Characterisation of airway disease associated with Sjögren disease.

Objective: Although airway disease associated with Sjögren's disease (Sjo-AD) is common, it is poorly studied compared with interstitial lung disease (ILD). In this study, we aimed to assess factors associated with Sjo-AD, the characteristics and prognosis of this manifestation.

Methods: We performed a retrospective multicentric study involving nine centres.

Comparing clinical features between males and females with VEXAS syndrome: data from literature analysis of patient reports.

Objectives: VEXAS syndrome is an autoinflammatory disease associated with a somatic mutation of the X-linked UBA1 gene in haematopoietic progenitor cells. This disorder was originally described as a disease affecting men, but rare cases of VEXAS syndrome in women have since been reported. The theoretical existence of phenotypic sex differences in this X-linked disease is debated.

Laboratory tests for investigating anemia: From an expert system to artificial intelligence.

Objective: To compare the laboratory tests conducted in real-life settings for patients with anemia with the expected prescriptions derived from an optimal checkup.

Methods: A panel of experts formulated an "optimal laboratory test assessment" specific to each anemia profile. A retrospective analysis was done of the laboratory tests conducted according to the type of anemia (microcytic, normocytic or macrocytic).

Systemic sclerosis, silica exposure and cellular therapies: The sand in the gears?

Systemic sclerosis (SSc) is a chronic orphan autoimmune disease with the highest mortality rate among rheumatic diseases. SSc-related interstitial-lung disease (ILD) remains among the leading causes of SSc-related mortality with still few therapeutic effective strategies. In patients with crystallin silica exposure, SSc is recognized as an occupational disease according to the French social security system (Table 25A of the general insurance regimen).

Clinical and histological features of histiocytoid Sweet syndrome associated with VEXAS syndrome.

Background: VEXAS (Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is caused by acquired somatic mutations in UBA1. Sweet-syndrome-like skin disorders [and especially histiocytoid Sweet syndrome (HSS)] may be associated with VEXAS syndrome.

Objectives: To characterize the clinical and histopathological features of HSS in patients with VEXAS syndrome.

Genome sequencing identify chromosome 9 inversions disrupting ENG in 2 unrelated HHT families.

Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is a dominant inherited vascular disorder. The clinical diagnosis is based on the Curaçao criteria and pathogenic variants in the ENG and ACVRL1 genes are responsible for most cases of HHT. Four families with a negative targeted gene panel and selected by a multidisciplinary team were selected and whole-genome sequencing was performed according to the recommendations of the French National Plan for Genomic Medicine.

Emerging diagnostic and therapeutic challenges for skin fibrosis in systemic sclerosis.

Systemic sclerosis (also called scleroderma, SSc) is a chronic autoimmune disorder characterized by excessive collagen deposition leading to skin fibrosis and various internal organ manifestations. The emergent diagnostics and therapeutic strategies for scleroderma focus on early detection and targeted interventions to improve patient outcomes and quality of life. Diagnostics for SSc have evolved significantly in recent years, driven by advancements in serological markers and imaging techniques.

Successful Introduction of Benralizumab for Eosinophilic Ascites.

Eosinophilic ascites is a rare disorder, reported in both adult and pediatric patients, characterized by high eosinophil counts in the peritoneal fluid. Eosinophilic ascites appears as a manifestation of various diseases such as parasitic and fungal infections, malignancy, and hypereosinophilic syndrome. It also represents an uncommon manifestation of eosinophilic gastroenteritis, usually treated with corticosteroids.

Deep RNA sequencing of muscle tissue reveals absence of viral signatures in dermatomyositis.

: To explore a possible connection between active viral infections and manifestation of dermatomyositis (DM). Skeletal muscle biopsies were analyzed from patients diagnosed with juvenile (n=10) and adult (n=12) DM. Adult DM patients harbored autoantibodies against either TIF-1γ (n=7) or MDA5 (n=5).

History of pre-eclampsia does not appear to be a risk factor for vascular phenotype in women with systemic sclerosis.

Background: Vascular phenotype is associated with a poor prognosis in systemic sclerosis (SSc). The identification of its risk factors could facilitate its early detection.

Objectives: To explore risk factors for a vascular phenotype of SSc, among them a history of pre-eclampsia.

The First Documented Ibuprofen-Induced Toxic Epidermal Necrolysis in the Middle East and North Africa Region: A Case Report, Complications, and Management.

Introduction: Stevens-Johnson syndrome (SJS), Stevens-Johnson/toxic epidermal necrolysis overlap syndrome (SJS/TEN) and toxic epidermal necrolysis (TEN) are rare, acute, potentially lethal conditions, considered to be part of the severe cutaneous adverse reactions (SCARs) spectrum, with TEN being the most life-threatening. The distinction between these three entities is based on the extent of total skin surface involvement, with SJS involving < 10%, SJS/TEN involving 10-30% and TEN involving > 30% of total body surface area. These mucocutaneous reactions are most commonly caused by a hypersensitivity reaction to a drug, with infections and vaccines being possible, less common etiologies.

Defining the course of neurosarcoidosis according to presentation at onset and disease modifying treatment: a cohort study of 84 patients.

Background: Neurosarcoidosis is a rare manifestation of sarcoidosis with heterogeneous presentations. Patient management is challenging due to the current lack of knowledge about the long-term disease course.

Objective: To identify specific disease courses of neurosarcoidosis according to the clinical and paraclinical presentations at onset.

Vacuoles in circulating immature myeloid cells in VEXAS syndrome and comparison with UBA1-wild type patients.

We did not identify any vacuole-related differences in circulating immature myeloid cells between VEXAS patients and UBA1-WT 'VEXAS-like' patients. The similar vacuolization of circulating immature myeloid cells between VEXAS and UBA1-WT patients is explained by the main bloodstream passage of late precursors, in which the vacuolization is already similar in bone marrow in both cases.

FLT3L-dependent dendritic cells control tumor immunity by modulating Treg and NK cell homeostasis.

FLT3-L-dependent classical dendritic cells (cDCs) recruit anti-tumor and tumor-protecting lymphocytes. We evaluate cancer growth in mice with low, normal, or high levels of cDCs. Paradoxically, both low or high numbers of cDCs improve survival in mice with melanoma.

Neointimal myofibroblasts contribute to maintaining Th1/Tc1 and Th17/Tc17 inflammation in giant cell arteritis.

Vascular smooth muscle cells (VSMCs) have been shown to play a role in the pathogenesis of giant cell arteritis (GCA) through their capacity to produce chemokines recruiting T cells and monocytes in the arterial wall and their ability to migrate and proliferate in the neointima where they acquire a myofibroblast (MF) phenotype, leading to vascular stenosis. This study aimed to investigate if MFs could also impact T-cell polarization. Confocal microscopy was used to analyze fresh fragments of temporal artery biopsies (TABs).

[F]FDG PET-MR characterization of aortitis in the IL1rn mouse model of giant-cell arteritis.

Background: Metabolic imaging is routinely used to demonstrate aortitis in patients with giant-cell arteritis. We aimed to investigate the preclinical model of aortitis in BALB/c IL1rn mice using [F]fluorodeoxyglucose ([F]FDG) positron emission tomography-magnetic resonance (PET-MR), gamma counting and immunostaining. We used 15 first-generation specific and opportunistic pathogen-free (SOPF) 9-week-old IL1rn mice, 15 wild-type BALB/cAnN mice and 5 s-generation specific pathogen-free (SPF) 9-week-old IL1rn.