Immunology and Biochemistry for Rare Inflammatory Diseases (IBRID)
Team Composition
Staff
Cyril Gitiaux, PU-PH, HDR
Armelle Melet, MCU
Mélanie Poinsot, IE
Sabrina Guermah, ADT
PhD Students
Thomas Moreau
Margaux Cescato
Diego Bletry
Scientific projects and goals:
-
Research Projects
Pathophysiology of juvenile dermatomyositis.
Juvenile dermatomyositis is a rare auto immune disease of unknown origin characterized by sustained overproduction of type I interferons. We are more specifically interested in the mechanisms responsible for this type I interferon overproduction/signature, and its impact on disease progression and severity. In line with this questioning, we are involved in clinical trials evaluating the benefit of inhibitors of interferon signalling in these patients.
Genotype / phenotype association in genetic auto-inflammatory diseases.
Numerous genes have been associated with inflammatory diseases. Interestingly, mutations in distinct domains of a same protein can be associated with distinct diseases, or variable manifestation of a same disease. We are interested in the molecular and cellular alterations resulting from mutations in different domains of a same protein, and its association with clinical features.
Pathophysiology of adult idiopathic inflammatory and autoimmune diseases.
The pathophysiology of most non-genetic inflammatory and autoimmune diseases is still poorly understood. We aim to identify deregulations of specific pathways within professional immune cells in patients suffering from idiopathic inflammatory or autoimmune diseases. This work should provide new insights into the mechanisms leading to the onset of the diseases and help identify new therapeutic targets or markers that predict the response to treatments
Main collaborators:
Dr Brigitte Bader Meunier – Necker Hospital
Dr Benjamin Chaigne – Cochin Hospital
Dr Julien Dairou – UMR8601
Dr Darragh Duffy – Pasteur Institut
Pr Sophie Georgin-Lavialle – Tenon Hospital
Dr Laurent Le Corre – UMR8601
Dr Isabelle Melki – Trousseau Hospital
Dr Nicolas Michalski – Institut de L’audition / Pasteur Institut
Pr Pierre Quartier – Necker Hospital
Pr Angèle Soria – Tenon Hospital
Immunology and Biochemistry for Rare Inflammatory Diseases (IBRID)
Team composition
PhD Students
Thomas Moreau
Margaux Cescato
Diego Bletry
Scientific projects and goals:
- Research Projects
Pathophysiology of juvenile dermatomyositis.
Juvenile dermatomyositis is a rare auto immune disease of unknown origin characterized by sustained overproduction of type I interferons. We are more specifically interested in the mechanisms responsible for this type I interferon overproduction/signature, and its impact on disease progression and severity. In line with this questioning, we are involved in clinical trials evaluating the benefit of inhibitors of interferon signalling in these patients.
Genotype / phenotype association in genetic auto-inflammatory diseases.
Numerous genes have been associated with inflammatory diseases. Interestingly, mutations in distinct domains of a same protein can be associated with distinct diseases, or variable manifestation of a same disease. We are interested in the molecular and cellular alterations resulting from mutations in different domains of a same protein, and its association with clinical features.
Pathophysiology of adult idiopathic inflammatory and autoimmune diseases.
The pathophysiology of most non-genetic inflammatory and autoimmune diseases is still poorly understood. We aim to identify deregulations of specific pathways within professional immune cells in patients suffering from idiopathic inflammatory or autoimmune diseases. This work should provide new insights into the mechanisms leading to the onset of the diseases and help identify new therapeutic targets or markers that predict the response to treatments
Main collaborators:
Dr Brigitte Bader Meunier – Necker Hospital
Dr Benjamin Chaigne – Cochin Hospital
Dr Julien Dairou – UMR8601
Dr Darragh Duffy – Pasteur Institut
Pr Sophie Georgin-Lavialle – Tenon Hospital
Dr Laurent Le Corre – UMR8601
Dr Isabelle Melki – Robert Debré Hospital
Dr Nicolas Michalski – Institut de L’audition / Pasteur Institut
Pr Pierre Quartier – Necker Hospital
Pr Angèle Soria – Tenon Hospital
Main Publications
Maillet F*, Schmidt C*, et al: Increased mortality in diffuse systemic sclerosis patients with high circulating IFNα levels. J Invest Derm. 2024
Fayand A*, Cescato M* et al:Pathogenic variants in the NLRP3 LRR domain at position 861 are responsible for a boost-dependent atypical CAPS phenotype. J Allergy Clin Immunol. 2023
Fayand A, et al: Successful treatment of JAK1 associated inflammatory disease. J Allergy Clin Immunol. 2023
Rodero M, et al: Onset and Relapse of Juvenile Dermatomyositis Following Asymptomatic SARS-CoV-2 Infection. J Clin Immunol 2022
Le Voyer T et al, JAK inhibitors are effective in a subset of patients with juvenile dermatomyositis: a monocentric retrospective study. Rheumatology 2021.
All Publications
Increased Mortality in Patients with Diffuse Systemic Sclerosis with High Circulating IFNα Levels. J Invest Derm 2024
Implication of the LRR Domain in the Regulation and Activation of the NLRP3 Inflammasome. Cells 2024
Bader Meunier B et al, Osteonecrosis in patients with juvenile dermatomyositis: is it associated with anti-MDA5 autoantibody? Rheumatology. 2023
Targeting the chemokine receptor CXCR4 with histamine analog to reduce inflammation in juvenile arthritis. Front Immunol 2023
Dabbak I, Rodero MP et al, Efficacy and tolerance of corticosteroids and methotrexate in patients with juvenile dermatomyositis: a retrospective cohort study. Rheumatology 2022
Onset and Relapse of Juvenile Dermatomyositis Following Asymptomatic SARS-CoV-2 Infection. J Clin Immunol. 2021
Sci Immunol. 2021
X-linked recessive TLR7 deficiency in ~1% of men under 60 years old with life-threatening COVID-19.Sci Immunol. 2021
Autoantibodies neutralizing type I IFNs are present in ~ 4% of uninfected individuals over 70 years old and account for ~ 20% of COVID-19 deaths.
Gitiauc C, […], Bader Meunier B* and Rodero MP* ,Inhibition of IFNa secretion in cells from patients with JDM under TBK1 treatment revealed by Single Molecular Assay technology . Rheumatology, 2019