378 results match your criteria: "Centre National de Référence des Maladies Auto-Immunes et Systémiques Rares[Affiliation]"

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.

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Distinct pathophysiological pathways support stratification of Sjögren's disease based on symptoms, clinical, and routine biological data.

Arthritis 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.

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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.

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Allogeneic umbilical cord-derived mesenchymal stromal cells as treatment for systemic lupus erythematosus: a single-centre, open-label, dose-escalation, phase 1 study.

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.

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Crystalline silica on the lung-environment interface: Impact on immunity, epithelial cells, and therapeutic perspectives for autoimmunity.

Autoimmun 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.

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[Living with… chronic atrophic polychondritis].

Rev 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).

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[Between the normal and the pathological: The concept of pre-disease applied to systemic autoimmune rheumatic diseases].

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.

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Do SMS/e-mail reminders increase influenza vaccination of rheumatoid arthritis patients under anti-TNF: a nested randomized controlled trial in the ART e-cohort.

Rheumatology (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.

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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.

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[French internal medicine is at a turning point in its history. Summary of the 2024 French Internal Medicine meeting].

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, université Paris-Cité, AP-HP, Paris, France; Inserm UMRS 1138, centre de recherche des cordeliers, université Paris-Cité, Paris, France.

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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.

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Lymphadenopathy in systemic lupus erythematosus: no microbial trigger found by shotgun metagenomics in a retrospective study on 38 patients.

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.

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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.

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Article Synopsis
  • * Researchers measured the prevalence of IgG anti-IgA in PID patients compared to healthy controls and found a low percentage of PID patients with these antibodies and hypersensitivity.
  • * The study suggests that complement activation might be a more significant factor in hypersensitivity reactions to immunoglobulin preparations, rather than just the presence of IgG anti-IgA.
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Article Synopsis
  • Onconephrology is a growing field focused on improving care for patients dealing with both cancer and kidney diseases, emphasizing the need for detailed kidney histology data.
  • A retrospective analysis of 154 cancer patients who underwent kidney biopsies showed high rates of complications like proteinuria and kidney injury, with significant histological findings such as interstitial fibrosis and tubular atrophy.
  • The most common cause of kidney issues was found to be nephrotoxicity from anticancer drugs, with many patients having multiple diagnoses, indicating the complex nature of kidney damage in cancer treatment.
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2024 update of the recommendations of the French Society of Rheumatology for the diagnosis and management of patients with rheumatoid arthritis.

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.

Article Synopsis
  • The French Society of Rheumatology updated its recommendations for managing rheumatoid arthritis (RA) based on the latest EULAR guidelines, involving a wide range of experts and patient representatives.
  • The guidelines stress the importance of shared decision-making and comprehensive management for individuals with RA or those at risk, focusing on both drug and non-drug therapies.
  • Additional emphasis is on the diagnosis and treatment of RA-related interstitial lung disease (RA-ILD), highlighting the need for collaboration between rheumatologists and pulmonologists for effective management.
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Peripheral-to-central extracorporeal corporeal membrane oxygenation switch in refractory cardiogenic shock patients: outcomes and bridging strategies.

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.

Article Synopsis
  • This study examines the effectiveness of switching patients from peripheral veno-arterial ECMO (pECMO) to central ECMO (cECMO) as a treatment strategy for refractory cardiogenic shock (rCS).
  • Out of 80 patients analyzed, only 38% were successfully bridged to recovery, heart transplantation, or a ventricle assist device, while the remaining 62% died during cECMO treatment.
  • Complications were common, with high rates of renal issues and bleeding, and the study found that myocardial infarction significantly increased the risk of in-hospital mortality.
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Article Synopsis
  • A web-based ecological momentary assessment (EMA) tool was developed to monitor symptoms in patients with Sjögren's disease (SjD), focusing on daily variability in symptoms over three months.
  • The study involved 45 participants, primarily women, who recorded their symptoms through the WebApp, allowing comparisons with traditional paper questionnaires.
  • Results showed a high level of user satisfaction (SUS score of 90) and effective symptom tracking, confirming the tool's usefulness for accurately capturing daily experiences of SjD patients.
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Immunopathogenesis of systemic lupus erythematosus: An update.

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.

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Manual versus automatic chest compression devices for cardiopulmonary resuscitation under zero gravity (The MACCC - 0G STUDY).

Resuscitation

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:

Article Synopsis
  • The study investigated the effectiveness of automatic chest compression devices (ACCDs) compared to manual CPR in a microgravity environment, specifically during parabolic flights.
  • Results showed that the LUCAS 3© device achieved the best compression depth (53.0 mm), far surpassing the other devices and manual CPR methods, which had significantly lower depths.
  • Despite the ACCDs providing decent compression rates, manual CPR offered a higher rate (115 cpm), but only LUCAS 3© met international CPR guidelines for effectiveness in microgravity scenarios.
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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).

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Treatment modalities of marginal zone lymphoma and overall survival, haematological response, and underlying Sjögren's disease activity: a multicentre, retrospective, observational study.

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:

Article Synopsis
  • Sjögren's disease is an autoimmune condition with a significant risk of developing lymphoma, but effective management strategies for this dual challenge are not well established.
  • A study was conducted involving 106 patients who had both Sjögren's disease and lymphoma, focusing on understanding treatment approaches and outcomes related to lymphoma progression and Sjögren's disease symptoms.
  • The findings highlighted that mucosa-associated lymphoid tissue lymphoma was the most common type in these patients, and specific factors were analyzed to assess their impact on patients’ prognosis and survival rates.
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[Trends in the choice of internal medicine (and clinical immunology) at the end of the national ranking examination since 2010].

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.

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[Role of university hospital doctors specialized in internal medicine in health professions training and institutions in France: Survey of the National College of Teachers of Internal Medicine (CEMI)].

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.

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