A5018386324- Systemic Sclerosis and Related Diseases
- Vasculitis and related conditions
- Eosinophilic Disorders and Syndromes
- Inflammatory Myopathies and Dermatomyositis
- Sarcoidosis and Beryllium Toxicity Research
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Dermatologic Treatments and Research
- Urticaria and Related Conditions
- Autoimmune Bullous Skin Diseases
- Systemic Lupus Erythematosus Research
- Neutrophil, Myeloperoxidase and Oxidative Mechanisms
- Viral-associated cancers and disorders
- Otitis Media and Relapsing Polychondritis
- Eosinophilic Esophagitis
- Mast cells and histamine
- Skin Diseases and Diabetes
- Cytomegalovirus and herpesvirus research
- Ocular Diseases and Behçet’s Syndrome
- COVID-19 Clinical Research Studies
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- SARS-CoV-2 and COVID-19 Research
- Long-Term Effects of COVID-19
- Autoimmune and Inflammatory Disorders Research
- Diagnosis and Treatment of Venous Diseases
- Rheumatoid Arthritis Research and Therapies
Assistance Publique – Hôpitaux de Paris
2016-2025
Hôpital Cochin
2016-2025
Université Paris Cité
2015-2025
Institut Cochin
2024
Délégation Paris 5
2012-2021
Collaborative Group (United States)
2020
Stanford University
2020
Sorbonne Paris Cité
2013-2020
Centre de Référence Déficits Immunitaires Héréditaires
2013-2017
Hôpital Necker-Enfants Malades
2013-2017
Abstract Objective Earlier studies of eosinophilic granulomatosis with polyangiitis (Churg‐Strauss) (EGPA), limited patient numbers and followup durations, demonstrated that clinical presentation at diagnosis, but not outcome, differed according to antineutrophil cytoplasmic antibody (ANCA) status. This study was undertaken describe the main characteristics a larger cohort their long‐term outcomes. Methods A retrospective EGPA patients in French Vasculitis Study Group who satisfied American...
Eosinophilic granulomatosis with polyangiitisObjective: To develop disease-specific recommendations for the diagnosis and management of eosinophilic polyangiitis (Churg-Strauss syndrome) (EGPA).Methods: The EGPA Consensus Task Force experts comprised 8 pulmonologists, 6 internists, 4 rheumatologists, 3 nephrologists, 1 pathologist allergist from 5 European countries USA.Using a modified Delphi process, list 40 questions was elaborated by 2 members sent to all participants prior...
The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail. In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation disease. study population included 157 patients (mean± sd age 49.4±14.1 years), a mean± blood eosinophil count 7.4±6.4×10 9 L −1 diagnosis. There 11.8±18.2 years from the asthma to diagnosis EGPA, 1.4±8.4 first peripheral...
Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by failure of superoxide production in phagocytic cells. The characterised recurrent infections and inflammatory events, frequently affecting the lungs. Improvement life expectancy now allows most patients to reach adulthood. We aimed describe pattern pulmonary manifestations occurring during adulthood CGD patients. This was retrospective study French national cohort adult (≥16 years old) with CGD. Medical data were...
Epstein-Barr virus (EBV) preferentially infects epithelial cells and B lymphocytes sometimes T NK lymphocytes. Persistence of EBV-infected results in severe lymphoproliferative disorders (LPDs). Diagnosis EBV-driven or cell LPD chronic active EBV diseases (CAEBV) is difficult, often requiring biopsies. Herein, we report a flow-FISH cytometry assay that detects expressing EBV-encoded small RNAs (EBERs), allowing rapid identification among PBMCs. B, T, and/or were detectable various...
To investigate the association of air pollution exposure with severity interstitial lung disease (ILD) at diagnosis and ILD progression among patients systemic sclerosis (SSc)-associated ILD.We conducted a retrospective two-center study SSc-associated diagnosed between 2006 2019. Exposure to pollutants particulate matter up 10 2.5 µm in diameter (PM10, PM2.5), nitrogen dioxide (NO2), ozone (O3) was assessed geolocalization coordinates patients' residential address. Logistic regression models...
Although prognosis of Chronic Granulomatous Disease (CGD) has greatly improved, few studies have focused on its long-term outcome. We studied the clinical course and sequelae CGD patients diagnosed before age 16, at various adult time points.Cross-sectional French nationwide retrospective study screened through National Reference Center for Primary Immunodeficiencies (CEREDIH) registry.Eighty (71 males [88.7%], 59 X-linked [73.7%], median 23.9 years [minimum, 16.6; maximum, 59.9]) were...
The complications of kidney graft preservation fluid infected by Candida sp. may range in severity from trivial infections to life-threatening complications, including arteritis and anastomotic rupture. Mandatory nephrectomy has recently been proposed as a means preventing arterial wall rupture such cases. We describe the clinical features outcome renal transplantation cadaveric donor eight recipients with testing positive for Six patients were treated antifungal drugs. After 1-2 years...
Background Longitudinal follow-up of interstitial lung diseases (ILDs) at CT mainly relies on the evaluation extent ILD, without accounting for shrinkage. Purpose To develop a deep learning–based method to depict worsening ILD based shrinkage detection from elastic registration chest scans in patients with systemic sclerosis (SSc). Materials and Methods Patients SSc evaluated between January 2009 October 2017 who had undergone least two unenhanced supine pulmonary function tests (PFTs)...
Abstract Objective Stratifying the risk of death in SSc-related interstitial lung disease (SSc-ILD) is a challenging issue. The extent fibrosis on high-resolution CT (HRCT) often assessed by visual semiquantitative method that lacks reliability. We aimed to assess potential prognostic value deep-learning–based algorithm enabling automated quantification ILD HRCT patients with SSc. Methods correlated occurrence during follow-up, and evaluated additional predicting based model including...
Churg-Strauss syndrome (CSS), first described in 1951, is a rare vasculitis of small- and medium-sized vessels. It characterized by constant association with asthma eosinophilia, the presence anti-myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibodies (ANCA) ~40% patients. Vasculitis typically develops previously asthmatic eosinophilic middle-aged patient most frequently involves peripheral nerves skin. Other organs, however, may be affected must screened for vasculitis, especially...
<h3>Background</h3> Primary Sjögren9s syndrome (pSS) is a systemic autoimmune disease characterized by B-cell hyperactivation. In some patients, multiple autoantibodies develop, including anti-neutrophil cytoplasmic antibody (ANCA), which could lead to the development of ANCA-associated vasculitis (AAV). <h3>Objectives</h3> To describe clinical presentation, therapy and prognosis patients diagnosed with both pSS AAV. <h3>Methods</h3> Nation-wide survey in France collecting information on...
Objective Myocardial involvement may occur during systemic sclerosis ( SS c) and can lead to impaired myocardial contraction and/or arrhythmia. Cardiac magnetic resonance imaging MRI ) is used for noninvasive characterization of the myocardium. The aim this study was evaluate utility cardiac with intravoxel incoherent motion IVIM diffusion‐weighted DWI longitudinal relaxation time (T1) sequence mapping assessment microvascular interstitium impairment in c. Methods In single‐center...
Total lung capacity (TLC) is seldom assessed in the prediction of systemic sclerosis (SSc) disease severity. To describe and analyse TLC SSc. We performed a retrospective multicentre study SSc patients enrolled French national cohort with at least one assessment, described based on baseline measurements, modelized trajectories SSc, associated measures prognosis. Two thousand three hundred forty-seven were included study. Baseline was severity survival, as well occurrence interstitial (ILD),...
Mixed connective tissue disease (MCTD) is a rare systemic disorder that belongs to diseases (CTD). Few studies are available on MCTD treatment. We conducted an observational study within the French cohort. Data were collected at diagnosis, during follow-up, and last follow-up (LFU). studied three treatment groups i) no treatment, ii) hydroxychloroquine (HCQ) and/or glucocorticoids (GC) iii) disease-modifying antirheumatic drugs (DMARDs)/immunosuppressant (IS). Three hundred fifteen patients...