Publications by authors named "Marine Fouillet-Desjonqueres"
Objectives: Childhood-onset systemic lupus erythematosus (cSLE) is a rare autoimmune disease with significant morbidity. Although B cell-depleting agents show promise for refractory cSLE, there is limited research on rituximab therapy in children. This study aimed to retrospectively assess the indications, efficacy, and safety of RTX in cSLE, using data from the Juvenile Inflammatory Rheumatism (JIR) cohort database.
View Article and Find Full Text PDFBackground: Osteogenesis Imperfecta (OI) is a rare genetic disorder characterized by bone fragility and susceptibility to fractures. No curative treatment currently exists, and limited data are available on the effects of adapted physical activity (APA). This study evaluates the impact of APA on bone health, physical function, respiratory function, and quality of life in pediatric children with OI.
View Article and Find Full Text PDFObjectives: - Janus Kinase inhibitors (JAKi) are a new class of drugs available for pediatric rheumatic diseases. This study aimed to describe the safety and effectiveness of JAKi in these diseases, with a focus on longitudinal interferon-stimulated genes (ISG) assessment.
Methods: - We present a single-center retrospective study of children with refractory pediatric rheumatic diseases including connective tissue diseases, monogenic type I interferonopathies or juvenile idiopathic arthritis, receiving JAKi.
Article Synopsis
- Still's disease (SD) is an autoinflammatory condition with varying forms, and this study aimed to compare adult-onset SD (AOSD) and systemic juvenile idiopathic arthritis (sJIA) while identifying prognosis.
- Researchers analyzed data from 238 patients, finding that 64% had AOSD, with symptoms like sore throat and muscle pain being more common in this group, as well as higher levels of liver enzymes and inflammatory markers.
- Key findings indicated that symptoms like fever and skin rash are linked to better recovery chances, while high lactate dehydrogenase levels suggest a poorer prognosis, supporting the idea that SD can evolve into chronic arthritis if not treated promptly.
Article Synopsis
- Distinguishing macrophage activation syndrome (MAS) from a simple flare of Still's disease (SD) is complex; researchers aimed to identify clinical features and outcomes related to MAS and its predictive factors in SD patients.
- In a study involving 206 SD patients, 20 (9.7%) experienced MAS, which presented more often with symptoms like hepatomegaly and neurological issues, alongside higher rates of cytopenias, liver problems, and abnormal coagulation.
- High serum ferritin levels, specifically above 3500 μg/L, demonstrated strong predictive value for MAS development, with specific factors like neurological symptoms and coagulopathy aiding in early detection.
Canakinumab in patients with systemic juvenile idiopathic arthritis and active systemic features: results from the 5-year long-term extension of the phase III pivotal trials.
Ann Rheum Dis
December 2018
Objectives: To evaluate the long-term efficacy and safety of canakinumab in patients with active systemic juvenile idiopathic arthritis (JIA).
Methods: Patients (2-19 years) entered two phase III studies and continued in the long-term extension (LTE) study. Efficacy assessments were performed every 3 months, including adapted JIA American College of Rheumatology (aJIA-ACR) criteria, Juvenile Arthritis Disease Activity Score (JADAS) and ACR clinical remission on medication criteria (CR).
Takayasu arteritis (TA) is a large vessel vasculitis that usually affects young female patients during the second and third decades of life, but has been reported in children as young as 24 months of age. Aim of this report was to describe four children (two girls) with TA, as well as summarizing main published studies. The mean age at presentation of our cases was 11 years (range 8-15).
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