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Introduction
Immune regulation
Cell therapy
Transplantation
Tissue regeneration
Publications
Publications (224)
Langerhans cell Histiocytosis (LCH) and Erdheim-Chester disease (ECD) are clonal myeloid disorders, associated with MAP-Kinase activating mutations and an increased risk of neurodegeneration. Surprisingly, we found pervasive PU.1+ microglia mutant clones across the brain of LCH and ECD patients with and without neurological symptoms, associated wit...
Introduction
Increasing evidence has shown that coronavirus disease 19 (COVID-19) severity is driven by a dysregulated immunological response. Previous studies have demonstrated that natural killer (NK) cell dysfunction underpins severe illness in COVID-19 patients, but have lacked an in-depth analysis of NK cell markers as a driver of death in the...
Objective
Recent studies have demonstrated the feasibility and favorable long-term results of tracheobronchial replacement using stented cryopreserved aortic allografts. We propose to investigate the outcomes of this emerging technique in the subgroup of patients with extensive tracheal cancer.
Methods
This study was based on 13 patients with prim...
CD4+ regulatory T (Treg) cells play an important role in maintaining immune homeostasis. Although these cells were initially studied as a homogenous cohort, we now know that they have unprecedented underlying heterogeneity. This heterogeneity is reflected in their phenotype and functions. As human Treg subpopulations are very small in numbers, it i...
Objective
To help identify homogeneous subgroups among patients with anti-polymyositis-scleroderma-antibodies (PM-Scl-Abs) positive auto-immune diseases regardless of diagnostic classifications.
Methods
This multicentric (four hospitals) retrospective study collected all consecutive patients (from 2011 to 2021) with positive testing for anti-PM-Sc...
Erdheim-Chester disease (ECD) is a rare histiocytosis, considered to be an inflammatory myeloid neoplasm. Tropism for specific involvements of the disease remains unexplained. Vascular endothelial growth factor-A (VEGF) is implicated in cancer pathophysiology and mutations of the RAS oncogene have been shown to induce the upregulation of VEGF gene...
Objectives
Type-I interferons (IFNs-I) have potent antiviral effects. IFNs-I are also overproduced in patients with systemic lupus erythematosus (SLE). Autoantibodies (AAbs) neutralising IFN-α, IFN-β and/or IFN-ω subtypes are strong determinants of hypoxemic COVID-19 pneumonia, but their impact on inflammation remains unknown.
Methods
We retrospec...
Over the past 25 years, we have demonstrated the feasibility of airway bioengineering using stented aortic matrices experimentally then in a first-in-human trial (n=13). The present TRITON-01 study analyzed all the patients who had airway replacement at our center to confirm that this innovative approach can be now used as usual care. For each pati...
Background
Adults who have been infected with SARS-CoV-2 can develop a multisystem inflammatory syndrome (MIS-A), including fulminant myocarditis. Yet, several patients fail to meet MIS-A criteria, suggesting the existence of distinct phenotypes in fulminant COVID-19–related myocarditis.
Objectives
This study sought to compare the characteristics...
Purpose:
Epidemiologic studies have documented lower rates of active smokers compared to former or non-smokers in symptomatic patients affected by coronavirus disease 2019 (COVID-19). We assessed the efficacy and safety of nicotine administered by a transdermal patch in critically ill patients with COVID-19 pneumonia.
Methods:
In this multicentr...
7059
Background: Erdheim-Chester disease (ECD) is a rare histiocytosis characterized by tissue infiltration of CD68 ⁺ , CD1a ⁻ histiocytes, derived from cells of the mononuclear phagocyte system harboring recurrent mutations in the MAPK-signaling pathway. Vascular endothelial growth factor-A, also referred as VEGF, is a major regulator of angiogene...
The capacity of pre-existing immunity to human common coronaviruses (HCoV) to cross-protect against de novo COVID-19is yet unknown. In this work, we studied the sera of 175 COVID-19 patients, 76 healthy donors and 3 intravenous immunoglobulins (IVIG) batches. We found that most COVID-19 patients developed anti-SARS-CoV-2 IgG antibodies before IgM....
Background: Identification of prognostic factors in COVID-19 remains a global challenge. The role of smoking is still controversial.
Methods: PCR-positive in- and outpatients with symptomatic COVID-19 from a large French University hospital were systematically interviewed for their smoking status, use of e-cigarette, and nicotinic substitutes. The...
Introduction
Les anticorps anti-PM-Scl peuvent être associés aux myopathies inflammatoires, à la sclérodermie systémique (ScS), aux connectivites mixtes ; toutes pouvant donner des pneumopathies interstitielles diffuses (PID). Notre objectif était de : (1) déterminer les facteurs associés à la présence d’une PID et décrire les caractéristiques des...
Introduction
La vaccination s’est imposée comme le principal atout de la lutte contre la pandémie COVID. Pourtant, les patients atteints de maladies auto-immunes ont été exclus des principaux essais thérapeutiques ayant permis de valider la tolérance et l’efficacité de la vaccination contre le SARS-CoV2. Nous avons évalué l’activité de la maladie e...
Objectives
Our aim was to evaluate systemic lupus erythematosus (SLE) disease activity and SARS-CoV-2-specific immune responses after BNT162b2 vaccination.
Methods
In this prospective study, disease activity and clinical assessments were recorded from the first dose of vaccine until day 15 after the second dose in 126 patients with SLE. SARS-CoV-2...
Background
Lymphopenia and the neutrophil/lymphocyte ratio may have prognostic value in COVID-19 severity.
Objective
We investigated neutrophil subsets and functions in blood and bronchoalveolar lavage (BAL) of COVID-19 patients on the basis of patients’ clinical characteristics.
Methods
We used a multiparametric cytometry profiling based to matu...
Rationale:
The importance of the adaptative T cell response in the control and resolution of viral infection has been well-established. However, the nature of T cell-mediated viral control mechanisms in life-threatening stages of COVID-19 has yet to be determined.
Objective:
The aim of the present study was to determine the function and phenotyp...
Objectives: Our aims were to evaluate Systemic Lupus Erythematosus (SLE) disease activity and SARS-CoV-2 specific immune responses after BNT162b2 vaccination.
Methods: In this prospective study, disease activity and clinical assessments were recorded from the first dose of vaccine, until day 15 after the second dose in 126 SLE patients. SARS-CoV-2...
Introduction
L’épidémie actuelle de maladie à coronavirus 2019 (COVID-19) peut toucher tous les patients atteints de maladies auto-immunes et systémiques. Les patients souffrant d’un lupus systémique (LS) ont des facteurs de risque de développer des formes sévères de COVID-19 en raison des comorbidités fréquemment associées au LS (insuffisance réna...
Résumé
Introduction
Les auto-anticorps aident le clinicien à classer les maladies auto-immunes systémiques en sous-groupes homogènes selon le phénotype clinique et le pronostic associé. Les anticorps anti-PM-Scl, très rarement observés, ont été décrits au cours de différentes connectivites. En particulier, ils ont été associés aux myopathies infla...
Anecdotal evidence rapidly accumulated during March 2020 from sites around the world that sudden hyposmia and hypogeusia are significant symptoms associated with the SARS-CoV-2 pandemic. Our objective was to describe the prevalence of hyposmia and hypogeusia and compare it in hospitalized and non-hospitalized COVID-19 patients to evaluate an associ...
Humoral immune responses are typically characterized by primary IgM antibody responses followed by secondary antibody responses associated with immune memory and comprised of of IgG, IgA and IgE. Here we measured acute humoral responses to SARS-CoV-2, including the frequency of antibody-secreting cells and the presence of SARS-CoV-2-specific neutra...
Introduction
Un dogme en immunologie stipule que la réponse anticorps IgM précède les réponses IgG et IgA. Le nouveau coronavirus SARS-CoV-2 (syndrome respiratoire aigu sévère coronavirus 2), agent pathogène à l’origine de la maladie à coronavirus 19 (COVID-19), induit une réponse anticorps spécifique IgG, IgM et IgA. Les IgA anti-SARS-CoV-2 ont ce...
Introduction
Les maladies du spectre des neuromyélites optiques (neuromyelitis optica spectrum disorder, NMOSD) sont des maladies démyélinisantes auto-immunes du système nerveux central. Elles se manifestent par des épisodes récidivants de myélites et de névrite optique (NO). Le plus souvent, elles sont associées à la présence d’anticorps anti-aqua...
Rational
Lymphopenia and neutrophil/lymphocyte ratio may have prognostic value in coronavirus disease 2019 (COVID-19) severity.
Objective
We sought to investigate the representation of neutrophil subsets in severe and critical COVID-19 patients based on Intensive Care Units (ICU) and non-ICU admission.
Methods
We developed a multi-parametric neut...
Regulatory T (Treg) cells expressing the FOXP3 transcription factor are presently under investigation by many teams globally as a cellular therapy to induce tolerance in transplantation. This is primarily due to their immunosuppressive and homeostatic functions. Depending on the type of allograft, Treg cells will need to infiltrate and function in...
Introduction
Alors que la pandémie de COVID-19 reste encore active, l’identification de facteurs pronostiques de la maladie reste un enjeu important. Parmi ceux-ci, le tabagisme a un rôle qui reste controversé.
Nous avons donc voulu vérifier si le taux de fumeur chez les patients COVID-19 était différent de celui de la population française.
Matéri...
Introduction
Au cours du mois de mars 2020, de nombreux cas d’hyposmie et hypogueusie soudaines associées au SARS-CoV-2 ont été signalés partout dans le monde. Notre objectif était de décrire la prévalence de l’hyposmie et de l’hypogueusie et de la comparer chez les patients COVID-19 hospitalisés et non hospitalisés pour évaluer l’association de ce...
It is currently unknown whether acquired immunity to common alpha- and beta-coronaviruses provides cross-protection against SARS-CoV-2. In this study, we found that certain patient sera and intravenous immunoglobulins (IVIG) collected prior to the COVID-19 outbreak were cross-reactive to SARS-CoV-2 full-length Spike, S2 domain, and nucleoprotein. H...
We read with great interest the comment by Suarez‐Perez et al. on our article.[1] We share their concerns regarding the need for a cautious interpretation of antiphospholipid antibodies (aPLA) positivity in patients with coronary virus disease 2019 (COVID‐19). Herein, we would like to add further insights in the discussion.
Coronavirus disease 2019 (COVID‐19) is associated with both severe systemic inflammation and a prothrombotic state, as reflected by significant increases in fibrinogen and D‐dimers levels that have been associated with poor prognosis and high rates of severe pulmonary embolism. A recent report suggested a role for antiphospholipid antibodies (aPLA)...
Background: Identification of prognostic factors in COVID-19 remains a global challenge. The role of smoking is still controversial.
Objective: To evaluate the rate of daily smokers in patients with COVID-19.
Methods: COVID-19 in-and outpatients from a large French university hospital were systematically interviewed for their smoking status, use of...
A major dogma in immunology has it that the IgM antibody response precedes secondary memory responses built on the production of IgG, IgA and, occasionaly, IgE. Here, we measured acute humoral responses to SARS-CoV-2, including the frequency of antibody-secreting cells and the presence of specific, neutralizing, antibodies in serum and broncho-alve...
Objectives
The predominance of extramuscular manifestations (e.g., skin rash, arthralgia, interstitial lung disease [ILD]) as well as the low frequency of muscle signs in anti–melanoma differentiation-associated gene 5 antibody–positive (anti-MDA5+) dermatomyositis caused us to question the term myositis-specific antibody for the anti-MDA5 antibody...
Background
The understanding of Erdheim-Chester Disease (ECD) pathogenesis has been greatly improved these past few years with the discovery of activating MAPK pathway mutations in most of ECD patients. However, the inflammatory phenotype of ECD remains widely unknown.
Objectives
We aimed to explore the inflammatory phenotype of Erdheim-Chester di...
Importance: As the pandemic of COVID-19 is still under progression, identification of prognostic factors remains a global challenge. The role of smoking has been suggested among the disease risk factors, although it is highly controversial. Objective: To evaluate whether the rate of daily smokers in patients with COVID-19 was different to that in t...
SARS-CoV-2 epidemics raises a considerable issue of public health at the planetary scale. There is a pressing urgency to find treatments based upon currently available scientific knowledge. Therefore, we tentatively propose a hypothesis which hopefully might ultimately help saving lives. Based on the current scientific literature and on new epidemi...
Importance: As the pandemic of COVID-19 is still under progression, identification of prognostic factors remains a global challenge. The role of cigarette smoking has been suggested among the disease’s epidemiological risk factors, although it is highly controversial. Objective: To evaluate the correlation of daily smoking with the susceptibility t...
SARS-CoV-2 epidemics raises a considerable issue of public health at the planetary scale. There is a pressing urgency to find treatments based upon currently available scientific knowledge. Therefore, we tentatively propose a hypothesis which hopefully might ultimately help saving lives. Based on the current scientific literature and on new epidemi...
Importance: As the pandemic of COVID-19 is still under progression, identification of prognostic factors remains a global challenge. The role of cigarette smoking has been suggested among the disease’s epidemiological risk factors, although it is highly controversial. Objective: To evaluate the correlation of daily smoking with the susceptibility t...
Importance: As the pandemic of COVID-19 is still under progression, identification of prognostic factors is a global challenge. Among epidemiological risk factors, the role of smoking, to date, is unclear. Objective: To accurately evaluate a possible correlation of daily smoking with an increased susceptibility to SARS-CoV-2 infection. Participants...
Background: Hashimoto's thyroiditis (HT) and Graves' disease (GD) are autoimmune thyroid disorders (AITDs). These conditions have been associated to abnormalities in circulating regulatory T cells (Tregs). We postulated that immune perturbations could be more pronounced at the thyroid tissue level.
Methods: The phenotype of PBMCs and immune cells i...
The induction of graft tolerance remains the holy grail of transplantation. This is important as chronic allograft dysfunction and the side effects of immunosuppression regimens place a major burden on the lives of transplant patients and their healthcare systems. This has mandated the need to understand the immunobiology of graft rejection and ide...
Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAVs) are autoimmune diseases in which pathogenic autoantibodies recognizing either MPO or PR3 proteins play a key role. Molecular mimicry through bacterial antigens, the presentation of peptides complementary to PR3, and the presentation of MPO and PR3 proteins through NETs can...
Objectives
To compare the efficacy to prevent flares of maintenance versus withdrawal of 5 mg/day prednisone in systemic lupus erythematosus (SLE) patients with clinically quiescent disease.
Methods
A monocentric, 12-month, superiority, open-label, randomised (1:1) controlled trial was conducted with 61 patients continuing 5 mg/day prednisone and...
Introduction
L’implication du virus Epstein-Barr (EBV) est fortement suspectée dans la physiopathologie du lupus systémique (LS). L’EBV est un herpes virus ubiquitaire dont la cible cellulaire principale est le lymphocyte B. Il induit aussi la production d’interféron-alpha par les cellules dendritiques plasmacytoïdes. Les profils sérologiques EBV e...
Introduction
Le syndrome des anti-synthétases (SAS) est une myopathie inflammatoire associée aux anticorps anti-synthétases, dont les complications systémiques graves sont difficilement prévisibles. Le développement de facteurs pronostiques est essentiel. L’objectif de cette étude était d’évaluer le titre de l’anticorps anti-Jo1, chef de file des a...
Objectives:
JDM and juvenile overlap myositis represent heterogeneous subtypes of juvenile idiopathic inflammatory myopathy (JIIM). Chronic evolution can occur in up to 60% of cases, and morbidity/mortality is substantial. We aimed to describe the clinical, biological, histological and type I IFN status in JIIM associated with anti-melanoma differ...
Context - Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by tissue infiltration of CD68 (+), CD1a (-) histiocytes in various organs. It has been classified as a myeloid neoplasia since the discovery of the RAS-RAF-MEK-ERK pathway alterations. Angiogenesis displays an important role in neoplastic and inflamma...
Objectives
Maintenance of remission has become central in the management of systemic lupus erythematosus (SLE). The importance of interferon-alpha (IFN-α) in the pathogenesis of SLE notwithstanding, its expression in remission has been poorly studied as yet. To study its expression in remission and its prognostic value in the prediction of a diseas...
Objectives:
Interstitial lung disease (ILD) is a common feature of mixed connective tissue disease. However, many patients do not meet the criteria for mixed connective tissue disease and thus may be diagnosed as interstitial pneumonia with autoimmune features. The aim of this study was to characterize ILD associated with anti-ribonucleoprotein (R...
Background:
Renal biopsy is the cornerstone of systemic lupus erythematosus (SLE) nephritis and antiphospholipid syndrome (APS) nephropathy management. However, transcutaneous renal biopsy (TCRB) is hampered by the antithrombotic treatment frequently prescribed for those diseases. Transjugular renal biopsy (TJRB) offers an attractive alternative f...
Background
Remission and relapses prevention are the main objectives of the treatment of Systemic Lupus Erythematosus (SLE). Interferon alpha (IFNα) is a key cytokine in SLE, but its usefulness for the definition of remission and for the prediction of flare has not been validated in clinical practice.
Objectives
To study the association between se...
Objectives
Infliximab is increasingly used to treat neurosarcoidosis. We aimed to determine the efficacy and tolerance of an infliximab biosimilar for treating neurosarcoidosis.
Methods
We conducted a retrospective single-center study to describe the efficacy, safety and immunogenicity of an infliximab biosimilar in neurosarcoidosis patients. We c...
Objective
No simple or standardized assay is available to quantify interferon‐α (IFNα) in routine clinical practice. Single‐molecule array (Simoa) digital enzyme‐linked immunosorbent assay (ELISA) technology enables direct IFNα quantification at attomolar (femtogram per milliliter [fg/ml]) concentrations. This study was undertaken to assess IFNα di...
CD4⁺ T Regulatory cells expressing the FOXP3 transcription factor (Treg cells) are instrumental for the prevention of autoimmune diseases. There is increasing evidence that the human T regulatory population is highly heterogeneous in phenotype and function. Numerous studies conducted in human autoimmune diseases have shown that Treg cells are impai...
Introduction
Une pneumopathie interstitielle diffuse (PID) rapidement évolutive de pronostic sombre peut révéler une dermatomyosite amyopathique avec anticorps anti-MDA5. Les signes extra-thoraciques peuvent être très discrets et les tests immunologiques actuels sont longs, parfois difficiles à interpréter et non quantitatifs. La mise en évidence r...
Glomeruloid haemangioma (GH) is considered a specific marker of POEMS syndrome, despite some published GH cases unrelated to POEMS syndrome. To present two cases with GH and atypical presentations of Erdheim-Chester disease (ECD) or POEMS syndrome, as well as a retrospective monocentric study of histologically-confirmed GH. Clinical, biological and...
Introduction
La Polychondrite Atrophiante (PCA) est une maladie rare. L’atteinte trachéo-bronchique est l’une des atteintes les plus graves de cette maladie. L’évolution de cette atteinte à moyen et long terme sous traitement est peu décrite.
Patients et méthodes
Il s’agit d’une étude rétrospective monocentrique incluant les patients atteints :.
–...
Introduction
Le lupus systémique (LS) est une maladie auto-immune chronique touchant divers organes. L’obtention d’une rémission et la prévention des rechutes font partie des objectifs principaux du traitement de cette maladie. À ce jour, les cliniciens ne disposent d’aucun marqueur biologique fiable pour prédire d poussée de LS. L’interféron alpha...
Introduction
La maladie d’Erdheim-Chester (MEC) est une histiocytose non langerhansienne rare, caractérisée par une infiltration tissulaire d’histiocytes CD68(+), CD1a(−). Elle est considérée comme une néoplasie myéloïde depuis la découverte des altérations de la voie RAS-RAF-MEK-ERK. L’histiocytose langerhansienne, appartenant également aux histio...
Background:
Commensals induce local IgA responses essential to the induction of tolerance to gut microbiota, but it remains unclear whether antimicrobiota responses remain confined to the gut.
Objective:
The aim of this study was to investigate systemic and intestinal responses against the whole microbiota under homeostatic conditions and in the...
A novel technology, photonic ring immunoassay (PRI), for detecting 12 autoantibodies simultaneously in whole blood in less than 15 minutes was evaluated by comparing results from 235 clinically diagnosed patients with standard laboratory tests. The overall agreement was greater than 91% for 10 of the 12 assays, with positive percent agreement great...
RAW DATA.
The file contains all the data obtained with the Maverick instruments and in the clincal lab and the corresponding diagnosis.
(XLSX)
