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.

Factors associated with sexual quality of life among men living with HIV.

Introduction: This study aims to assess the prevalence of sexual difficulties and identify factors associated with the Sexual Quality of Life (SQoL) among people living with HIV (PLWHA).

Methods: The study included 107 heterosexual men and 474 men who have sex with men (MSM) from five countries. Participants self-reported variables related to physical and mental health, as well as HIV-related parameters.

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.

Giant cell arteritis associated with scalp, tongue or lip necrosis: A French multicenter case control study.

Background: Scalp, tongue and/or lip necrosis are rare complications of GCA.

Objectives: To describe characteristics and outcome of patients with giant cell arteritis (GCA) -related scalp, tongue and/or lip necrosis.

Methods: A retrospective nationwide multicenter study included 20 GCA patients with scalp, tongue, and/or lip necrosis diagnosed between 1998 and 2021 and 80 GCA control patients matched for age, sex and management period.

Late-Onset Autoimmune Lymphoproliferative Syndrome in a Costa Rican Woman.

Autoimmune lymphoproliferative syndrome (ALPS) is a primary immune regulatory disorder (PIRD). This disease usually develops during childhood. However, atypically, some cases may have their onset in adulthood.

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.

Clinical presentation, course, and prognosis of patients with mixed connective tissue disease: A multicenter retrospective cohort.

Objectives: The objective of this study is to better characterize the features and outcomes of a large population of patients with mixed connective tissue disease (MCTD).

Methods: We performed an observational retrospective multicenter cohort study in France. Patients who fulfilled at least one diagnostic criterion set for MCTD and none of the criteria for other differentiated CTD (dCTD) were included.

Efficacy and safety of ligelizumab in adults and adolescents with chronic spontaneous urticaria: results of two phase 3 randomised controlled trials.

Background: Many patients with chronic spontaneous urticaria (CSU) do not achieve complete control of their symptoms with current available treatments. In a dose-finding phase 2b study, ligelizumab improved urticaria symptoms in patients with H1-antihistamine (H1-AH) refractory CSU. Here, we report the efficacy and safety outcomes from two ligelizumab phase 3 studies.

Early Ear, Nose and Throat Manifestations in Eosinophilic Granulomatosis with Poliangioitis: Results from Our Cohort Group and Literature Review.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, systemic necrotizing vasculitis affecting small-to-medium-sized vessels. EGPA's clinical manifestations are heterogeneous, affecting different organs and systems, and the upper respiratory tract can be affected by ear, nose and throat (ENT) involvement. The aim of our study was to assess type manifestations at the time of diagnosis in a cohort of EGPA patients and correlate findings with baseline variables (sex, age, antineutrophil cytoplasmic antibodies-ANCA-status) and literature reports.