Brigitte Bader‐Meunier
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View article: Juvenile-onset mixed connective tissue disease: A multicenter retrospective cohort study
Juvenile-onset mixed connective tissue disease: A multicenter retrospective cohort study Open
jMCTD share the same clinical characteristics as aMCTD patients, but less frequently have puffy fingers. Outcomes appear more favorable in jMCTD than aMCTD, with higher remission rates, albeit at the cost of more intensive treatment.
Chronic inflammatory arthritis in 22q11.2 deletion (DiGeorge) syndrome: a multicentric study Open
Background 22q11 deletion syndrome (22q11DS) is a genetic disorder caused by a deletion at chromosome 22q11.2. Chronic arthritis may be occasionally observed in these, if this manifestion represents a concomitant association of two disease…
IFN-α levels correlate with muscle disease activity only in juvenile dermatomyositis patients with anti-MDA5+ autoantibodies Open
Objective This study aimed to establish the role of myositis-specific antibodies (MSAs) in the association between type I interferon (IFN-I) plasma levels and disease activity in juvenile dermatomyositis (JDM). Methods We prospectively obt…
View article: Muscle Spatial Transcriptomic Reveals Heterogeneous Profiles in Juvenile Dermatomyositis and Persistence of Abnormal Signature After Remission
Muscle Spatial Transcriptomic Reveals Heterogeneous Profiles in Juvenile Dermatomyositis and Persistence of Abnormal Signature After Remission Open
This study aimed to investigate the spatial heterogeneity of molecular signature in the muscle of juvenile dermatomyositis (JDM) patients before and after treatment. Unsupervised reference-free deconvolution of spatial transcriptomics and …
NAP1 switches from an activator to a limiter of interferon induction by trapping TBK1 in condensates Open
Summary TBK1 kinase is a central regulator of type I IFN production. Upon activation of the IFN-β induction pathway, TBK1-adaptor proteins (NAP1, SINTBAD, TANK) form condensates with liquid properties. We showed that NAP1 condensates conce…
View article: Population pharmacokinetic modelling of prednisolone in systemic lupus erythematosus patients: Analysis of exposure and disease activity
Population pharmacokinetic modelling of prednisolone in systemic lupus erythematosus patients: Analysis of exposure and disease activity Open
Aims Prednisone is a widely used glucocorticoid in the treatment of lupus, although its dosing is often determined empirically. Prednisolone, the active metabolite of prednisone, is found in its free form in the serum. The goal of this stu…
Targeting interferon responses in juvenile dermatomyositis: Siglec-1 as an in vitro biomarker for JAK inhibitor efficacy Open
Objectives For IFN-driven diseases, such as juvenile dermatomyositis (JDM), there is a critical need for targeted therapies. We aimed to develop an in vitro model, using Siglec-1 as read-out, to evaluate inhibition of IFN-mediated response…
View article: Muscle spatial transcriptomic reveals heterogeneous profiles in untreated juvenile dermatomyositis and the persistence of pathological signature after remission
Muscle spatial transcriptomic reveals heterogeneous profiles in untreated juvenile dermatomyositis and the persistence of pathological signature after remission Open
This study aimed to investigate the spatial heterogeneity of molecular signature in the muscle of juvenile dermatomyositis (JDM) patients before and after treatment in comparison to healthy paediatric muscle tissue. Unsupervised reference-…
Approach to Janus kinase inhibition for juvenile dermatomyositis among CARRA and PReS providers Open
Objectives Janus kinase inhibition (JAKi) has been proposed as a treatment for idiopathic inflammatory myopathies to target increased interferon signalling. Predominantly retrospective reports have demonstrated effectiveness of JAKi in ref…
Systemic Inflammatory Diseases in Children With Sickle Cell Disease: A French Multicenter Observational Study on Diagnostic and Therapeutic Issues Open
Background Systemic inflammatory diseases (SIDs) have been reported in patients with sickle cell disease (SCD), but clinical data in children are scarce. Objectives To identify clinical and laboratory features at diagnosis of SID in childr…
PFMG2025–integrating genomic medicine into the national healthcare system in France Open
As of December the 31st 2023, €239M have been invested by the French government.
Efficacy of Ruxolitinib in Severe Pediatric Intra‐Alveolar Hemorrhages Unrelated to <i>COPA</i> Mutations Open
International audience
Performance of serum IL-18 levels for the follow-up of patients with familial Mediterranean fever Open
International audience
Autoimmune and Inflammatory Diseases in Children with Sickle Cell Disease: A French Multicenter Observational Study on Diagnostic and Therapeutic Issues Open
Autoimmune and inflammatory diseases (AIIDs) encompass a spectrum of systemic or organ-specific conditions, wherein the immune system is compromised due to a loss of self-tolerance. AIIDs have been reported in both adults and children with…
Long-term outcomes of childhood-onset systemic lupus erythematosus Open
Objective Data on the long-term outcome of patients with childhood-onset SLE (cSLE) are scarce. Aims of this study were to describe the long-term outcomes of cSLE and to identify factors associated with the development of damage and persis…
View article: Gain-of-function human <i>UNC93B1</i> variants cause systemic lupus erythematosus and chilblain lupus
Gain-of-function human <i>UNC93B1</i> variants cause systemic lupus erythematosus and chilblain lupus Open
UNC93B1 is a transmembrane domain protein mediating the signaling of endosomal Toll-like receptors (TLRs). We report five families harboring rare missense substitutions (I317M, G325C, L330R, R466S, and R525P) in UNC93B1 causing systemic lu…
Immuno-inflammatory involvement of adipose tissue in children Open
Adipose tissue is a highly immunologically active tissue that can be involved in many inflammatory diseases. In this presentation, only adipose tissue disorders associated with inflammatory diseases in children will be described, with the …
View article: Functional diversity of <i>NLRP3</i> gain-of-function mutants associated with CAPS autoinflammation
Functional diversity of <i>NLRP3</i> gain-of-function mutants associated with CAPS autoinflammation Open
NLRP3-associated autoinflammatory disease is a heterogenous group of monogenic conditions caused by NLRP3 gain-of-function mutations. The poor functional characterization of most NLRP3 variants hinders diagnosis despite efficient anti-IL-1…
View article: TIF1-gamma IgG2 isotype is not associated with malignancy in juvenile dermatomyositis patients
TIF1-gamma IgG2 isotype is not associated with malignancy in juvenile dermatomyositis patients Open
International audience
IFN-β plasmatic levels are best predictors of muscle disease activity than IFN-α in a longitudinal cohort of patients with juvenile dermatomyositis Open
This study was conducted to identify the contribution of myositis-specific antibodies (MSAs) on the correlation between type I interferon (IFN-I) plasma levels and disease activity in juvenile dermatomyositis (JDM) patients. We prospective…