Explanipedia

Manifestations rhumatologiques des syndromes myélodysplasiques Open

A. Mékinian, O. Fain · 2025

Comparative efficacy and safety of anakinra and canakinumab in patients with <span>VEXAS</span> syndrome – an international multicenter study Open

Tali Eviatar, Dafne Capelusnik, Corrado Campochiaro, Valentin Lacombe, Vincent Jachiet , et al. · 2025

Objectives The aim of this study was to compare differences in clinical response, drug survival, and adverse event rates between anakinra and canakinumab in VEXAS syndrome. Methods This multicenter international study includes VEXAS patien…

Deep hierarchical subtyping of multi-organ systemic sclerosis trajectories - a EUSTAR study Open

Cécile Trottet, Manuel Schürch, Ahmed E. Allam, L. Petelytska, I. Castellví , et al. · 2025

Low-dose interleukin-2 for recurrent early pregnancy loss: a proof-of-concept study Open

David Klatzmann, A. Mékinian, Noémie Abisror, C. Mcavoy, Claire Ribet , et al. · 2025

Regulatory T cells (Tregs) are essential for maternal-fetal tolerance, and their deficiency is implicated in unexplained recurrent early pregnancy loss (uREPL). Low-dose interleukin-2 (IL-2 _LD ) selectively activates Tregs. In t…

Low-dose interleukin-2 for recurrent early pregnancy loss: a proof-of-concept study Open

A. Mékinian, Noémie Abisror, Chloé McAvoy, Claire Ribet, Roberta Lorenzon , et al. · 2025

Background Regulatory T cells (Tregs) are essential for maternal-fetal tolerance, and their deficiency has been implicated in immune-mediated pregnancy loss. Low-dose interleukin-2 (IL-2 LD ) selectively expands and activates Tregs and has…

OP0271 DEVELOPMENT AND VALIDATION OF A EULAR DISEASE ACTIVITY SCORE IN ANTIPHOSPHOLIPID SYNDROME Open

Maria G. Tektonidou, R. Cervera, A. Tincani, Guillermo Pons‐Estel, S. Sciascia , et al. · 2025

POS0379 PAXIS: A RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED, DOSE FINDING PHASE 2 STUDY (PART 1) FOLLOWED BY AN OPEN-LABEL PERIOD (PART 2) TO ASSESS THE EFFICACY AND SAFETY OF PACRITINIB IN PATIENTS WITH VEXAS SYNDROME Open

D.B. Beck, Maël Heiblig, Sinisa Savic, Marcela A. Ferrada, A. Mékinian , et al. · 2025

Efficacy and safety of azacitidine for VEXAS syndrome: a large-scale retrospective study from FRENVEX Open

Vincent Jachiet, Olivier Kosmider, Maxime Beydon, Jérôme Hadjadj, Lin‐Pierre Zhao , et al. · 2025

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a severe monogenic disorder caused by somatic mutations in ubiquitin-like modifier activating enzyme 1 (UBA1), characterized by inflammation, cytopenias, and freq…

Deep Immunophenotyping and Clustering Identifies Biomarkers Predictive of Lymphoma in Primary Sjögren Disease Open

Cindy Marques, Paul Régnier, Anna Maciejewski‐Duval, C. Richard de Vesvrotte, Thomas Vazquez , et al. · 2025

Objective Patients with primary Sjögren disease (pSD) are prone to develop non‐Hodgkin lymphoma (NHL), but relevant biomarkers are lacking. We aimed to determine new biomarkers predictive of NHL in patients with pSD. Methods Two hundred si…

IgA vasculitis associated with chronic myelomonocytic leukemia Open

Bénédicte Rouvière, François Chasset, Noémie Abisror, Pierre Hirsch, O. Fain , et al. · 2025

Very long-term remission with azacitidine in VEXAS syndrome Open

Lin‐Pierre Zhao, Vincent Jachiet, Olivier Kosmider, Lise Larcher, Emmanuelle Clappier , et al. · 2025

Not available.

Cardiovascular events in patients with myositis: results from a French retrospective cohort Open

Camille Kasser, A. Mékinian, Alexis F. Guédon, Yves Allenbach, Olivier Fain , et al. · 2025

Introduction Idiopathic inflammatory myositis (IIM) are systemic diseases, including dermatomyositis (DM), inclusion body myositis (IBM), immune-mediated necrotising myopathy (IMNM), antisynthetase syndrome (ASSD) and overlap myositis (OM)…

Reclassification of 1200 women from EUROAPS* Registry based on the 2023 ACR/EULAR obstetric antiphospholipid syndrome classification criteria Open

Jaume Alijotas‐Reig, Enrique Esteve‐Valverde, Luís Sáez-Comet, Ariadna Anunciación-Llunell, Raquel Ferrer‐Oliveras , et al. · 2025

Utilisation de l’hydroxychloroquine dans les pathologies obstétricales récurrentes à médiation immune (en dehors du lupus systémique) : fondements et preuves scientifiques Open

A. Dernoncourt, V. Salle, Meryam Cheloufi, Gilles Kayem, A. Mékinian · 2024

Hydroxychloroquine (HCQ), a synthetic antimalarial, is recognized for its immunomodulatory, anti-inflammatory and vascular-protective effects. In 20-30% of cases of primary obstetrical antiphospholipid syndrome (APS), the combination of an…

Nonischemic Cardiac Manifestations in VEXAS Syndrome Open

Marie Robert, Alexis Mathian, Valentin Lacombe, Hervé Lobbes, Léopold Adélaïde , et al. · 2024

This case series reports on the clinical presentation, laboratory findings, imaging characteristics, treatments, and outcomes of nonischemic cardiac manifestations in patients with VEXAS syndrome with confirmed pathogenic UBA1 alterations.

VEXAS anemia is a mosaic erythroblastopenia Open

François Rodrigues, Giulia Hardouin, Sara El Hoss, Aya Ghoul, Emilie‐Fleur Gautier , et al. · 2024

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a recently discovered autoinflammatory disorder linked to somatic mutations in the UBA1 gene, resulting in a profound cytoplasm-restricted defect in ubiquitylation. The di…

Higher risk profile among patients with TET2-mutated giant cell arteritis: a cluster analysis Open

Alexis F. Guédon, Asmaa Ouafdi, Nabil Belfeki, Azeddine Dellal, N. Ghriss , et al. · 2024

Objective We aimed to assess the prevalence of clonal haematopoiesis (CH) in patients with giant cell arteritis (GCA) compared with controls and individuals with other autoimmune diseases (AIDs) and to identify high-risk clinical/genetic p…

Vasodilator drugs and heart-related outcomes in systemic sclerosis: an exploratory analysis Open

Alexis F. Guédon, Fabrice Carrat, Luc Mouthon, David Launay, Benjamin Chaigne , et al. · 2024

Background and aims Systemic sclerosis (SSc) is an autoimmune connective disease characterised by excessive extracellular matrix deposition and widespread skin and internal organ fibrosis including various cardiac manifestations. Heart inv…

French protocol for the diagnosis and management of giant cell arteritis Open

Hubert de Boysson, Valérie Devauchelle‐Pensec, C. Agard, Marc André, Boris Bienvenu , et al. · 2024

Giant cell arteritis (GCA) is a large-vessel vasculitis that mainly affects women over fifty. GCA usually involves branches from the external carotid arteries, causing symptoms such as headaches, scalp tenderness, and jaw claudication. The…

A Clinicopathological Description of Kidney Features in VEXAS Syndrome Open

Martin Mathurin, Pierre Hirsch, Vincent Jachiet, Jérôme Hadjadj, G. Le Guenno , et al. · 2024

French protocol for diagnosis and management of Cogan's syndrome Open

Laurent Arnaud, Alexandra Audemard‐Verger, Alexandre Bélot, Boris Bienvenu, C Burillon , et al. · 2024

Fatigue levels and associated factors in systemic sclerosis: a cross-sectional study of 2385 SPIN Cohort participants Open

Linda Kwakkenbos, Brooke Levis, Richard S. Henry, Gabrielle Virgili-Gervais, Marie‐Eve Carrier , et al. · 2024

Objectives The objectives of this study were to compare fatigue in a large multinational SSc cohort with general population data and identify associated sociodemographic, lifestyle and SSc disease factors. Methods Scleroderma Patient-cente…

Angioedema due to Acquired C1-Inhibitor Deficiency Associated With Monoclonal Gammopathies of Undetermined Significance Characteristics of a French National Cohort Open

Constance Lahuna, Federica Defendi, Laurence Bouillet, Isabelle Boccon‐Gibod, A. Mékinian , et al. · 2024

Complete remission of AAE-C1-INH is correlated to complete remission of the underlying hematological malignancy, as defined by an undetectable serum monoclonal immunoglobulin. In our MGUS-associated acquired angioedema cohort, we recorded …

Gastrointestinal disorders in systemic sclerosis: cluster analysis and prognosis from a French prospective cohort Open

Chloé Mollière, Alexis F. Guédon, Nathalie Kapel, Frédéric de Vassoigne, Farouk Graiess , et al. · 2024

GIT manifestations are frequent and severe in SSc. Smoking appears to be associated with severe disease. Anorectal manifestations may be associated with cardiac disease, but we need more studies to validate these results.

Prevalence and association of placental lesions with obstetrical features and outcome: data from French prospective study Open

J. Boujenah, Jonathan Cohen, Michael Allouche, Marianne Ziol, Amélie Benbara , et al. · 2024

The placenta analysis is important to understand the origin of adverse obstetrical outcome and the risk for subsequent pregnancy.

Intravenous Immunoglobulins for Recurrent Chronic Histiocytic Intervillositis: A Series of Case Studies Open

Noémie Abisror, Meryam Cheloufi, Jonathan Cohen, Aurore Coulomb, Chloé McAvoy , et al. · 2024

Introduction Chronic histiocytic intervillositis (CHI) is a rare inflammatory placental disease characterized by diffuse infiltration of monocytes into the intervillous space and is associated with adverse pregnancy outcomes. No treatment …

Hydroxychloroquine in recurrent pregnancy loss: data from a French prospective multicenter registry Open

A. Dernoncourt, Kaies Hedhli, Noémie Abisror, Meryam Cheloufi, Jonathan Cohen , et al. · 2024

STUDY QUESTION What are the outcomes of pregnancies exposed to hydroxychloroquine (HCQ) in women with a history of recurrent pregnancy loss (RPL), and what factors predict the course of these pregnancies beyond the first trimester? SUMMARY…

Experiences of telehealth during and after the COVID-19 pandemic and preferences for future care of people with systemic sclerosis: a cross-sectional study Open

Elsa‐Lynn Nassar, Gabrielle Virgili-Gervais, Marie‐Eve Carrier, Linda Kwakkenbos, Richard S. Henry , et al. · 2024

Kidney involvement in myelodysplastic syndromes Open

Marie-Camille Lafargue, Jean‐Paul Duong Van Huyen, Helmut G. Rennke, Marie Essig, Mickaël Bobot , et al. · 2024

Introduction The objective of this study was to describe kidney involvement in patients with myelodysplastic syndromes (MDS), their treatments, and outcomes. Methods We conducted a multicenter retrospective study in seven centers, identify…

Ophthalmic vascular manifestations in eosinophil-associated diseases: a comprehensive analysis of 57 patients from the CEREO and EESG networks and a literature review Open

Elisa Chapuis, Elodie Bousquet, Jean‐François Viallard, Benjamin Terrier, Zahir Amoura , et al. · 2024

Introduction Eosinophils have widespread procoagulant effects. In daily practice, eosinophil-related cardiovascular toxicity consists of endomyocardial damage, eosinophilic vasculitis and arterial or venous thrombosis. Here we aim to repor…

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