A5027640760- Inflammatory Myopathies and Dermatomyositis
- Eosinophilic Disorders and Syndromes
- Chronic Lymphocytic Leukemia Research
- Skin Diseases and Diabetes
- Muscle Physiology and Disorders
- Immunodeficiency and Autoimmune Disorders
- Cancer Immunotherapy and Biomarkers
- Parkinson's Disease and Spinal Disorders
- Peripheral Neuropathies and Disorders
- Muscle and Compartmental Disorders
- Systemic Sclerosis and Related Diseases
- Genetic Neurodegenerative Diseases
- Pneumocystis jirovecii pneumonia detection and treatment
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Viral Infections and Immunology Research
- Monoclonal and Polyclonal Antibodies Research
- Myasthenia Gravis and Thymoma
- Celiac Disease Research and Management
- CAR-T cell therapy research
- Neurogenetic and Muscular Disorders Research
- T-cell and B-cell Immunology
- HIV Research and Treatment
- Immune Cell Function and Interaction
- Lysosomal Storage Disorders Research
- COVID-19 Clinical Research Studies
Pitié-Salpêtrière Hospital
2016-2025
Inserm
2016-2025
Sorbonne Université
2016-2025
Centre de Recherche en Myologie
2016-2025
Assistance Publique – Hôpitaux de Paris
2016-2025
Institut de Myologie
2016-2025
Université Paris Cité
2011-2024
Tokai University
2024
Tokai University Hospital
2024
Universität Hamburg
2024
Adeno-associated viruses (AAVs) are small, nonenveloped single-stranded DNA that require helper to facilitate efficient replication. Despite the presence of humoral responses wild-type AAV in humans, remains one most promising candidates for therapeutic gene transfer treat many genetic and acquired diseases. Characterization IgG subclass study prevalence both neutralizing factors types 1, 2, 5, 6, 8, 9 human population importance development new strategies overcome these immune responses....
Idiopathic inflammatory myopathies are heterogeneous in their pathophysiologic features and prognosis. The emergence of myositis-specific autoantibodies suggests that subgroups patients exist.To develop a new classification scheme for idiopathic based on phenotypic, biological, immunologic criteria.An observational, retrospective cohort study was performed using database the French myositis network. Patients identified from referral centers neuromuscular diseases were included January 1,...
To report the clinicopathologic features and outcome of myositis in patients treated with immune checkpoint inhibitors (ICIs) (irMyositis).We retrospectively analyzed diagnosed irMyositis tertiary centers Paris, France, Berlin, Germany, from January 2015 to July 2017. The main outcomes were clinical manifestations muscle histology, which included major histocompatibility complex class I (MHC-I), C5b-9, CD3, CD4, CD8, CD20, CD68, programmed cell death protein 1 (PD-1), ligand (PD-L) 1, 2...
We describe a long-term observational study of large cohort patients with sporadic inclusion body myositis and propose weakness composite index that is easy to perform during clinic. Data collection from two groups (Paris Oxford) was completed either clinic visit (52%), or by extraction previous medical records (48%). One hundred thirty-six [57% males, 61 (interquartile range 55–69) years at onset] were included. At the last all had muscle (proximal British Medical Research Council scale...
Necrotizing autoimmune myopathy (NAM) is a group of acquired myopathies characterized by prominent myofiber necrosis with little or no muscle inflammation. Recently, researchers identified autoantibodies (aAb) against 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) in patients NAM, especially statin-exposed patients. Here we report what to our knowledge the first European cohort NAM.The serum 206 suspicion NAM was tested for detection anti-HMGCR aAb using an addressable laser bead...
Cancer can occur in patients with inflammatory myopathies. This association is mainly observed dermatomyositis, and myositis-specific antibodies have allowed us to delineate at an increased risk. Malignancy also reported necrotizing autoimmune myopathies, but the risk remains elusive. Anti-signal recognition particle or anti-HMGCR been specifically associated We aimed screening incidence of cancer A group (n = 115) myopathies without was analysed. occurred more frequently seronegative...
Aims The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled data. Methods Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated. Results Of 3067 cases, 69% female. most common subtype dermatomyositis (DM) (31%). Smoking more frequent in connective...
Dermatomyositis is an acquired auto-immune disease characterized by skin lesions and muscle-specific pathological features such as perifascicular muscle fibre atrophy vasculopathy. patients display upregulation of type I interferon-inducible genes in fibres, endothelial cells, peripheral blood. However, the effect interferon on tissue has not yet been determined. Our aim was to study pathogenicity vitro evaluate efficacy pathway blockade for therapeutic purposes. The activation...
<h3>Objectives</h3> The predominance of extramuscular manifestations (e.g., skin rash, arthralgia, interstitial lung disease [ILD]) as well the low frequency muscle signs in anti–melanoma differentiation-associated gene 5 antibody–positive (anti-MDA5+) dermatomyositis caused us to question term myositis-specific antibody for anti-MDA5 antibody, homogeneity disease. <h3>Methods</h3> To characterize anti-MDA5+ phenotype, an unsupervised analysis was performed on patients (n = 83/121) and...
Immune-checkpoint-inhibitor (ICI)-associated myotoxicity involves the heart (myocarditis) and skeletal muscles (myositis), which frequently occur concurrently are highly fatal. We report results of a strategy that included identification individuals with severe ICI myocarditis by also screening for managing concomitant respiratory muscle involvement mechanical ventilation, as well treatment CTLA4 fusion protein abatacept JAK inhibitor ruxolitinib. Forty cases definite were pathologic...
Anti-signal recognition particle (anti-SRP) autoantibodies are associated with severe acquired necrotizing myopathies. The role of these remains elusive, and the evolution anti-SRP levels over time is unknown. In this study, we developed an addressable laser bead immunoassay (ALBIA) technique to investigate a correlation between levels, serum creatine kinase (CK) muscle strength in patients myopathy.The diagnostic value ALBIA assay was determined by comparing 31 immunodot-positive those 190...
Hydroxychloroquine (HCQ) is an important medication for treating systemic lupus erythematosus (SLE). Its blood concentration ([HCQ]) varies widely between patients and a marker predictor of SLE flares. This prospective randomised, double-blind, placebo-controlled, multicentre study sought to compare standard adjusted HCQ dosing schedules that target [HCQ] ≥1000 ng/ml reduce was measured in 573 with (stable disease SELENA-SLEDAI≤12) treated at least 6 months. Patients from 100 750 were...
Adeno-associated viruses (AAV) are small, nonenveloped single-stranded DNA which require helper to facilitate efficient replication. These recombinant some of the most promising candidates for therapeutic gene transfer treat many genetic and acquired diseases. Nevertheless, presence humoral responses wild-type AAV common among humans is one limitations in vivo transduction efficacy using cognate vector. In this study, based on serum samples that we were able collect from various clinical...
Methods. We reviewed 34 adult patients with biopsy-proven EF. Analyses focused on the therapeutic management, including treatment modalities, responses and associated or predictive factors.
Idiopathic inflammatory myopathies can be classified as polymyositis, dermatomyositis, immune-mediated necrotizing myopathy, sporadic inclusion body myositis or non-specific myositis. Anti-Jo-1 antibody-positive patients are assigned to either polymyositis dermatomyositis suggesting overlapping pathological features. We aimed determine if anti-Jo-1 myopathy has a specific morphological phenotype. In series of 53 muscle biopsies patients, relevant descriptive criteria defining characteristic...
Giant cell arteritis (GCA) is a large-vessel vasculitis of unknown origin. Recent findings indicate that at least 2 separate lineages CD4+ T cells, Th1 and Th17 participate in vascular inflammation. The pathways driving these differentiations are incompletely understood, but may provide novel therapeutic targets. This study was undertaken to identify cytokines involved the pathogenesis GCA.Thirty GCA patients fulfilling American College Rheumatology criteria, with active disease or...
To characterize muscle fiber necrosis in immune-mediated necrotizing myopathies (IMNM) with anti-signal recognition particle (SRP) or anti-3-hydroxy-3-methylglutarylcoenzyme A reductase (HMGCR) antibodies and to explore its underlying molecular immune mechanisms.Muscle biopsies from patients IMNM were analyzed compared control myositis. In addition immunostaining reverse transcription PCR on samples, vitro primary cells was performed.Creatine kinase levels regeneration correlated the...