Treatment of systemic juvenile idiopathic arthritis: conventional treatment versus biologics.

Objective: We aimed to identify and compare systemic juvenile idiopathic arthritis (sJIA) patients receiving treatment with either glucocorticoids and/or conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs) or biologic drugs.

Methods: This was a retrospective cross-sectional study. sJIA patients (n=138) were categorized into two groups: Group A (n=51) consisted of individuals who received only glucocorticoids and/or csDMARDs, while Group B (n=87) included those who received at least one biologic drug.

TGFβ links EBV to multisystem inflammatory syndrome in children.

In a subset of children and adolescents, SARS-CoV-2 infection induces a severe acute hyperinflammatory shock termed multisystem inflammatory syndrome in children (MIS-C) at four to eight weeks after infection. MIS-C is characterized by a specific T cell expansion and systemic hyperinflammation. The pathogenesis of MIS-C remains largely unknown.

Highlights from the breakout session: vasculitis in paediatric rheumatology.

The 21st International Vasculitis Workshop, held in Barcelona, Spain, from April 7 to 10, 2024, highlighted advances in pediatric vasculitis, focusing on a holistic, multidisciplinary approach. Common childhood vasculitides, including IgA Vasculitis (IgAV) and Kawasaki Disease (KD), were discussed. The Ankara 2008 criteria for IgAV, endorsed by EULAR and PReS, were evaluated for their performance in adults, showing high sensitivity but necessitating further refinement for improved specificity.

Efficacy and safety profile of biotechnological agents and Janus kinase inhibitors in VEXAS syndrome: data from the international AIDA Network VEXAS registry.

Background: VEXAS syndrome, a recently identified systemic autoinflammatory disorder, poses new diagnostic and management challenges. Based on experience with other autoinflammatory diseases, anti-interleukin (IL)-1, anti-IL-6, anti-tumor necrosis factor (TNF) biotechnological agents, and Janus kinase inhibitors (JAKis) have been widely employed in VEXAS patients. The aim of this study is to evaluate the global effectiveness and safety of biotechnological agents and JAKis using data from the real-world context.

Safety of colchicine on fertility, pregnancy, and lactation: a systematic review and meta-analysis informing the EULAR/PReS recommendations for familial Mediterranean fever.

Objectives: To summarise the evidence of colchicine's effects on fertility, pregnancy, and lactation in the treatment of patients with familial Mediterranean fever (FMF).

Methods: Two reviewers and a methodologist conducted the systematic review. Together with an expert in FMF, they established the protocol and the PICOt questions.

Pediatric rheumatology on social media: experts ensure accuracy, public drives engagement - a comparative analysis.

We aimed to investigate the quality and characteristics of content related to pediatric rheumatology on social media, comparing posts by health professionals (HPs) and non-HP (NHPs). Content creators, engagement metrics, sentiment, and misinformation were evaluated in the 150 most popular posts from 18 hashtags related to pediatric rheumatology on Facebook, Instagram, and TikTok. The Journal of American Medical Association Benchmark Scale (JAMA) and the Patient Education Materials Assessment Tool for Audiovisual Materials (PEMAT-A/V) were used to assess quality, understandability and actionability in educational videos, respectively.

TEMPORARY REMOVAL: EULAR/ACR classification criteria for paediatric chronic nonbacterial osteomyelitis (CNO).

The publisher regrets that this article has been temporarily removed. A replacement will appear as soon as possible in which the reason for the removal of the article will be specified, or the article will be reinstated. The full Elsevier Policy on Article Withdrawal can be found at https://www.

Systematic literature review informing the EULAR points to consider for patient education in physical activity and self-management of pain during transitional care.

Objectives: To synthesise existing evidence on transitional care programmes for young people with juvenile-onset rheumatic and musculoskeletal diseases (jRMDs), focusing on their structure and implementation, the qualitative experiences and perceptions of those involved, and the quantitative outcomes associated with these programmes. Together with additional information, these insights have informed the European Alliance of Associations for Rheumatology (EULAR) Points to Consider for patient education in physical activity and self-management of pain in jRMDs during transitional care.

Methods: A systematic literature review was conducted with a broadened scope beyond patient education in physical activity and self-management of pain to provide a comprehensive overview of transitional care in rheumatology, aiming to optimise current strategies, support evidence-based approaches, and identify areas for clinical improvement.

Lack of HLH in FMF.

Background: Macrophage activation syndrome (MAS) is a severe complication of systemic juvenile idiopathic arthritis (sJIA), driven by excessive activation of T cells and macrophages, resulting in a cytokine storm. IFN-γ and IL-18 play crucial roles, with monocyte and macrophage hyperresponsiveness to IFN-γ amplifying MAS-related inflammation. Familial Mediterranean Fever (FMF), an autosomal recessive disease, is characterized by recurrent fever episodes due to MEFV gene mutations.

Development of CARRA/PReS-endorsed consensus Core and Expanded Datasets in childhood-onset systemic lupus erythematosus for international registry-based research.

Objectives: Childhood-onset systemic lupus erythematosus (cSLE), representing 15%-20% of individuals with SLE, has been difficult to study globally due to differences between registries. This initiative, supported by Childhood Arthritis Rheumatology Research Alliance (CARRA) and Paediatric Rheumatology European Society (PReS), aims to create Core and Expanded cSLE Datasets to standardise and enhance research worldwide.

Methods: 21 international cSLE experts and 4 patients participated in a Delphi process (questionnaires, 2 topic-specific focus groups and 3 virtual consensus meetings) to create 2 standardised cSLE datasets.

Antinuclear Antibodies Staining Patterns and Titers in Juvenile Idiopathic Arthritis.

Objective: The importance of antinuclear antibodies (ANA) patterns and titers in juvenile idiopathic arthritis (JIA) patients is not clearly determined in the literature. In our study, we evaluated the ANA staining patterns and titers in JIA patients.

Methods: JIA patients were retrospectively assessed.

Differences and Similarities Between Children and Adults With Rhupus Syndrome.

Aim: In this study, we aimed to evaluate and compare the characteristics of pediatric and adult rhupus patients.

Methods: Thirty pediatric patients with rhupus syndrome and 15 adult patients with rhupus syndrome were included in this study. Similarities and differences between both groups were evaluated.

Assessing cognitive functions in non-neuropsychiatric childhood systemic lupus erythematosus: Cross-sectional study.

Objectives: Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by multisystem, including neuropsychiatric, involvement. The nervous system is affected in 20-27 % of patients within approximately two years after diagnosis. This study aimed to examine neurocognitive impairment in childhood-onset SLE (cSLE) patients before the development of any neurological, psychiatric, or cognitive manifestations.

Transition of patients with systemic lupus erythematosus from pediatric to adult-oriented rheumatology care.

Background/aim: The transition from pediatric to adult-oriented care for individuals with juvenile-onset systemic lupus erythematosus (SLE) poses significant challenges. This study aimed to assess the outcomes of transitioning patients with juvenile-onset SLE from pediatric to adult-oriented care.

Materials And Methods: Patients with juvenile-onset SLE were included in the study.

Evaluation of cardiac function using echocardiography in childhood-onset systemic lupus erythematosus patients treated with hydroxychloroquine.

Background: This study aimed to evaluate the effects of hydroxychloroquine on cardiac functions and left ventricular mass in patients with childhood-onset systemic lupus erythematosus (cSLE).

Research Design And Methods: Fifty patients with cSLE undergoing treatment with hydroxychloroquine underwent echocardiographic evaluation. All patients exhibited negative disease activity markers and were clinically in remission.

Examination of antinuclear antibody staining patterns and titers in patients with childhood-onset systemic lupus erythematosus.

Objective: Antinuclear antibodies (ANA) staining patterns can provide useful information in systemic lupus erythematosus (SLE). In our study, we examined the frequency of ANA staining patterns in disease-related features in childhood-onset SLE patients.

Methods: ANA and its staining patterns were assessed in childhood-onset SLE patients.

Mevalonate kinase deficiency: an updated clinical overview and revision of the SHARE recommendations.

Mevalonate kinase deficiency (MKD), a rare auto-inflammatory disorder, arises from mutations in the gene, disrupting isoprenoid biosynthesis, and affecting cellular processes. This comprehensive review provides an updated perspective on MKD, including its aetiology, pathogenesis, diagnostic modalities, and therapeutic strategies. Based on recent research and clinical advances, our objective is to bridge the knowledge gaps in the 2015 SHARE guidelines.

Long-term follow-up of anti-TNF treatment in adult and pediatric DADA2 patients: Insights from real-world data.

Background/purpose: Our objective was to investigate real-world outcomes and treatment strategies in individuals affected by DADA2 using over 10-year period real-life experience.

Methods: This descriptive analysis encompassed all adult/pediatric patients with DADA2 from our Vasculitis Research Center prospective database. Patients on anti-TNF therapy have been specifically examined, analyzing the treatment's duration, indications, and outcomes.

Evaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry.

Objective: We aimed to (1) evaluate the cardiac involvement, with a focus on myocarditis, in patients with Still disease included in the multicenter Autoinflammatory Disease Alliance (AIDA) Network Still disease registry; and (2) assess the predictive factors for myocarditis by deriving a clinical risk patient profile for this severe manifestation.

Methods: A multicenter observational study was established, in which consecutive patients with Still disease in the AIDA Network Still disease registry were characterized by cardiac involvement. Cardiac involvement was defined according to the presence of pericarditis, tamponade, myocarditis, and/or aseptic endocarditis.