Update on streptococcal-associated rheumatic disease.

Purpose Of Review: This review provides a comprehensive perspective on poststreptococcal rheumatic manifestations in pediatric patients by integrating recent updates and a literature review, with particular focus on poststreptococcal reactive arthritis and acute rheumatic fever.

Recent Findings: Poststreptococcal reactive arthritis presents with a unique clinical profile, distinguishing it from other poststreptococcal conditions in pediatric patients, especially acute rheumatic fever. Recent updates underscore the importance of diligent monitoring and management to mitigate potential cardiac complications, despite the relatively low incidence of carditis following poststreptococcal reactive arthritis.

Atypical Presentations of Pediatric-acquired Thrombotic Thrombocytopenic Purpura.

Background: Immune thrombotic thrombocytopenic purpura (iTTP) in children is a rare, severe thrombotic microangiopathy. This condition is characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia due to reduced activity of the von Willebrand factor-cleaving protease ADAMTS13.

Methods: A retrospective case series evaluating data collected from the medical files of 4 children diagnosed with iTTP.

Obstacles in Early Diagnosis of Children With Juvenile Idiopathic Arthritis: A Nationwide Israeli Retrospective Study.

Objective: Characterization of the stages that patients with juvenile idiopathic arthritis (JIA) pass until they are diagnosed, and analysis of the different causes that lead to a delay in JIA diagnosis in Israel.

Methods: This is a retrospective cohort study conducted in 8 pediatric rheumatology centers in Israel. All patients diagnosed with JIA between October 2017 and October 2019 were included in the study.

Measuring the knowledge of juvenile idiopathic arthritis among Israeli paediatricians and paediatric orthopaedic surgeons: what needs to be improved?

Objectives: Approximately 1 child in 1,000 is affected by juvenile idiopathic arthritis (JIA). Persistent, undiagnosed JIA with high disease activity interferes with daily life and carries a risk of irreversible physical and psychosocial damage. Due to its relative rarity, primary care physicians often do not recognise it.

Effectiveness of the BNT162b2 mRNA COVID-19 vaccine among adolescents with juvenile-onset inflammatory rheumatic diseases.

Objectives: The effectiveness of the BNT162b2 mRNA COVID-19 vaccine for adolescents with juvenile-onset inflammatory or immune rheumatic diseases (IRDs) is unknown. Several studies have suggested attenuated immunogenicity in patients with IRD. This study evaluated the effectiveness of the BNT162b2 mRNA COVID-19 vaccine in preventing COVID-19 infection in adolescents with juvenile-onset IRD compared with controls without immune rheumatic disease.

Is fibrodysplasia ossificans progressiva an interleukin-1 driven auto-inflammatory syndrome?

Background: Fibrodysplasia ossificans progressiva (FOP) is the most catastrophic form of heterotopic ossification, due to ongoing intracellular signaling through the bone morphogenic protein pathway. The paroxysmal appearance of inflammatory lumps and elevated inflammatory markers during flares, suggest that FOP is an auto-inflammatory disease. Based on evidence, demonstrating a role for interleukin-1β (IL-1β) in other forms of heterotopic ossification, we hypothesized that treating FOP patients with anti-IL-1 agents could help lower the rate of FOP paroxysms and/or limit the symptoms and residual lesions.

[A REPORT ON THE PRACTICALITIES OF THE JOINT FELLOWSHIP IN PEDIATRIC RHEUMATOLOGY IN ISRAEL].

Background: The field of pediatric rheumatology was only recently recognized in Israel as a sub-specialty. To enable a fellowship program in this field comprising mainly uncommon diseases, the Meir and Shaare Zedek medical centers were authorized to implement a joint fellowship program, in which the trainees split their time between the two medical centers.

Objectives: This article presents the unique experience as a trainee in the joint fellowship program in pediatric rheumatology, while relating to the different characteristics of these centers, patient populations and variable disease exposure.

Juvenile polymyositis associated with ureteral necrosis: a diagnostic and therapeutic dilemma-case report and review of the literature.

We describe an 11-year-old girl, diagnosed with juvenile polymyositis (JPM), who developed right ureteral obstruction secondary to necrosis. We emphasize the dilemmas regarding optimal timing for surgical intervention and medical treatment. Vascular involvement, which could be a part of juvenile dermatomyositis, may also be a feature of JPM.

Canakinumab investigated for treating familial Mediterranean fever.

Introduction: Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory syndrome. The treatment of choice is colchicine. However, ~40% of patients are only partial responders and 5-10% are non-responders.

Cutaneous polyarteritis nodosa successfully treated with topical diflucortolone valerate: a case report & review of the literature.

Cutaneous Polyarteritis Nodosa (cPAN) was first described in 1931. cPAN is considered a rare disease, its true incidence is unknown. The age of onset is diverse.