Prevalence of Antiphospholipid Antibody Syndrome Among Patients with Recurrent Pregnancy Loss: Impact of the Revised 2023 ACR/EULAR Antiphospholipid Syndrome Criteria.

Current guidelines recommend systematic screening for rheumatic diseases (RDs), including antiphospholipid syndrome (APS), in patients with recurrent pregnancy loss (RPL). However, these recommendations are based on limited evidence, as data on the prevalence of RD in this specific population remain scarce. In particular, the impact of the recent update to the ACR/EULAR classification criteria for APS on the prevalence of RD among RPL patients has yet to be clarified.

Distinct pathophysiological pathways support stratification of Sjögren's disease based on symptoms, clinical, and routine biological data.

Objective: Recently, three distinct phenotypes of Sjögren's disease (SjD) patients have been described, based on cluster analysis: B-cell active with low symptoms (BALS), high systemic activity (HSA), and low systemic activity with high symptoms (LSAHS). We aimed to assess whether these clusters were associated with distinct biomarkers and the prognostic value of IFN signature.

Methods: The ASSESS cohort is a 20-year prospective cohort of SjD patients.

Effect of oral nintedanib vs placebo on epistaxis in hereditary hemorrhagic telangiectasia: the EPICURE multicenter randomized double-blind trial.

Epistaxis greatly affects patients with hereditary hemorrhagic telangiectasia (HHT). Although few systemic treatment exist, nintedanib, is a good candidate thanks to its anti-angiogenic activity. Our main objective was to evaluate the efficacy of oral nintedanib on epistaxis duration in HHT patients with moderate to severe epistaxis.

Immune checkpoint inhibitors-associated vasculitis: a heterogeneous condition with possible severe disease course.

Objective: To describe presentation, treatment and outcome of immune checkpoint inhibitor (ICI) associated-vasculitis in cancer patients in a multicentre study.

Methods: Thanks to the ImmunoCancer International Registry (ICIR), a multidisciplinary network focused on the research of the immune related adverse events related to cancer immunotherapies, patients presenting with a clinical and/or radiological suspicion of vasculitis, and histological evidence of vasculitis after being exposed to ICIs were retrospectively identified.

Results: Twenty eight cases were identified in the ICIR registry.

Bullous Pemphigoid Induced by Cefixime: A Rare Side Effect.

Bullous pemphigoid (BP) is the most prevalent autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and manifests with pruritus and localized or, most commonly, generalized bullous lesions. Numerous studies have established the association between BP and oral antidiabetic agents, particularly dipeptidyl peptidase 4 (DPP4) inhibitors, diuretics, and certain antibiotics, notably levofloxacin and cephalexin.

Are ultrasound salivary parenchymal lesions more severe in primary Sjögren patients with a longer disease duration? A cross-sectional study.

Objectives: Salivary gland ultrasound (SGUS) has an interest in primary Sjögren's disease (pSD) for diagnosis, but the evolution of parenchymal lesions over time is unknown. The objective of this study was to assess the severity of ultrasound abnormalities in relation to pSD duration from the time of buccal dryness onset.

Methods: In this cross-sectional international multicentre study, patients with pSD according to the 2002 or 2016 ACR/EULAR classification criteria were included.

Systemic sclerosis-related fecal incontinence: a scoping review focusing on a neglected manifestation.

Objectives: This scoping-review sought to summarize the current knowledge on the epidemiology, pathogenesis, clinical presentation, and the investigations that may help characterize faecal incontinence (FI) in patients with systemic sclerosis (SSc).

Methods: The planned scoping review was based on the methodological framework proposed by Arksey & O'Malley.Two databases were screened: PubMed (Medline), (webofSciences), data extraction was performed using a predefined template.

Granulocyte Colony-Stimulating Factor-Associated Aortitis Unveiled by 18 F-FDG PET/CT.

In oncology, G-CSF (granulocyte colony-stimulating factor) is often administered to counteract chemotherapy-induced neutropenia. Recent studies have highlighted a significant side effect, G-CSF-associated aortitis, with an incidence of ~0.4%.

Meta-analyses uncover the genetic architecture of Idiopathic Inflammatory Myopathies.

Objective: Idiopathic inflammatory myopathies (myositis, IIMs) are rare, systemic autoimmune disorders that lead to muscle inflammation, weakness, and extra-muscular manifestations, with a strong genetic component influencing disease development and progression. Previous genome-wide association studies identified loci associated with IIMs. In this study, we imputed data from two prior genome-wide myositis studies and analyzed the largest myositis dataset to date to identify novel risk loci and susceptibility genes associated with IIMs and its clinical subtypes.

Vasodilator drugs and heart-related outcomes in systemic sclerosis: an exploratory analysis.

Background And Aims: Systemic sclerosis (SSc) is an autoimmune connective disease characterised by excessive extracellular matrix deposition and widespread skin and internal organ fibrosis including various cardiac manifestations. Heart involvement is one of the leading causes of death among patients with SSc. In this study, we aimed to assess the effect of various vasodilator treatments.

Targeting interleukin-6 pathways in giant cell arteritis management: A narrative review of evidence.

Giant cell arteritis (GCA) is a chronic inflammatory vasculitis with a significant impact on vascular and patient health. It may present with non-specific symptoms and can lead to severe complications if not managed effectively. This narrative review explores the treatment of GCA with interleukin-6 (IL-6) pathway inhibitors, focusing on key studies from selected databases published between 2018 and 2024.

Insights on treatment of adult ITP: algorithm for management and role of multimodal therapy.

The management of immune thrombocytopenia (ITP) is continuously evolving with the development and introduction of newer therapies and a better understanding of the disease. Corticosteroids still represent the cornerstone of first-line treatment. Patients who fail to achieve remission with a short course of corticosteroids require subsequent therapy.

Discovery and functional analysis of a novel ALPK1 variant in ROSAH syndrome.

Retinal dystrophy, optic nerve oedema, splenomegaly, anhidrosis and migraine headache (ROSAH) syndrome is an autosomal dominant disorder and to date is known to be caused by either the Thr237Met or Tyr254Cys variant in the protein kinase ALPK1. Here, we identify a family in which ROSAH syndrome is caused by a novel variant in which Ser277 is changed to Phe. All six patients examined display ocular inflammation and optic nerve elevation, four have retinal degeneration and four are registered blind.

Feasibility and acceptability of a systematic offer of HIV rapid testing to Family Planning Centers visitors by non-physician professionals in France.

Background: The Human Immunodeficiency Virus (HIV) epidemic remains active among women in Europe, with significant missed opportunities for HIV testing. Although HIV testing falls within the scope of Family Planning Centers (FPCs), it is often not offered. This pilot study assessed the feasibility and acceptability of systematically offering HIV rapid testing by non-physician professionals, independent of medical appointments, to visitors and accompanying persons in FCPs.

The Effects of Mepolizumab on CRSwNP: Real-Life Evidence.

: This study aims to evaluate the efficacy and safety of mepolizumab in the treatment of severe uncontrolled CRSwNP with or without comorbid asthma in a real-life setting over the first six months of therapy. : A total of 45 patients with nasal polyps with or without comorbid asthma were treated with mepolizumab (100 mg q4w) for 6 months. The following outcomes were assessed before therapy (V), and after 6 months (V): endoscopic nasal polyp score (NPS), nasal congestion score (NCS), sinonasal outcome test (SNOT-22), visual analog scale (VAS), nasal flow rate (PNIF), olfactory test (SS-I), and asthma control test (ACT).

2q33 Deletions Underlying Syndromic and Non-syndromic CTLA4 Deficiency.

Purpose: CTLA4 deficiency is an inborn error of immunity (IEI) due to heterozygosity for germline loss-of-function variants of the CTLA4 gene located on chromosome 2q33.2. CTLA4 deficiency underlies pleiotropic immune and lymphoproliferation-mediated features with incomplete penetrance.

Toxic Epidermal Necrolysis Induced by Doxycycline: A Case Report and Literature Review.

Lyell syndrome, also known as toxic epidermal necrolysis (TEN), is a rare but life-threatening skin condition, often triggered by certain medications. Antiepileptics, allopurinol, and some nonsteroidal anti-inflammatory drugs are the most common causes. Some antibiotics are also common culprits, although tetracyclines are rarely linked to this condition.

Real-life use of the PEXIVAS reduced-dose glucocorticoid regimen in granulomatosis with polyangiitis and microscopic polyangiitis.

Background: The PEXIVAS (Plasma exchange and glucocorticoids in severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis) trial showed that a reduced-dose glucocorticoid regimen (redGC) was non-inferior to a standard-dose regimen (standGC) with respect to death or end-stage kidney disease (ESKD) in patients with ANCA-associated vasculitis (AAV). However, the primary endpoint did not include disease progression or relapse, cyclophosphamide was the main induction therapy and rituximab (RTX)-treated patients tended to have a higher risk of death or ESKD with redGC. We aimed to evaluate the real-world use of redGC.

Autoantibodies against a subunit of mitochondrial respiratory chain complex I in inclusion body myositis.

Background: Autoantibodies are found in up to 80 % of patients with idiopathic inflammatory myopathies (IIM) and are associated with distinct clinical phenotypes. Autoantibodies targeting cytosolic 5'-nucleotidase 1A (anti-NT5C1A) are currently the only known serum biomarker for the subgroup inclusion body myositis (IBM), although detected even in other autoimmune diseases. The aim of the study was to identify new autoimmune targets in IIM.

Use of Intravenous Immunoglobulin in the Treatment of Pemphigoid Gestationis: A Case Report.

Gestational pemphigoid is a rare autoimmune skin condition specific to pregnancy and the postpartum period, with a variable course. There are currently no standardized guidelines referring to evidence-based therapeutic strategies. Intravenous immunoglobulin (IVIG) has recently emerged as a safe and effective steroid-sparing option as a second-line treatment for cases refractory to conventional steroid therapy and for managing relapses.