Infants with Kawasaki Disease (KD) have a higher risk of incomplete presentations, IVIG resistance, and coronary artery lesions (CALs). IL-1 plays a key role in the pathogenesis, highlighting its potential as a therapeutic target.

To describe the clinical characteristics, diagnostic findings, treatment, and outcomes of patients with Juvenile Idiopathic Arthritis (JIA) whose initial manifestation was cervical spine arthritis.

Sexual and reproductive health (SRH) guidelines exist for adults with rheumatic diseases (RDs), but not for their adolescent counterparts. SRH discussions are regarded as important areas of discussion by pediatric rheumatology providers. However, adolescents with RDs have unique needs that are not well elucidated. The purpose of this study was to explore SRH knowledge and information sources among female adolescents with systemic lupus erythematosus (SLE), undifferentiated connective tissue disease (UCTD), mixed connective tissue disease (MCTD), or an overlap disease given their increased risk for SRH-related adverse outcomes.

Establishing a predictive model using clinical indicators for the early identification of JIA-U.

Juvenile fibromyalgia (JFM) is a chronic pain condition affecting children and adolescents, often accompanied by emotional distress. Evidence suggests that autonomic dysregulation, as measured by reduced heart rate variability (HRV), may play a key role in symptom expression. This study aimed to explore clinical, emotional, and physiological differences between children with and without JFM.

Juvenile dermatomyositis (JDM), the most common inflammatory myopathy of childhood, is a complex autoimmune condition with significant disease burden and variable treatment responses. While conventional immunosuppression remains standard, advances in immunopathology are revealing new therapeutic and biomarker opportunities.

Despite the call for inclusion of routine mental health screening in pediatric rheumatology practice, the practical aspects of implementation in real world clinical settings have not yet been demonstrated. The aims of this study were: (1) to determine the acceptability and practicality of mental health screening in patients with Juvenile Idiopathic Arthritis (JIA) during routine visits at a pediatric hospital multidisciplinary clinic; (2) to understand the frequency and severity of anxiety and depressive symptoms within this sample.

In patients with juvenile idiopathic arthritis (JIA), the approach to discontinuing biological treatments after a period of remission remains exploratory. We aimed to identify one or more predictors of relapse after the discontinuation of biologic/targeted synthetic disease-modifying anti-rheumatic drugs.

Familial Mediterranean Fever (FMF) is an autoinflammatory disease characterized by a wide clinical variability among individuals, indicating that both genetic and environmental factors influence disease progression. Although the Mediterranean Diet (MD) is recognized for its anti-inflammatory properties, its relationship with FMF severity remains uninvestigated. This study aims to explore the association between MD adherence and FMF severity, while considering individuals' genetic background, lifestyle, and comorbid health conditions.

Hemophagocytic lymphohistiocytosis (HLH) is an excessive immune activation syndrome. The genetic studies on every patient diagnosed with HLH recently became a standard of care. The likelihood of identifying a gene mutation is highest in the youngest patients.

Canakinumab is a fully human monoclonal antibody that primarily targets interleukin-1β (IL-1β) and is primarily indicated for systemic juvenile idiopathic arthritis (sJIA). Since it is predominantly used in children aged 0-16 years, exploring its safety in real-world settings is of paramount importance.

Juvenile idiopathic arthritis (JIA) is a chronic inflammatory condition that frequently persists into adulthood, posing long-term challenges in disease control and quality of life. However, clinical management during the transitional and young adult phases remains insufficiently characterized, especially in comparison with adult-onset rheumatoid arthritis (RA). This study aimed to compare disease activity, medication use, and treatment practices between patients with oligoarticular/polyarticular JIA and those with RA, focusing on individuals aged 16-30 years.

Neutrophils contribute to the pathogenesis of many autoimmune diseases. While the neutrophil to lymphocyte ratio (NLR) is associated with disease activity in adult dermatomyositis, its clinical utility in juvenile dermatomyositis (JDM) is unknown.

Autosomal recessive Aicardi-Goutières syndrome (AGS) and autosomal dominant familial chilblain lupus (FCL) are rare type I interferonopathies that can both result from loss-of-function variants in the gene, which encodes a DNA exonuclease. Although phenotypic variability is well recognized in -related disorders, intrafamilial phenotypic discordance is seldom seen.

Kawasaki disease (KD) in infants presents unique clinical challenges, including higher rates of intravenous immunoglobulin (IVIG) resistance and coronary complications. This study aimed to evaluate the predictive value of inflammatory markers derived from complete blood count, specifically neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte ratio (MLR), and pan-immune inflammation value (PIV), for IVIG resistance in infants under 12 months with KD.

Takayasu arteritis (TA) is a granulomatous inflammatory disease of unknown etiology that affects the aorta and its branches, including the coronary and pulmonary arteries. Diagnosis in pediatric age is late because the initial manifestations are nonspecific.

Coronary artery lesion (CAL) in children with Kawasaki disease (KD) is a major acquired heart disease. This study aims to investigate the C-reactive protein to high-density lipoprotein cholesterol (CRP/HDL-C) ratio as a predictor of CAL and intravenous immunoglobulin (IVIG) resistance in KD.

Since the molecular characterization of periodic fever syndromes led to the concept of autoinflammation, the pace of scientific advancement in this field has been dramatic. Here, we review many of the most impactful new discoveries in autoinflammation, as presented at the 2024 Pediatric Rheumatology European Society Congress. This includes new genes and diseases, such as SHARPIN mutations and dominant-negative mutations in OTULIN as causes of disorders of ubiquitination, PMVK mutations as potential causes of a mevalonate kinase deficiency mimic, and ARF1 and REXO2 as causes of interferonopathy. Several new molecular mechanisms and mutations were also reported for older diseases including coatomer protein complex subunit alpha (COPA) syndrome, Aicardi-Goutières syndrome, PLCG2-associated immune dysregulation (PLAID), and NLRP3-AID. Finally, molecular and omics studies of STING1-associated vasculopathy with onset in infancy (SAVI) and haploinsufficiency of A20 (HA20) contributed to advances in underlying autoinflammatory biology.

Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease with highly heterogenous clinical manifestations and severity. Herpes zoster (HZ) is a viral disease caused by reactivation of varicella-zoster virus which remains dormant in the dorsal root sensory ganglia after a previous varicella infection. There is limited information on the association between HZ and childhood-onset SLE (cSLE). This study aimed to determine the risk factors for HZ in patients diagnosed with cSLE.