378 results match your criteria: "Centre National de Référence des Maladies Auto-Immunes et Systémiques Rares[Affiliation]"
Front Immunol
December 2024
Centre National de la Recherche Scientifique (CNRS) Unité Mixte de Recherche (UMR), Université Paris Cité, Paris, France.
Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by an overactive immune response, particularly involving excessive production of type I interferons. This overproduction is driven by the phosphorylation of IRF7, a crucial factor in interferon gene activation. Current treatments for SLE are often not very effective and can have serious side effects.
View Article and Find Full Text PDFArthritis Rheumatol
December 2024
Department of Rheumatology, Hôpital Bicêtre, Assistance Publique - Hôpitaux de Paris, Université Paris-Saclay, Le Kremlin-Bicêtre, France.
Objective: Recently, three distinct phenotypes of Sjögren's disease (SjD) patients have been described, based on cluster analysis: B-cell active with low symptoms (BALS), high systemic activity (HSA), and low systemic activity with high symptoms (LSAHS). We aimed to assess whether these clusters were associated with distinct biomarkers and the prognostic value of IFN signature.
Methods: The ASSESS cohort is a 20-year prospective cohort of SjD patients.
J Patient Rep Outcomes
December 2024
Department of Rheumatology, Hôpitaux Universitaires de Strasbourg, INSERM UMR-S 1109, Centre National de Référence des Maladies Auto-immunes Systémiques Rares (RESO), Strasbourg, France.
Objectives: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with heterogeneous clinical manifestations which significantly impacts the daily lives of patients. Herein, we aimed to (i) investigate patients' perspectives on and experience with SLE; (ii) identify meaningful aspects of health (MAHs) and concepts of interest (COIs) in SLE that could be evaluated using digital clinical measures (DCMs); and (iii) identify target DCMs for their assessment.
Methods: A mixed-methods, multistep approach was deployed for (i) exploring patients' experience with SLE through a social media listening study and focused group discussions with patients; (ii) mapping patients' experiences to define MAHs and identify COIs measurable using DCMs; (iii) selecting DCMs for the target COIs; and (iv) identifying types of wearable sensors for measuring COIs in the patients.
Lancet Rheumatol
December 2024
SITI, CHU Rennes, Etablissement Français du Sang Bretagne, Rennes, France; INSERM UMR 1236, Université Rennes, INSERM, Etablissement Français du Sang Bretagne, Rennes, France. Electronic address:
Background: Patients with systemic lupus erythematosus (SLE) with inadequate responses to standard therapies have unmet therapeutic needs. The immunomodulatory, proangiogenic, and antifibrotic properties of mesenchymal stromal cells support their use in treating patients with SLE. We aimed to assess the safety of a single intravenous infusion of allogeneic umbilical cord-derived mesenchymal stromal cells in patients with severe SLE.
View Article and Find Full Text PDFAutoimmun Rev
December 2024
Univ. Bordeaux, CNRS, ImmunoConcEpT, UMR 5164, F-33000 Bordeaux, France; CHU de Bordeaux, FHU ACRONIM, Centre national de référence des maladies auto-immunes et systémiques rares Est/Sud-Ouest (RESO), F-33000 Bordeaux, France; CHU de Bordeaux, Service de Rhumatologie, UMR 5164, F-33000 Bordeaux, France.
Crystalline silica (the most abundant form of silicon dioxide) is a natural element that is ubiquitous in the Earth's crust. Chronic personal or professional exposure has been implicated in various pathologies, including silicosis and autoimmune diseases since the early 20th century. More recently, a specific pathogenic role for crystalline silica has been identified through its impact on lung epithelial cells as well as immune cells present at this organism barrier.
View Article and Find Full Text PDFRev Prat
November 2024
Service de médecine interne 2, centre de référence des maladies auto-immunes et autoinflammatoires systémiques rares de l'adulte d'Île-de-France, Centre et Martinique, hôpital de La Pitié-Salpêtrière, AP-HP, Paris, France. Association francophone contre la polychondrite chronique atrophiante (AFPCA).
Rev Med Interne
November 2024
Service d'immuno-rhumatologie, CHU de Montpellier, Inserm U1046, CNRS UMR 9214, université de Montpellier, physiologie et médecine expérimentale du cœur et des muscles (PhyMedExp), Montpellier, France.
The incidence of systemic autoimmune diseases is constantly rising. They are chronic diseases requiring prolonged treatment, with considerable psychosocial impact. While attention to the promising results obtained with CAR-T cells in refractory patients is justified, it seems important not to overlook the opportunities for prevention based on the identification of a pre-disease state.
View Article and Find Full Text PDFRheumatology (Oxford)
November 2024
Department of Rheumatology and National Reference Center for Rare Systemic Autoimmune Diseases, AP-HP, Hôpital Bicêtre, Université Paris-Saclay, Le Kremlin Bicêtre, France.
Objectives: To evaluate the effectiveness of short message service (SMS) and/or email reminders in improving influenza vaccination coverage rates among rheumatoid arthritis (RA) patients treated with anti-TNF therapies, and to identify factors associated with vaccination.
Methods: A nested randomized controlled trial in the ART e-cohort, an ongoing French nationwide multicentre prospective cohort of RA patients treated with anti-TNF therapy. Patients were 1:1 randomized, with stratification on age.
Ann Rheum Dis
November 2024
Department of Internal Medicine, Hospital Cochin, AP-HP, Paris, France
Background: The PEXIVAS (Plasma exchange and glucocorticoids in severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis) trial showed that a reduced-dose glucocorticoid regimen (redGC) was non-inferior to a standard-dose regimen (standGC) with respect to death or end-stage kidney disease (ESKD) in patients with ANCA-associated vasculitis (AAV). However, the primary endpoint did not include disease progression or relapse, cyclophosphamide was the main induction therapy and rituximab (RTX)-treated patients tended to have a higher risk of death or ESKD with redGC. We aimed to evaluate the real-world use of redGC.
View Article and Find Full Text PDFRev Med Interne
November 2024
Service de médecine interne, centre de référence maladies auto-immunes et auto-inflammatoires systémiques rares d'Île-de-France, de l'Est et de l'Ouest, hôpital Cochin, université Paris-Cité, AP-HP, Paris, France; Inserm UMRS 1138, centre de recherche des cordeliers, université Paris-Cité, Paris, France.
Arthritis Res Ther
October 2024
Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
Objective: Patients with diffuse cutaneous systemic sclerosis (dcSSc) frequently show spontaneous improvement of skin fibrosis. Our aim was to examine whether an improvement in skin fibrosis predicts lower likelihood of visceral organ progression and better survival.
Methods: Patients from the European Scleroderma Trials and Research (EUSTAR) cohort with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, and valid mRSS at 12±3 months follow up were included.
Rheumatology (Oxford)
October 2024
Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Groupement Hospitalier Pitié-Salpêtrière, centre national de référence pour le lupus, le syndrome des anticorps anti-phospholipides et autres maladies auto-immunes rares, Service de Médecine Interne 2, Institut E3M, Paris, France.
Objectives: Lymphadenopathy is a classical manifestation of systemic lupus erythematosus (SLE) flare, occurring in approximately half of patients during the course of the disease. Lymphadenopathy in SLE is frequently associated with fever. Microbial infection may play a role in SLE onset and flares.
View Article and Find Full Text PDFLupus
December 2024
Pediatric Department, Bordeaux University Hospital, Bordeaux, France.
Introduction: Systemic Lupus Erythematosus (SLE) can be diagnosed using the 2012 criteria of the Systemic Lupus International Collaborating Clinics (SLICC) and, more recently, the 2019 criteria of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR). Hematological involvement is scored differently by these classifications. Our objective was to compare both criteria in a cohort of children with autoimmune cytopenia (AIC)-associated SLE.
View Article and Find Full Text PDFClin Rev Allergy Immunol
December 2024
Univ. Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille, France.
BMC Nephrol
October 2024
Department of Nephrology, Peupliers Private Hospital, Paris, France.
Joint Bone Spine
December 2024
Université de Montpellier, Montpellier, France; Service de rhumatologie, CHU de Montpellier, CHU Lapeyronie, Montpellier, France; Inserm U1046, CNRS UMR 9214, University of Montpellier, Physiology and Experimental Medicine of the Heart and Muscles (PhyMedExp), Montpellier, France.
Ann Intensive Care
October 2024
Service de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié-Salpêtrière, 47-83, Boulevard de l'Hôpital, Paris Cedex, 75651, France.
RMD Open
October 2024
Rheumatology Department, Centre National de Référence des Maladies Auto-Immunes Rares (CERAINOM), CHU Brest, Brest, France
Autoimmun Rev
October 2024
Service d'immunologie Clinique et de médecine interne, Hôpitaux Universitaires de Strasbourg, Centre National de Référence des Maladies Systémiques Auto-immunes Rares, Strasbourg, France.
Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease characterized by dysregulated immune responses leading to widespread inflammation and damage in various organs. Environmental factors such as infections, hormonal influences and exposure to ultraviolet light can trigger the disease in genetically predisposed individuals. Genome-wide association studies have identified over 100 susceptibility loci linked to immune regulation, interferon (IFN) signaling and antigen presentation in SLE.
View Article and Find Full Text PDFResuscitation
October 2024
Service de Médecine Intensive et Réanimation, Hôpital Brabois, CHRU Nancy, 54500 Vandœuvre-Lès-Nancy, France; Faculté de Médecine de Nancy, Université de Lorraine, INSERM UMR_S1116, Vandœuvre-Lès-Nancy, France. Electronic address:
Proteomics Clin Appl
November 2024
Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille, France.
Purpose: High throughput technologies have identified molecular patterns in colorectal cancer (CRC) cells, aiding in modeling responses to anti-cancer treatments. The different responses observed depend on the type of cancer, the tumour grade and the functional programme of the cancer cells. Recent studies suggest that the unfolded protein response (UPR), autophagy and apoptosis could be involved in treatment resistance mechanisms by interacting with the tumour microenvironment (TME).
View Article and Find Full Text PDFChest
August 2024
Service de Pneumologie, Université Sorbonne Paris Nord, Assistance Publique-Hôpitaux de Paris, Hôpital Avicenne, Bobigny, France; Université Sorbonne Paris Nord, INSERM U1272, Paris, France.
Lancet Rheumatol
October 2024
Department of Rheumatology, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Le Kremlin-Bicêtre, Paris, France; Center for Immunology of Viral Infections and Auto-Immune Diseases, INSERM UMR 1184, Université Paris-Saclay, Le Kremlin-Bicêtre, Paris, France. Electronic address:
Rev Med Interne
November 2024
Service de médecine interne, centre de référence maladies auto-immunes et auto-inflammatoires systémiques rares d'Île-de-France, de l'Est et de l'Ouest, hôpital Cochin, Assistance publique-Hôpitaux de Paris, Paris-Centre, université Paris Cité, Paris, France.
Introduction: The 2017 reform of the third cycle of medical studies (R3C) was accompanied by modifications in the formats and number of specialty diplomas. The aim of this study was to investigate the evolution of the choice of the internal medicine and clinical immunology specialty before and after 2017.
Methods: We used the median ranking and its evolution, as well as incoming and outgoing remorse rights, as markers of attractiveness.
Rev Med Interne
November 2024
Service de médecine interne, centre de référence des maladies auto-immunes et auto-inflammatoires systémiques rares d'Île de France, de l'Est et de l'Ouest, hôpital Cochin, AP-HP, université Paris-Cité, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France. Electronic address:
Rationale: On the occasion of the General stage meeting of Internal Medicine, the National College of Internal Medicine Teachers (CEMI) conducted a survey on teaching activity among all French university hospital (HU) internal medicine specialists.
Method: The survey was carried out in September 2023 by sending an email to 101 hospital practitioners university professors (PU-PH) and 18 hospital practitioners assistant professors (MCU-PH) of internal medicine in subsection 53-01 of the National council of universities (CNU), as well as to the 11 HU internists working in immunology (subsection 47-01) or therapeutics (subsection 48-04).
Results: Seventy-three HUs (56.