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.

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.

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.

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).

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.

Deficiency of adenosine deaminase 2 as an unrecognized cause of early-onset stroke and cranial nerve palsy.

Objective: The aim of this study is to evaluate the clinical, laboratory, and radiological findings and prognosis of patients with adenosine deaminase 2 deficiency (DADA2) and to highlight the conditions that DADA2 should be considered in the differential diagnosis in patients with neurological findings.

Methods: A case series of six DADA2 patients was presented in this retrospective, descriptive study. Clinical and laboratory data, treatment protocols, and prognosis of the patients were recorded.

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.

Evaluation of vascular and perfusional changes in the retina and choroid of children with multisystem inflammatory syndrome.

Background: Multisystem inflammatory syndrome in children (MIS-C) is hyperinflammation following coronavirus disease 2019 (COVID-19), which affects many organs. The retina and choroid are affected by COVID-19 through microangiopathy and thrombosis but the literature on MISC-C is limited.

Methods: Thirty children (60 eyes) with MIS-C (the study group, or SG) and 32 age-and gender-matched healthy children (64 eyes) (the control group, or CG) were included in the prospective case-control study.

Clues for inflammatory diseases in the differential diagnosis of a child with sacroiliitis.

Background: The purpose of this study was to compare the demographic, clinical and laboratory characteristics of patients with enthesitis-related arthritis (ERA), familial Mediterranean fever (FMF) and inflammatory bowel disease (IBD), which are inflammatory diseases that may develop sacroiliitis. Thus, it was aimed to reveal various findings that may indicate primary disease in patients with sacroiliitis.

Methods: Pediatric patients aged 6-18 years, who were being followed with a diagnosis of ERA (n = 62), FMF (n = 590), and IBD (n = 56) over the period 2013-2021 were included in the study.

Intussusception as a rare manifestation of immunoglobulin A vasculitis: Risk factors and treatment.

Aim: To identify the risk factors associated with intussusception in children with immunoglobulin A vasculitis (IgAV)-gastrointestinal (GI) tract involvement and to evaluate the outcomes of medical treatment and surgical intervention and the course of patients with intussusception.

Methods: This retrospective study was conducted in 157 patients under 18 years of age who were followed up with the diagnosis of IgAV-GI tract involvement between January 2015 and September 2022. The characteristics of the patients who developed intussusception were evaluated in detail.

Intravenous immunoglobulin therapy in immunoglobulin A vasculitis with gastrointestinal tract involvement.

Objective: The aim of this study is to evaluate the outcomes of patients who received intravenous immunoglobulin (IVIG) for immunoglobulin A vasculitis (IgAV) with gastrointestinal (GI) tract involvement, and to determine the differences between the groups that responded to IVIG and those that did not.

Methods: This retrospective study comprised 152 patients with IgAV between 2018 and 2022. Sixty-five patients (43%) had GI tract involvement.

Preadolescent-versus adolescent-onset immunoglobulin A vasculitis: The impact of age on prognosis.

Background: This study aimed to investigate the characteristics of patients with preadolescent- and adolescent-onset immunoglobulin A vasculitis (IgAV) and to determine whether age affects IgAV outcomes in adolescents.

Methods: Demographic, clinical, and laboratory data of 333 patients diagnosed with IgAV at the Department of Pediatric Rheumatology, University of Health Sciences, Ankara City Hospital, were evaluated retrospectively. The patients were classified into two groups: preadolescents (<10 years) and adolescents (10-19 years).

Steroid-resistant peripheral neuropathy in a child: a rare finding in immunoglobulin a vasculitis.

Background: Immunoglobulin A vasculitis (IgAV; Henoch-Schönlein purpura) is the most common vasculitis of childhood, affecting the small vessels with systemic involvement, especially the skin, joints, gastrointestinal system and kidneys. Peripheral neuropathy is very rare. Herein, we present a patient who was diagnosed as IgAV and developed refractory peripheral neuropathy in the course of disease.

Role of Biological Agents in the Treatment of SARS-CoV-2-Associated Multisystem Inflammatory Syndrome in Children.

Objectives: The aims of this study were to evaluate the role of biological agents in the treatment of severe multisystem inflammatory syndrome in children (MIS-C) and to assess the current application, outcomes, and adverse effects in patients who are followed up in a pediatric intensive care unit (PICU).

Patients And Methods: This observational, descriptive, medical records review study was performed on patients with MIS-C admitted to the PICU between September 1 and November 1, 2020. Through medical records review, we confirmed that patients were positive for current or recent SARS-CoV-2 infection or for COVID-19 exposure history within the 4 weeks before the onset of symptoms.

Heart rate variability in juvenile systemic lupus erythematosus patients.

Başaran Ö, Çetin İİ, Aydın F, Uncu N, Çakar N, Ekici F, Çelikel Acar B. Heart rate variability in juvenile systemic lupus erythematosus patients. Turk J Pediatr 2019; 61: 733-740.

Wegener granulomatosis as an uncommon cause of panhypopituitarism in childhood.

Wegener granulomatosis (WG) is a cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA)-associated, multi-system, necrotizing granulomatous vasculitis. Inflammation of the nasal or oral mucosa, and lung and kidney involvements are typical in the course of the disease. In rare cases, pituitary involvement may occur and cause panhypopituitarism.