Julien Haroche's research while affiliated with Hôpitaux Universitaires La Pitié salpêtrière - Charles Foix and other places

Publications (543)

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Erdheim–Chester disease (ECD) is a rare condition with underestimated neurological involvement. Mild psychiatric symptoms such as mood swings have been rarely described in the clinical spectrum of neuro-ECD. We here describe the first patient with psychiatric manifestations of delirium revealing ECD with neurological involvement with favorable evol...
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Aseptic abscess (AA) syndrome is a rare type of inflammatory disorder involving polymorphonuclear neutrophils (PMNs), often associated with inflammatory bowel disease (IBD). This study sought to describe the clinical characteristics and evolution of this syndrome in a large cohort. We included all patients included in the French AA syndrome registe...
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Introduction: Data on severe heart valve disease (HVD), including Libman-sacks endocarditis, associated with systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS) requiring valvular surgery are scarce. Method: Observational retrospective multicentric analysis of 23 adults with SLE and/or APS and HVD, between 1996 and 2019 and...
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Introduction La sarcoïdose est une maladie granulomateuse multisystémique de cause inconnue. La prévalence de l’atteinte osseuse est estimée entre 3 et 30 %. Elle est symptomatique dans environ la moitié des cas. La tomographie par émission de positrons (TEP) au 18F-fluorodéoxyglucose (FDG) associée à la tomodensitométrie (TDM) est un examen fréque...
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e19085 Background: Erdheim-Chester disease (ECD) is a rare L-group histiocytosis. Orbital involvement is found in a third of cases, but few data are available concerning the radiological features of ECD-related orbital disease (ECD-ROD). Our aim was to characterize the initial radiological phenotype and outcome of patients with ECD-ROD. Methods: In...
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Introduction Le syndrome de fuite capillaire idiopathique (SFCI) ou maladie de Clarkson est une pathologie rare définie par la récurrence d’épisodes de fuite capillaire en présence d’une gammapathie monoclonale. Elle entre dans le cadre des gammapathies monoclonales de signification clinique. Le tableau caractéristique est celui d’un choc hypovolém...
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Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasm characterized by proliferation of tumor histiocytes that involves multiple organs including central nervous system. The physiopathologic process underlying degenerative neuro-LCH (i.e., DN-LCH) remains imperfectly settled. Since the main clinical features of DN-LCH are cere...
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Introduction Les histiocytoses forment un groupe hétérogène de pathologies caractérisées par la prolifération et l’accumulation de cellules dendritiques et macrophagiques au sein de tissus causant des dysfonctions d’organe très variables. Les histiocytoses sont classées en 5 groupes (selon des critères anatomopathologiques/moléculaires) avec un phé...
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Introduction L’efficacité et la tolérance des traitements d’entretien de la rémission au cours du lupus systémique (LS) restent à définir. Au terme d’une surveillance d’un an, nous avons montré que les patients atteints d’un LS cliniquement inactif et traités au long cours par 5 mg de prednisone sont moins susceptibles de présenter une poussée s’il...
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Introduction La maladie d’Erdheim-Chester (MEC) est une histiocytose rare du groupe L. L’atteinte orbitaire (AO) de la MEC (MEC-AO) est retrouvée dans un tiers des cas – avec notamment l’exophtalmie qui constitue un des signes classiques de la maladie – mais peu de données sont disponibles concernant les caractéristiques radiologiques de MEC-AO. P...
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Introduction Le lupus systémique est une maladie auto-immune rare chronique. Les manifestations neuropsychiatriques (NP) ont une prévalence de 30 à 40 % chez les patients lupiques. Ces manifestations sont graves, associées à une morbidité et une mortalité accrues. Les échanges plasmatiques (EP) sont cités dans les recommandations de traitements des...
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Objectives: Systemic rheumatic diseases (SRDs) are a group of inflammatory disorders that can need intensive care unit (ICU) admission during a flare-up, requiring administration of immunosuppressants. We undertook this study to determine the frequency, outcome, and occurrence associated factors of infections in flare-up SRD patients receiving imm...
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Erdheim-Chester disease (ECD) is a rare L-group histiocytosis. Orbital involvement is found in a third of cases, but few data are available concerning the radiological features of ECD-related orbital disease (ECD-ROD). Our aim was to characterize the initial radiological phenotype and outcome of patients with ECD-ROD. Initial and follow-up orbital...
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Erdheim–Chester disease (ECD) is characterized by excessive production and accumulation of histiocytes within multiple tissues and organs. ECD patients harbor recurrent mutations of genes associated with the RAS/RAF/MEK/ERK signaling pathway, particularly, the BRAF V600E mutation. Following our previous finding that miR-15a-5p is the most prominent...
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Langerhans cell histiocytosis (LCH) can affect children and adults with wide variety of clinical manifestations, including unifocal, single-system multifocal, single-system pulmonary (smoking-associated), or multisystem disease. The existing paradigms in the management of LCH in adults are mostly derived from the pediatric literature. Over the last...
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La maladie d'Erdheim Chester (MEC) est une maladie rare appartenant aux histiocytosis du groupe L. L'atteinte orbitaire(AO) survient dans 1/3 des cas mais les descriptions radiologiques sont rares. Notre objectif était de décrire les aspects radiologiques de l'AO liée à la MEC (AO-MEC) de la plus large cohorte mondiale de MEC suivie au centre natio...
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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....
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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...
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(1) Background: The Antiphospholipid Syndrome (APS) is a systemic autoimmune disorder characterized by arterial and/or venous thrombosis, pregnancy morbidity and raised titers of antiphospholipid antibodies. Cerebral vein thrombosis (CVT) is a rare form of cerebrovascular accident and an uncommon APS manifestation; the information in the literature...
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Introduction Les monocytes jouent un rôle important dans la pathogénèse des histiocytoses [1], [2]. Peu de donnés sont disponibles concernant l’étude de leur phénotype dans les histiocytoses ; et les différences avec d’autres syndromes myéloprolifératifs ou maladies inflammatoires. Matériels et méthodes Le phénotype des sous-populations des monocy...
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The historic term ‘histiocytosis’ meaning ‘tissue cell’ is used as a unifying concept for diseases characterized by pathogenic myeloid cells that share histological features with macrophages or dendritic cells. These cells may arise from the embryonic yolk sac, fetal liver or postnatal bone marrow. Prior classification schemes align disease designa...
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Résumé La maladie d’Erdheim-Chester (EC) est une maladie rare faisant partie du « groupe L » de la classification révisée des histiocytoses de 2016. Les manifestations les plus évocatrices sont l’atteinte bilatérale des os longs, la fibrose péri-rénale ou péri-artérielle et le diabète insipide, mais cette maladie peut atteindre isolément ou simulta...
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Introduction Le syndrome des anticorps antiphospholipides (SAPL) est une maladie auto-immune rare, caractérisée par des thromboses de sites variés et/ou des événements obstétricaux, associés à la présence d’anticorps antiphospholipides persistants. L’expression clinique du SAPL est hétérogène et malgré des études antérieures montrant des associatio...
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Introduction Le syndrome des anticorps anti-phospholipides (SAPL) est défini par un événement thrombotique artériel ou veineux et/ou des événements obstétricaux (fausses couches spontanées, insuffisance placentaire), associés à la présence persistante d’anticorps antiphospholipides. Parmi les événements artériels, les atteintes cérébro-vasculaires...
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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...
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Introduction Les manifestions psychiatriques ne sont pas décrites dans le spectre des atteintes neurologiques de la maladie d’Erdheim-Chester [1] ; en particulier en tant que première manifestation de la maladie [2]. Observation Une femme caucasienne de 81 ans souffrait d’un délire associé à des hallucinations. Elle avait pour antécédents une mala...
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Background: Erdheim-Chester disease (ECD) is a non-Langerhans cell histiocytic disorder that almost exclusively affects adults. Reports of pediatric-onset ECD are only anecdotal. Additionally, a comprehensive clinical and molecular characterization of pediatric ECD is lacking. Methods: All prevalent and incident cases followed at three ECD referral...
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ALK-positive histiocytosis is a rare subtype of histiocytic neoplasm first described in 2008 in three infants with multisystemic disease involving the liver and hematopoietic system. This entity has subsequently been documented in case reports and series to occupy a wider clinicopathologic spectrum with recurrent KIF5B-ALK fusions. The full clinico...
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Erdheim–Chester disease is a rare histiocytosis characterized by iconic features associated with compatible histology. Most patients have somatic mutations in the MAP-kinase pathway gene, and the mutations occur in CD14+ monocytes. Differentiation of the myeloid lineage plays a central role in the pathogenesis of histiocytosis. Monocytes are myeloi...
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Inorganic antigens may contribute to paediatric sarcoidosis. Thirty-six patients matched with 36 healthy controls as well as a group of 21 sickle-cell disease (SCD) controls answered an environmental questionnaire. Patients’ indirect exposure to inorganic particles, through coresidents’ occupations, was higher than in healthy and SCD controls (medi...
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Erdheim–Chester disease (ECD) is a rare, systemic, non-Langerhans cell histiocytosis neoplasm, which is characterized by the infiltration of CD63+ CD1a- histiocytes in multiple tissues. The BRAFV600E mutation is frequently present in individuals with ECD and has been detected in hematopoietic stem cells and immune cells from the myeloid and systemi...
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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...
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Background SARS-CoV-2 infection in children is generally milder than in adults, yet a proportion of cases result in hyperinflammatory conditions often including myocarditis. Methods To better understand these cases, we applied a multi-parametric approach to the study of blood cells of 56 children hospitalized with suspicion of SARS-CoV-2 infection...
Preprint
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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...
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Trained immunity (TI) is a pro-inflammatory program induced in monocyte/macrophages upon sensing of specific pathogens and characterized by immunometabolic and epigenetic changes enhancing cytokine production. Maladaptive activation of TI (i.e., in the absence of infection) might result in detrimental inflammation and disease development; however,...
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Introduction L’implication de l’IFN-alpha (IFNα) dans le lupus systémique (LS) est bien démontrée. Celles de l’IFN-béta (IFNβ), -gamma (IFNγ) et -lambda (λ) restent à caractériser. L’objectif de cette étude était d’évaluer l’expression de ces différents IFNs dans le LS en utilisant une technique ultrasensible de dosage. Patients et méthodes Les sé...
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Introduction Les valvulopathies associées au lupus érythémateux systémique (LES) et/ou au syndrome des anti-phospholipides (SAPL), dont l’endocardite de Libman-Sachs, sont des complications rares de ces deux pathologies. L’objectif de cette étude était de décrire les caractéristiques cliniques, biologiques, écho-cardiographiques et évolutives des p...
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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...
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Background Compared to secondary antiphospholipid syndrome (APS), little is known on intima-media thickness (IMT) in primary APS. Previous reports suggest that intima-media thickness may be associated with anti-cardiolipin antibodies, anti-β2-glycoprotein-1 antibodies, and with stroke in APS¹. Objectives To assess clinical significance of intima-m...
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Immune thrombocytopenia (ITP) is a rare autoimmune-mediated condition characterized by isolated thrombocytopenia (<100 G/L) after exclusion of other causes. Mostly primary, it is associated with hematological malignancy, autoimmune disorders, or infection in 20% of patients. It is exceptionally described in patients with histiocytosis, mostly in ch...
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Histiocytoses are heterogeneous hematopoietic diseases characterized by the accumulation of CD68(+) cells with various admixed inflammatory infiltrates. The identification of the pivotal role of the mitogen-activated protein kinase (MAPK) pathway has opened new avenues of research and therapeutic approaches. We review the neurologic manifestations...
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Objective: Peritoneal or mesenteric tumours may correspond to several tumour types or tumour-like conditions, some of them being represented by histiocytosis. This rare condition often poses diagnostic difficulties that can lead to important time delay in targeted therapies. Our aim was to describe main features of histiocytoses with mesenteric lo...
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Histiocytoses constitute a heterogeneous group of rare disorders, characterised by infiltration of almost any organ by myeloid cells with diverse macrophage or dendritic cell phenotypes. Histiocytoses can start at any age. Diagnosis is based on histology in combination with appropriate clinical and radiological findings. The low incidence and broad...
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Purpose: To describe the posterior ophthalmic manifestations of catastrophic antiphospholipid syndrome (CAPS). Methods: Retrospective case series of patients presenting with CAPS and posterior segment ocular manifestations. The main outcomes were the type of posterior segment manifestations at CAPS diagnosis, specifically retinal vascular occlus...
Preprint
SARS-CoV-2 infection in children is generally milder than in adults, yet a proportion of cases result in hyperinflammatory conditions often including myocarditis. To better understand these cases, we applied a multi-parametric approach to the study of blood cells of 56 children hospitalized with suspicion of SARS-CoV-2 infection. The most severe fo...
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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...
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Introduction Les histiocytoses sont un groupe d’affections hétérogène, parmi lesquelles on distingue les maladies comportant une composante clonale constante associée à une mutation somatique sur la voie des MAPkinases (histiocytoses du groupe L comportant la maladie d’Erdheim-Chester, MEC et l’histiocytose Langerhansienne, HL), et les histiocytose...
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Introduction Le lénalidomide a montré des résultats intéressants dans des petites séries de cas dans le traitement du lupus érythémateux cutané (LEC) sévère. Cependant, les données d’efficacité et de tolérance à long terme sont limitées en particulier en cas de lupus systémique (LES) associé. Cette étude a évalué l’efficacité et la tolérance du lén...
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Introduction Le diagnostic de purpura thrombopénique est retenu en cas de thrombopénie isolée, après exclusion des autres causes de thrombopénie. Le purpura thrombopénique peut être primitif ou associé à certaines pathologies (infections, maladies auto-immunes, déficits immunitaires) mais n’est pas décrit chez les patients atteints d’histiocytoses...
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The pathogenesis of histiocytic neoplasms is driven by mutations activating the MAPK/ERK pathway, but little is known about the transcriptional and post-transcriptional alterations involved in these neoplasms. We analyzed microRNA (miRNA) expression in plasma samples and tissue biopsies of Erdheim–Chester disease (ECD) and Langerhans cell histiocyt...
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Background Sarcoidosis is a rare disease of unknown cause with wide heterogeneity in clinical features and outcomes. We aimed to explore sarcoidosis phenotypes and their clinical relevance with particular attention to extrapulmonary subgroups. Patients and methods The EpiSarc (Epidemiology of Sarcoidosis) study is a French retrospective multicente...
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AIM To describe MRI features, including diffusion-weighted imaging (DWI), magnetic resonance spectroscopy (MRS), and perfusion-weighted imaging (PWI), of intra-axial tumour-like presentations of four different subtypes of histiocytosis. MATERIAL AND METHODS The brain MRI findings of 23 patients with histologically proven histiocytosis were reviewe...
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Erdheim-Chester disease (ECD) is a clonal hematopoietic disorder characterized by the accumulation of foamy histiocytes within organs, in particular frequent retroperitoneal involvement, and a high frequency of BRAFV600E mutations. Although ECD is not commonly recognized to have overt peripheral blood (PB) or bone marrow (BM) disease, we recently i...
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Aim: To present the first case series of patients with Langerhans cell histiocytosis (LCH) also affected by Crohn's disease (CD), both of which are granulomatous diseases, and in LCH investigate the role of interleukin (IL)-23, which is a well-described disease mediator in CD. Methods: A case series of three patients with LCH and CD were describ...
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Objective: Central nervous system (CNS) involvement in Erdheim-Chester disease (ECD) leads to significant morbidity and mortality. To assess CNS manifestations in a French cohort of 253 ECD patients, we determined clinical characteristics and outcomes, including those under targeted therapies. Methods: This was a retrospective longitudinal study...
Preprint
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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...
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Résumé Les vascularites cérébrales sont un groupe hétérogène d'affections neurologiques de diagnostic difficile. Elles peuvent être primitives ou associées à une autre affection neurologique ou multi-systémique, principalement auto-immune ou infectieuse. Les causes infectieuses sont fréquentes et de mécanismes physiopathologiques variés : (1) liées...
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(1) Background: Pediatric sarcoidosis is a rare and mostly severe disease. Very few pediatric series with a prolonged follow-up are reported. We aimed to evaluate the evolution of pediatric sarcoidosis in adulthood. (2) Material and methods: Patients over 18-years-old with a pediatric-onset sarcoidosis (≤15-year-old) who completed at least a three-...
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Objectives Patients with autoimmune encephalitis (AE) are likely to exhibit an acute onset of severe psychiatric features, including psychosis and/or catatonia. Based on the high prevalence of catatonia in AE and our clinical experience, we hypothesized that catatonia might be a marker of severity requiring more aggressive treatment approaches. Me...
Preprint
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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...
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Background Current data suggest that COVID-19 is less frequent in children, with a milder course. However, over the past weeks, an increase in the number of children presenting to hospitals in the greater Paris region with a phenotype resembling Kawasaki disease (KD) has led to an alert by the French national health authorities. Methods Multicentr...