untreated. The endocrinological effects of mercury exposure are not well-known.

Gray zone in the spectrum of autoinflammatory diseases: familial Mediterranean fever accompanying periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome: single-center experience.

Despite the advanced knowledge concerning autoinflammatory diseases (AID), more data regarding the optimal treatment options and outcomes of the children who met the criteria of more than one AID are required. This study aimed to describe the demographic and clinical characteristics of children from familial Mediterranean fever (FMF)-endemic countries who meet both the FMF and the periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome criteria. Moreover, we aimed to measure the response rates to colchicine and tonsillectomy and evaluate the factors affecting the colchicine response in these patients.

The impact of COVID-19 on clinical course and treatment among patients with juvenile systemic sclerosis.

Objectives: This study aimed to explore the influence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic among patients with juvenile systemic sclerosis (JSS).

Patients And Methods: Twenty-seven patients (22 females, 5 males; mean age: 20 years; range, 17 to 22 years) diagnosed with JSS and followed up at the department of pediatric rheumatology were included in the cross-sectional study. A web-based survey was performed by focusing on patients' complaints, accessibility to health care, and compliance with routine treatment from January 1, 2021, to January 10, 2021.

Ovarian reserve in children with juvenile idiopathic arthritis using biologic disease-modifying anti-rheumatic drugs.

Background/objectives: The aim of the study is to assess the effect of juvenile idiopathic arthritis (JIA) and biologic disease-modifying anti-rheumatic drugs (bDMARDs) on ovarian reserve in children.

Materials And Methods: A cross-sectional study was performed from March 2021 to March 2022 and included 81 patients with JIA and 49 healthy children. Serum anti-Mullerian hormone (AMH), follicle-stimulating hormone (FSH), luteinizing hormone (LH), and estradiol levels were analyzed using electrochemiluminescence methods.