Brigitte Bader‐Meunier
A5046909004- Autoimmune and Inflammatory Disorders Research
- Systemic Lupus Erythematosus Research
- Inflammasome and immune disorders
- Immunodeficiency and Autoimmune Disorders
- Inflammatory Myopathies and Dermatomyositis
- Eosinophilic Disorders and Syndromes
- Adolescent and Pediatric Healthcare
- Erythrocyte Function and Pathophysiology
- Blood disorders and treatments
- Blood groups and transfusion
- interferon and immune responses
- Kawasaki Disease and Coronary Complications
- Renal Diseases and Glomerulopathies
- Platelet Disorders and Treatments
- Hemoglobinopathies and Related Disorders
- Acute Lymphoblastic Leukemia research
- Hematological disorders and diagnostics
- Diabetes and associated disorders
- Immune Cell Function and Interaction
- Parvovirus B19 Infection Studies
- COVID-19 Clinical Research Studies
- T-cell and B-cell Immunology
- Osteomyelitis and Bone Disorders Research
- Liver Diseases and Immunity
- Vasculitis and related conditions
Inserm
2016-2025
Assistance Publique – Hôpitaux de Paris
2016-2025
Université Paris Cité
2015-2025
Hôpital Necker-Enfants Malades
2016-2025
Institut des Maladies Génétiques Imagine
2016-2025
Hôpital Robert-Debré
2006-2025
Sorbonne Paris Cité
2013-2024
Hospices Civils de Lyon
2010-2024
Institut Necker Enfants Malades
2004-2024
Centre National pour la Recherche Scientifique et Technique (CNRST)
2023
Current data suggest that COVID-19 is less frequent in children, with a milder course. However, over the past weeks, an increase number of children presenting to hospitals greater Paris region phenotype resembling Kawasaki disease (KD) has led alert by French national health authorities. Multicentre compilation patients KD since April 2020, associated detection severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ('Kawa-COVID-19'). A historical cohort 'classical' served as...
Innate immunity to viral infection involves induction of the type I IFN response; however, dysfunctional regulation this pathway leads inappropriate inflammation. Here, we evaluated a nonconsanguineous family mixed European descent, with 4 members affected by systemic inflammatory and autoimmune conditions, including lupus, variable clinical expression. We identified germline dominant gain-of-function mutation in TMEM173, which encodes stimulator gene (STING), individuals. STING is key...
A number of open-label studies have suggested the potential benefit rituximab (RTX) in systemic lupus erythematosus (SLE). However, 2 recent randomized controlled trials (RCTs) RTX, primary end points were not met. We undertook this study to evaluate safety and efficacy RTX off-trial patients with SLE seen regular clinical practice.We analyzed prospective data from French AutoImmunity Rituximab (AIR) registry, which includes on autoimmune disorders treated RTX.One hundred thirty-six received...
Abstract Objective To assess the efficacy of etanercept in patients with juvenile idiopathic arthritis (JIA), and to tolerance these etanercept. Methods All JIA active chronic polyarthritis, who were first treated between November 1999 June 2001 18 French centers because poor response or intolerance methotrexate, included this open‐label, prospective, multicenter study. A standardized questionnaire was sent treating physicians. We assessed validated international core‐set score for activity...
Type I interferons (IFNs) are essential mediators of antiviral responses. These cytokines have been implicated in the pathogenesis autoimmunity, most notably systemic lupus erythematosus (SLE), diabetes mellitus, and dermatomyositis, as well monogenic type interferonopathies. Despite a fundamental role health disease, direct quantification IFNs has challenging. Using single-molecule array (Simoa) digital ELISA technology, we recorded attomolar concentrations IFNα healthy donors, viral...
Autosomal dominant deficiency of signal transducer and activator transcription 3 (STAT3) is the main genetic etiology hyper-immunoglobulin (Ig) E syndrome. We documented molecular, cellular, clinical features 60 patients with heterozygous STAT3 mutations from 47 kindreds followed in France. identified 11 known 13 new STAT3. Low levels interleukin (IL)-6–dependent phosphorylation nuclear translocation (or accumulation) were observed Epstein-Barr virus-transformed B lymphocytes (EBV-B cells)...
<h3>Objective</h3> To evaluate the genetic findings, demographic features and clinical presentation of tumour necrosis factor receptor-associated autoinflammatory syndrome (TRAPS) in patients from Eurofever/EUROTRAPS international registry. <h3>Methods</h3> A web-based registry collected retrospective data on with <i>TNFRSF1A</i> sequence variants inflammatory symptoms. Participating hospitals included paediatric rheumatology centres adult a specific interest diseases. Cases were...
End of April 2020, French clinicians observed an increase in cases presenting with paediatric inflammatory multisystem syndrome (PIMS). Nationwide surveillance was set up and demonstrated temporospatial association the coronavirus disease (COVID-19) epidemic for 156 reported as at 17 May: 108 were classified confirmed (n = 79), probable 16) or possible 13) post-COVID-19 PIMS cases. A continuum clinical features from Kawasaki-like to myocarditis observed, requiring intensive care 67%
Necrotizing autoimmune myopathy (NAM) is a group of acquired myopathies characterized by prominent myofiber necrosis with little or no muscle inflammation. Recently, researchers identified autoantibodies (aAb) against 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) in patients NAM, especially statin-exposed patients. Here we report what to our knowledge the first European cohort NAM.The serum 206 suspicion NAM was tested for detection anti-HMGCR aAb using an addressable laser bead...
IgA vasculitis (IgAV, formerly known as Henoch-Schönlein purpura) is the most common cause of systemic in childhood. To date, there are no internationally agreed, evidence-based guidelines concerning appropriate diagnosis and treatment IgAV children. Accordingly, regimens differ widely. The European initiative SHARE (Single Hub Access point for paediatric Rheumatology Europe) aims to optimize care children with rheumatic diseases. aim therefore was provide agreed consensus recommendations...
Objective To document more fully the characteristics of chronic recurrent multifocal osteomyelitis (CRMO) in pediatric patients, to collect data on outcomes and management disease, define prognostic factors. Methods One hundred seventy‐eight patients were included (123 female 55 male patients), with a mean ± SD age at diagnosis 10.9 2.9 years. Inclusion criteria CRMO, evidence least one lesion osteitis confirmed by imaging, development syndrome before 18 Results Longitudinal clinical imaging...
<h3>Objective</h3> To evaluate genetic, demographic and clinical features in patients with cryopyrin-associated periodic syndrome (CAPS) from the Eurofever Registry, a focus on genotype-phenotype correlations predictive disease severity markers. <h3>Methods</h3> A web-based registry retrospectively collected data CAPS. Experts independently validated all cases. Patients carrying <i>NLRP3</i> variants germline-mutation-negative were included. <h3>Results</h3> 136 analysed. The median age at...
Microbial nucleic acid recognition serves as the major stimulus to an antiviral response, implying a requirement limit misrepresentation of self acids non-self and induction autoinflammation. By systematic screening using panel interferon-stimulated genes we identify two siblings singleton variably demonstrating severe neonatal anemia, membranoproliferative glomerulonephritis, liver fibrosis, deforming arthropathy increased anti-DNA antibodies. In both families biallelic mutations in DNASE2,...
Childhood-onset systemic lupus erythematosus (cSLE) is a rare, multisystem and potentially life-threatening autoimmune disorder with significant associated morbidity. Evidence-based guidelines are sparse management often based on clinical expertise. SHARE (Single Hub Access point for paediatric Rheumatology in Europe) was launched to optimise disseminate regimens children young adults rheumatic diseases like cSLE. Here, we provide evidence-based recommendations diagnosis treatment of In view...
There is little known about the impact of SARS-CoV-2 on patients with inflammatory rheumatic and musculoskeletal diseases (iRMD). We examined epidemiological characteristics associated severe disease, then death. also compared mortality between hospitalised for COVID-19 without iRMD.Individuals suspected iRMD-COVID-19 were included in this French cohort. Logistic regression models adjusted age sex used to estimate ORs 95% CIs COVID-19. The most significant clinically relevant factors...