Jérôme Hadjadj
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View article: In silico modeling guides identification of novel JAK1 variants associated with immune dysregulation
In silico modeling guides identification of novel JAK1 variants associated with immune dysregulation Open
Characterization of primary immune dysregulations and deficiency disorders caused by hyperactivating variants of the JAK/STAT pathway highlighted its crucial role in immune cell development and response. To systematically evaluate pathogen…
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: Whole Exome Sequencing of Adult Patients With Evans Syndrome Reveals Pathogenic Variants Associated With Autoimmunity
Whole Exome Sequencing of Adult Patients With Evans Syndrome Reveals Pathogenic Variants Associated With Autoimmunity 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: 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: Clinical manifestations, disease penetrance, and treatment in individuals with SOCS1 insufficiency: a registry-based and population-based study
Clinical manifestations, disease penetrance, and treatment in individuals with SOCS1 insufficiency: a registry-based and population-based study Open
View article: Angioedema Due to Acquired C1-Inhibitor Deficiency Without Hematological Condition: A Multicenter French Cohort Study of 34 Patients
Angioedema Due to Acquired C1-Inhibitor Deficiency Without Hematological Condition: A Multicenter French Cohort Study of 34 Patients Open
AAE-C1-INH without a hematological condition displays a different clinical and biological presentation from lymphoma-associated AAE-C1-INH. No autoimmune disease was identified. Unlike rituximab, long-term prophylaxis seems to prevent AE a…
View article: Efficacy of valaciclovir in preventing herpes zoster in patients receiving anifrolumab
Efficacy of valaciclovir in preventing herpes zoster in patients receiving anifrolumab Open
International audience
View article: Relevance of anti–platelet factor 4/heparin antibodies and platelet activation in systemic inflammatory diseases and thrombosis disorders: insight from the COVID-19 pandemic
Relevance of anti–platelet factor 4/heparin antibodies and platelet activation in systemic inflammatory diseases and thrombosis disorders: insight from the COVID-19 pandemic Open
View article: Nonischemic Cardiac Manifestations in VEXAS Syndrome
Nonischemic Cardiac Manifestations in VEXAS Syndrome Open
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.
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: Angioedema due to Acquired C1-Inhibitor Deficiency Associated With Monoclonal Gammopathies of Undetermined Significance Characteristics of a French National Cohort
Angioedema due to Acquired C1-Inhibitor Deficiency Associated With Monoclonal Gammopathies of Undetermined Significance Characteristics of a French National Cohort Open
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 …
View article: Severe enterovirus infections in patients with immune-mediated inflammatory diseases receiving anti-CD20 monoclonal antibodies
Severe enterovirus infections in patients with immune-mediated inflammatory diseases receiving anti-CD20 monoclonal antibodies Open
Objective Patients with X linked agammaglobulinemia are susceptible to enterovirus (EV) infections. Similarly, severe EV infections have been described in patients with impaired B-cell response following treatment with anti-CD20 monoclonal…
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: Neutralizing Antibody Levels as a Correlate of Protection Against <scp>SARS‐CoV</scp>‐2 Infection: A Modeling Analysis
Neutralizing Antibody Levels as a Correlate of Protection Against <span>SARS‐CoV</span>‐2 Infection: A Modeling Analysis Open
Although anti‐severe acute respiratory syndrome‐coronavirus 2 antibody kinetics have been described in large populations of vaccinated individuals, we still poorly understand how they evolve during a natural infection and how this impacts …
View article: PLCG2-associated immune dysregulation (PLAID) comprises broad and distinct clinical presentations related to functional classes of genetic variants
PLCG2-associated immune dysregulation (PLAID) comprises broad and distinct clinical presentations related to functional classes of genetic variants Open
View article: Severe <scp>SARS‐CoV‐2</scp> infection in rituximab‐treated patients with autoimmune cytopenia: A multicenter observational study
Severe <span>SARS‐CoV‐2</span> infection in rituximab‐treated patients with autoimmune cytopenia: A multicenter observational study Open
Rituximab (RTX), a monoclonal anti-CD20 antibody targeting B cells, is one of the main therapies for many autoimmune diseases, including immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). During the first waves of the CO…
View article: Infections sévères à entérovirus chez les patients traités par rituximab pour une maladie auto-immune : série de cas et revue de la littérature
Infections sévères à entérovirus chez les patients traités par rituximab pour une maladie auto-immune : série de cas et revue de la littérature Open
View article: Incidence et facteurs de risque d’infection sévère à SARS-CoV-2 chez les patients traités par rituximab pour une cytopénie auto-immune : étude rétrospective multicentrique de 222 patients
Incidence et facteurs de risque d’infection sévère à SARS-CoV-2 chez les patients traités par rituximab pour une cytopénie auto-immune : étude rétrospective multicentrique de 222 patients Open
View article: Impact of rituximab on humoral response to SARS-CoV-2 vaccination in previously vaccinated patients with autoimmune diseases
Impact of rituximab on humoral response to SARS-CoV-2 vaccination in previously vaccinated patients with autoimmune diseases Open
View article: Modelling the association between neutralizing antibody levels and SARS-CoV-2 viral dynamics : implications to define correlates of protection against infection
Modelling the association between neutralizing antibody levels and SARS-CoV-2 viral dynamics : implications to define correlates of protection against infection Open
Background While anti-SARS-CoV-2 antibody kinetics have been well described in large populations of vaccinated individuals, we still poorly understand how they evolve during a natural infection and how this impacts viral clearance. Methods…
View article: Platelet activation and coronavirus disease 2019 mortality: Insights from coagulopathy, antiplatelet therapy and inflammation
Platelet activation and coronavirus disease 2019 mortality: Insights from coagulopathy, antiplatelet therapy and inflammation Open
View article: Longitudinal analysis of serum neutralization of SARS-CoV-2 Omicron BA.2, BA.4, and BA.5 in patients receiving monoclonal antibodies
Longitudinal analysis of serum neutralization of SARS-CoV-2 Omicron BA.2, BA.4, and BA.5 in patients receiving monoclonal antibodies Open
View article: Monogenic early-onset lymphoproliferation and autoimmunity: Natural history of STAT3 gain-of-function syndrome
Monogenic early-onset lymphoproliferation and autoimmunity: Natural history of STAT3 gain-of-function syndrome Open
STAT3 GOF patients present with a wide array of immune-mediated disease including lymphoproliferation, autoimmune cytopenias, and multisystem autoimmunity. Patient care tends to be siloed, without a clear treatment strategy. Thus, early id…
View article: Longitudinal analysis of serum neutralization of SARS-CoV-2 Omicron BA.2, BA.4 and BA.5 in patients receiving monoclonal antibodies
Longitudinal analysis of serum neutralization of SARS-CoV-2 Omicron BA.2, BA.4 and BA.5 in patients receiving monoclonal antibodies Open
The emergence of novel Omicron lineages, such as BA.5, may impact the therapeutic efficacy of anti-SARS-CoV-2 neutralizing monoclonal antibodies (mAbs). Here, we evaluated the neutralization and ADCC activity of 6 therapeutic mAbs against …
View article: Clonal hematopoiesis is not significantly associated with COVID-19 disease severity
Clonal hematopoiesis is not significantly associated with COVID-19 disease severity Open
View article: P1408: MONOALLELIC AND BIALLELIC GERMLINE MUTATIONS AFFECTING THE TRANSCRIPTION FACTOR HELIOS CAUSE PLEIOTROPIC DEFECTS OF IMMUNITY
P1408: MONOALLELIC AND BIALLELIC GERMLINE MUTATIONS AFFECTING THE TRANSCRIPTION FACTOR HELIOS CAUSE PLEIOTROPIC DEFECTS OF IMMUNITY Open
Background: Helios, encoded by IKZF2, is a member of the Ikaros family of transcription factors with pivotal roles in T-follicular helper, NK- and T-regulatory cell physiology. Somatic IKZF2 mutations are frequently found in lymphoid malig…
View article: Gain-of-function <i>IKZF1</i> variants in humans cause immune dysregulation associated with abnormal T/B cell late differentiation
Gain-of-function <i>IKZF1</i> variants in humans cause immune dysregulation associated with abnormal T/B cell late differentiation Open
IKZF1/IKAROS is a key transcription factor of lymphocyte development expressed throughout hematopoiesis. Heterozygous germline IKZF1 haploinsufficient ( IKZF1 HI ) and dominant-negative ( IKZF1 DN ) variants in humans cause B cell immune d…