Impaired cytotoxic function and exhausted phenotype of natural killer cells in VEXAS syndrome.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an autoinflammatory disorder caused by acquired somatic UBA1 mutations in hematopoietic stem cells, affecting peripheral myeloid and natural killer (NK) cells. Given the high rate of severe infections observed in VEXAS patients, we hypothesized that NK cell dysfunction contributes to this increased susceptibility. We conducted a comprehensive immune characterization of peripheral NK cells in patients with VEXAS (n=40), patients with autoinflammatory diseases without UBA1 mutations (n=22), and elderly gender-matched healthy controls (HCs) (n=16).

[Place of chitotriosidase as a serum biomarker of sarcoidosis: A narrative review of the literature].

Sarcoidosis is an inflammatory systemic disease characterized by epithelio-gigantocellular granulomatosis without cell necrosis. The diagnosis is pathologic. There are many biomarkers of sarcoidosis.

[Assessment of internship sites in Internal Medicine and Clinical Immunology, 7 years after the Residency Training Program Reform: Findings from a 2024 year-end survey in France].

Objective: To compile a list of approved training sites for the Residency Training Program [Diplôme d'Études Spécialisées] in Internal Medicine and Clinical Immunology (DES-MIIC) in France.

Method: All local coordinators of the DES-MIIC were contacted to establish the list of approved internship sites for the MIIC DES within their geographical subdivision.

Results: We listed 244 approved training sites, of which 87 (35.

Long-term efficacy and safety of the Lupus-Cruces Nephritis protocol: a propensity score study of the Lupus-Cruces and Lupus-Bordeaux cohorts.

Objective: To assess the efficacy and toxicity of the Lupus-Cruces Nephritis (LCN) protocol compared with standard of care (SOC) with cyclophosphamide (CYC) or mycophenolate in patients with lupus nephritis (LN) during an extended follow-up time up to 10 years.

Methods: Patients with biopsy-proven class III, IV or V LN treated with LCN were compared with SOC. Patients in the LCN were treated with a CYC plus repeated methylprednisolone pulse-based regimen.

Pulmonary arterial hypertension in adults with Still's disease: another pulmonary manifestation associated with HLA-DRB1*15.

Objectives: Inflammatory lung disease in Still's disease (SD) has recently been described. Among its manifestations, pulmonary arterial hypertension (PAH) is a rare and life-threatening event, with only a few case reports published. The objective was to report the largest adult cohort of PAH occurring in the context of SD.

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

VEXAS (Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic) syndrome is a severe monogenic disorder caused by somatic UBA1 mutations, characterized by inflammation, cytopenias and frequent association with myelodysplastic neoplasms (MDS). Steroid dependence is common, and targeted therapies have demonstrated limited efficacy. Azacitidine (AZA), a hypomethylating agent used in MDS, has shown potential in VEXAS but data remain limited.

CD4/CD8 T-cell ratio in bronchoalveolar lavage fluid as a marker of sarcoidosis severity: a retrospective study.

Background: Sarcoidosis is a complex multisystemic disease with a wide range of clinical manifestations. Its severity and evolution are highly variable and the prognostic utility of paraclinical markers is debated. We analysed the utility of the CD4/CD8 T-cell ratio (R) in bronchoalveolar lavage (BAL) fluid as a marker of sarcoidosis severity.

Thrombocytopenia following kidney transplantation: a frequent, underestimated and potentially severe complication.

Introduction: Cytopenias occur frequently after kidney transplantation but posttransplantation thrombocytopenia (PTTCP) frequency has rarely been reported.

Methods: This monocenter, retrospective study aimed to describe PTTCP frequency, causes, treatments and outcomes. PTTCP was defined as thrombocytopenia with ≥2 platelet counts <100×10/L after first month posttransplantation.

Neurological manifestations in patients with VEXAS syndrome.

Background: VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a recently described syndrome linked to somatic mutations in the UBA1 gene, causing systemic autoinflammatory manifestations. To date, few data are available concerning neurological manifestations. The aim of this study was to describe their prevalence, clinical spectrum and outcome under treatment.

Exposure to COVID-19 Pandemic-Related Stressors and Their Association With Distress, Psychological Growth and Drug Use in People With HIV in Nouvelle Aquitaine, France (ANRS CO3 AQUIVIH-NA Cohort-QuAliV-QuAliCOV Study).

We investigated people living with HIV (PLWH)'s exposure to COVID-19 pandemic stressors and their association with distress, psychological growth, and substance use. PLWH in the ANRS CO3 AQUIVIH-NA cohort's QuAliV study (Nouvelle Aquitaine, France) completed an adapted CAIR Lab Pandemic Impact Questionnaire (C-PIQ) and reported substance use between 9/2021 to 3/2022. We described cumulative stressor exposure (score 0-16) and explored variation by PLWH characteristics (demographic, HIV-related, risk factors, psychosocial).

Safety of Fertility Treatments in Women With Systemic Lupus Erythematosus: Data From a Prospective Population-Based Study.

Objective: To assess safety of fertility treatments in women with systemic lupus erythematosus (SLE).

Design: Data from the multicentre French observational GR2 (Groupe de Recherche sur la Grossesse et les Maladies Rares) study (2014-ongoing).

Setting: Seventy-six centres in France.

Inflammasome-targeted therapy might prevent adverse perinatal outcomes of recurrent chronic intervillositis of unknown etiology.

Chronic histiocytic intervillositis of unknown origin (CHI) is a rare placental disorder associated with adverse pregnancy outcomes, frequent recurrence, and a lack of effective preventive strategies. Recent insights indicate a potential link between CHI-associated inflammatory lesions and the inflammasome pathway, suggesting innovative therapeutic avenues. Here we show a potential role of the inflammasome pathway in CHI through comprehensive transcriptomic analysis of grade 2 or 3 histopathologic CHI samples, paired with placental controls.

No Effect of Low-Dose Glucocorticoid Maintenance Therapy on Damage in SLE Patients in Prolonged Remission: A Propensity Score Analysis of the Longitudinal Lupus-Cruces-Bordeaux Inception Cohort.

: Prolonged remission on low-dose glucocorticoids (GC) is a main goal in patients with systemic lupus erythematosus (SLE). The aim of this study is to assess whether GC ≤ 5 mg/d increases the risk of damage accrual in patients with SLE in prolonged remission. : Observational study of routine clinical care data of the inception Lupus Cruces-Bordeaux cohort.

Course of immune thrombocytopenia according to the site of platelet destruction identified by indium-111 platelet scintigraphy.

In primary immune thrombocytopenia (ITP), predictors of disease evolution and treatment response are needed. Data based on the site of platelet destruction are scarce. We performed a retrospective single-centre study of adult patients with primary ITP undergoing at least one Indium-111 platelet scintigraphy (IPS) between 2009 and 2018.

[Role of university hospital doctors specialized in internal medicine in health professions training and institutions in France: Survey of the National College of Teachers of Internal Medicine (CEMI)].

Rationale: On the occasion of the General stage meeting of Internal Medicine, the National College of Internal Medicine Teachers (CEMI) conducted a survey on teaching activity among all French university hospital (HU) internal medicine specialists.

Method: The survey was carried out in September 2023 by sending an email to 101 hospital practitioners university professors (PU-PH) and 18 hospital practitioners assistant professors (MCU-PH) of internal medicine in subsection 53-01 of the National council of universities (CNU), as well as to the 11 HU internists working in immunology (subsection 47-01) or therapeutics (subsection 48-04).

Results: Seventy-three HUs (56.

Safety of immune checkpoint inhibitor rechallenge after severe immune-related adverse events: a retrospective analysis.

Immune checkpoint inhibitors (ICIs) present clinicians with the challenge of managing immune-related adverse events (irAEs), which can range from mild to severe due to immune system activation . While guidelines recommend discontinuing ICIs for grade 3 partial and all grade 4 irAEs, there is growing interest in rechallenging patients based on oncological outcomes, particularly for cardiovascular and neurological irAEs where data remains scarce . We retrospectively evaluated the safety of ICI rechallenge following grade 3-4 irAEs, specifically focusing on cardiovascular and neurological events, in patients discussed at our multidisciplinary immunotoxicity assessment board between 2019 and 2021.

Hydroxychloroquine levels in pregnancy and materno-fetal outcomes in systemic lupus erythematosus patients.

Objectives: Data about hydroxychloroquine (HCQ) levels during pregnancy are sparse. We assessed HCQ whole-blood levels at first trimester of pregnancy as a potential predictor of maternal and obstetric/fetal outcomes in patients with systemic lupus erythematosus (SLE).

Methods: We included pregnant SLE patients enrolled in the prospective GR2 study receiving HCQ, with at least one available first-trimester whole-blood HCQ assay.

Efficacy and safety of targeted therapies in VEXAS syndrome: retrospective study from the FRENVEX.

Objectives: Vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic (VEXAS) syndrome is an adult-onset autoinflammatory disease associated with somatic ubiquitin-like modifier-activating enzyme 1 (UBA1) mutations. We aimed to evaluate the efficacy and safety of targeted therapies.

Methods: Multicentre retrospective study including patients with genetically proven VEXAS syndrome who had received at least one targeted therapy.

Methylprednisolone Pulses and Prolonged Remission in Systemic Lupus Erythematosus: A Propensity Score Analysis of the Longitudinal Lupus-Cruces-Bordeaux Inception Cohort.

Objective: The objective of this study was to analyze the effect of methylprednisolone pulses (MP), given during the first year after the diagnosis of systemic lupus erythematosus (SLE), in achieving prolonged remission according to the degree of lupus activity at presentation.

Methods: We conducted an observational study of routine clinical care data from the Lupus-Cruces-Bordeaux cohort. The end point was prolonged remission (ie, during five consecutive yearly visits).