Vincent Jachiet
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View article: Comparative efficacy and safety of anakinra and canakinumab in patients with <scp>VEXAS</scp> syndrome – an international multicenter study
Comparative efficacy and safety of anakinra and canakinumab in patients with <span>VEXAS</span> syndrome – an international multicenter study Open
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…
View article: OP0287 HETEROGENEITY OF THE CLINICAL PRESENTATION IN VEXAS SYNDROME: INSIGHTS FROM 318 CASES FROM THE FRENCH VEXAS STUDY GROUP (FRENVEX).
OP0287 HETEROGENEITY OF THE CLINICAL PRESENTATION IN VEXAS SYNDROME: INSIGHTS FROM 318 CASES FROM THE FRENCH VEXAS STUDY GROUP (FRENVEX). Open
View article: Efficacy of erythroid‐stimulating agent and luspatercept in VEXAS syndrome: A multicenter retrospective study by the FRENVEX group
Efficacy of erythroid‐stimulating agent and luspatercept in VEXAS syndrome: A multicenter retrospective study by the FRENVEX group Open
View article: POS0383 IL-1 INHIBITION IN PATIENTS WITH VEXAS SYNDROME – A RETROSPECTIVE INTERNATIONAL MULTICENTER STUDY
POS0383 IL-1 INHIBITION IN PATIENTS WITH VEXAS SYNDROME – A RETROSPECTIVE INTERNATIONAL MULTICENTER STUDY Open
View article: Correction: Neurological manifestations in patients with VEXAS syndrome
Correction: Neurological manifestations in patients with VEXAS syndrome Open
View article: Efficacy and safety of azacitidine for VEXAS syndrome: a large-scale retrospective study from FRENVEX
Efficacy and safety of azacitidine for VEXAS syndrome: a large-scale retrospective study from FRENVEX Open
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…
View article: Very long-term remission with azacitidine in VEXAS syndrome
Very long-term remission with azacitidine in VEXAS syndrome Open
Not available.
View article: VEXAS anemia is a mosaic erythroblastopenia
VEXAS anemia is a mosaic erythroblastopenia Open
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…
View article: A Clinicopathological Description of Kidney Features in VEXAS Syndrome
A Clinicopathological Description of Kidney Features in VEXAS Syndrome Open
View article: French protocol for diagnosis and management of Cogan's syndrome
French protocol for diagnosis and management of Cogan's syndrome Open
View article: Bridging the Gap: Registry and Population-Based Perspectives on SOCS1 Insufficiency
Bridging the Gap: Registry and Population-Based Perspectives on SOCS1 Insufficiency Open
View article: Clinical and pathological features of cutaneous manifestations in VEXAS syndrome: A multicenter retrospective study of 59 cases
Clinical and pathological features of cutaneous manifestations in VEXAS syndrome: A multicenter retrospective study of 59 cases Open
View article: Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients
Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients Open
Objective A new adult-onset autoinflammatory syndrome has been described, named VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic). We aimed to compare the clinical characteristics, the laboratory features and the outcomes be…
View article: 6-Sulfo LacNAc monocytes are quantitatively and functionally disturbed in systemic sclerosis patients
6-Sulfo LacNAc monocytes are quantitatively and functionally disturbed in systemic sclerosis patients Open
Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis, microangiopathy, and autoantibodies. We previously reported that circulating follicular helper T (cTfh) cells are increased in SSc and induce plasmablast differen…
View article: L’infection a SARS-CoV-2 pourrait-elle induire des rhumatismes inflammatoires chroniques ? Une enquête nationale française
L’infection a SARS-CoV-2 pourrait-elle induire des rhumatismes inflammatoires chroniques ? Une enquête nationale française Open
View article: Further characterization of clinical and laboratory features in VEXAS syndrome: large‐scale analysis of a multicentre case series of 116 French patients*
Further characterization of clinical and laboratory features in VEXAS syndrome: large‐scale analysis of a multicentre case series of 116 French patients* Open
VEXAS syndrome has a large spectrum of organ manifestations and shows different clinical and prognostic profiles. It also raises a potential impact of the identified UBA1 mutation.
View article: Tocilizumab and COVID-19: Timing of administration assessment
Tocilizumab and COVID-19: Timing of administration assessment Open
View article: Eltrombopag for myelodysplastic syndromes or chronic myelomonocytic leukaemia with no excess blasts and thrombocytopenia: a French multicentre retrospective real‐life study
Eltrombopag for myelodysplastic syndromes or chronic myelomonocytic leukaemia with no excess blasts and thrombocytopenia: a French multicentre retrospective real‐life study Open
Summary Despite a moderate prevalence in low‐risk myelodysplastic syndromes (MDS) and chronic myelomonocytic leukaemia (CMML), thrombocytopenia remains a risk of severe bleeding and therapeutic options are still limited. There are only a f…
View article: Efficacité du Tocilizumab dans la COVID-19 modérée à sévère : une cohorte française exposé-non exposé
Efficacité du Tocilizumab dans la COVID-19 modérée à sévère : une cohorte française exposé-non exposé Open
View article: Tocilizumab au cours du COVID-19 : le plus tôt serait le mieux ? Une analyse exploratoire de cohorte
Tocilizumab au cours du COVID-19 : le plus tôt serait le mieux ? Une analyse exploratoire de cohorte Open
View article: Inflammatory and Immune Disorders Associated with Myelodysplastic Syndromes
Inflammatory and Immune Disorders Associated with Myelodysplastic Syndromes Open
Systemic auto-inflammatory or autoimmune diseases (SIADs) develop in up to a quarter of patients with myelodysplastic syndromes (MDS) or chronic myelomonocytic leukemia (CMML). With or without the occurrence of SIADs, the distribution of M…
View article: Acute Myocarditis Revealing Adult-Onset Still’s Disease
Acute Myocarditis Revealing Adult-Onset Still’s Disease Open
View article: Venous thromboembolism during systemic inflammatory and autoimmune diseases associated with myelodysplastic syndromes, chronic myelomonocytic leukaemia and myelodysplastic/myeloproliferative neoplasms: a French multicentre retrospective case-control study
Venous thromboembolism during systemic inflammatory and autoimmune diseases associated with myelodysplastic syndromes, chronic myelomonocytic leukaemia and myelodysplastic/myeloproliferative neoplasms: a French multicentre retrospective case-control study Open
VTE is a common complication in MDS/CMML-associated SIADSs with an increased risk of bleeding when treated by anticoagulants. In the MDS/CMML subgroup, SIADS flares and MDS/CMML progression seem to be prothrombotic risk factors.
View article: Longitudinally Extensive Myelitis Associated With Immune Checkpoint Inhibitors
Longitudinally Extensive Myelitis Associated With Immune Checkpoint Inhibitors Open
Myelitis is a rare but severe complication of ICIs that shows limited response to glucocorticoids. Considering the poor functional outcome associated with longitudinally extensive myelitis, strong and protracted immune therapy combinations…
View article: Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia
Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia Open
Myelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML) are associated with systemic inflammatory or autoimmune diseases in 10-20 % of cases. Among them, immune thrombocytopenia (ITP) has been reported but large studies …
View article: Vasculitis associated with myelodysplastic syndrome and chronic myelomonocytic leukemia: French multicenter case-control study
Vasculitis associated with myelodysplastic syndrome and chronic myelomonocytic leukemia: French multicenter case-control study Open
View article: Circulating follicular helper T cells are increased in systemic sclerosis and promote plasmablast differentiation through the IL-21 pathway which can be inhibited by ruxolitinib
Circulating follicular helper T cells are increased in systemic sclerosis and promote plasmablast differentiation through the IL-21 pathway which can be inhibited by ruxolitinib Open
View article: Clinical, imaging, and histological presentations and outcomes of stroke related to sarcoidosis
Clinical, imaging, and histological presentations and outcomes of stroke related to sarcoidosis Open
View article: Autoimmune manifestations associated with myelodysplastic syndromes
Autoimmune manifestations associated with myelodysplastic syndromes Open
View article: Mucosal‐associated Invariant Cells are Deficient in Systemic Sclerosis
Mucosal‐associated Invariant Cells are Deficient in Systemic Sclerosis Open
Systemic sclerosis ( SS c) is an autoimmune disease, characterized by fibrosis of the skin and other organs, vascular impairment and deficient immune responses. Mucosal‐associated invariant T cells ( MAIT ) have been involved in various in…