Opportunities and limitations of B cell depletion approaches in SLE.

B cell depletion with rituximab, a chimeric monoclonal antibody that selectively targets B cells by binding CD20, has been used off label in severe and resistant systemic lupus erythematosus (SLE) for over two decades. Several biological mechanisms limit the efficacy of rituximab, including immunological reactions towards the chimeric molecule, increased numbers of residual B cells, including plasmablasts and plasma cells, and a post-treatment surge in B cell-activating factor (BAFF) levels. Consequently, rituximab induces remission in only a proportion of patients, and safety issues limit its use.

Clinical practice recommendations for the diagnosis and management of X-linked hypophosphataemia.

X-linked hypophosphataemia (XLH) is a rare metabolic bone disorder caused by pathogenic variants in the PHEX gene, which is predominantly expressed in osteoblasts, osteocytes and odontoblasts. XLH is characterized by increased synthesis of the bone-derived phosphaturic hormone fibroblast growth factor 23 (FGF23), which results in renal phosphate wasting with consecutive hypophosphataemia, rickets, osteomalacia, disproportionate short stature, oral manifestations, pseudofractures, craniosynostosis, enthesopathies and osteoarthritis. Patients with XLH should be provided with multidisciplinary care organized by a metabolic bone expert.

Autologous haematopoietic stem cell transplantation for treatment of multiple sclerosis and neuromyelitis optica spectrum disorder - recommendations from ECTRIMS and the EBMT.

Autologous haematopoietic stem cell transplantation (AHSCT) is a treatment option for relapsing forms of multiple sclerosis (MS) that are refractory to disease-modifying therapy (DMT). AHSCT after failure of high-efficacy DMT in aggressive forms of relapsing-remitting MS is a generally accepted indication, yet the optimal placement of this approach in the treatment sequence is not universally agreed upon. Uncertainties also remain with respect to other indications, such as in rapidly evolving, severe, treatment-naive MS, progressive MS, and neuromyelitis optica spectrum disorder (NMOSD).

Two-photon NAD(P)H-FLIM reveals unperturbed energy metabolism of Ascaris suum larvae, in contrast to host macrophages upon artemisinin derivatives exposure.

Soil-transmitted helminths (STH) are widespread, with Ascaris lumbricoides infecting millions globally. Malaria and STH co-infections are common in co-endemic regions. Artemisinin derivatives (ARTs)-artesunate, artemether, and dihydroartemisinin-are standard malaria treatments and are also known to influence the energy metabolism of parasites, tumors, and immune cells.

Melanoma bone metastasis-induced osteocyte ferroptosis via the HIF1α-HMOX1 axis.

Osteocytes are the main cells in mineralized bone tissue. Elevated osteocyte apoptosis has been observed in lytic bone lesions of patients with multiple myeloma. However, their precise contribution to bone metastasis remains unclear.

Serum Biomarkers of Pulmonary Damage and Risk for Progression of Rheumatoid Arthritis-Associated Interstitial Lung Disease.

Objective: To investigate baseline and change of pulmonary damage biomarkers (serum Krebs von den Lungen 6 [KL-6], human surfactant protein D [hSP-D], and matrix metalloproteinase 7 [MMP-7]) with rheumatoid arthritis-associated interstitial lung disease (RA-ILD) progression.

Methods: In the Korean Rheumatoid Arthritis Interstitial Lung Disease (KORAIL) cohort, a prospective cohort, we enrolled patients with RA and ILD confirmed by chest computed tomography imaging and followed annually. ILD progression was defined as worsening in physiological and radiological domains of the 2022 American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society guideline for progressive pulmonary fibrosis (PPF).

Epidemiology of Antisynthetase Syndrome and Risk of Malignancy in a Population-based Cohort (1998-2019).

Objective: Population-based epidemiology studies about antisynthetase syndrome (ASSD) are lacking. Our aims were to determine the incidence and prevalence of ASSD and assess malignancy risk among patients following ASSD diagnosis.

Methods: A retrospective, population-based cohort of adults with incident ASSD residing in Olmsted County, Minnesota, in 1998-2019 was assembled.

Psychometric Evaluation of the Scleroderma Skin Questionnaire: A Novel Patient Reported Outcome for Skin Disease in Patients with Systemic Sclerosis.

Objective: To evaluate the psychometric properties of the Scleroderma Skin Questionnaire (SSQ), a novel patient-reported outcome (PRO) to assess systemic sclerosis (SSc) related skin symptoms.

Methods: The SSQ was administered to 799 adults (mean age 52.7; 82% female) enrolled in the SSc Collaborative National Quality and Efficacy Registry (CONQUER).

Effect of Characteristic Inflammatory and Structural Pelvic Magnetic Resonance Imaging Lesions on Expert Assessment of Axial Juvenile Spondyloarthritis.

Objective: To evaluate the influence of pelvic magnetic resonance imaging (MRI) findings on axial disease assessment in juvenile spondyloarthritis (JSpA).

Methods: This was a cross-sectional study of patients with JSpA with suspected axial disease. Three experts reviewed each case and rated their confidence (-3 to +3) in the presence of axial disease, first with clinical data and second with clinical and MRI data.

Clinical characteristics and influencing factors of hyperkyphosis in a Chinese Cohort with axial spondyloarthritis: a multicentre retrospective study.

Objective: This study aimed to investigate the clinical characteristics and influencing factors of axial spondyloarthritis (axSpA) hyperkyphosis in a Chinese cohort.

Methods: A cross-sectional study was conducted on 607 patients with axSpA attending 12 hospitals across 11 centers from March 2022 to March 2024.Univariate and multivariate logistic regression analyses were used to explore the relevant influencing factors of hyperkyphosis.

Pulmonary Arterial Hypertension Incidence in Scleroderma Patients Treated with Bosentan for Digital Ulcers: Evidence from the Italian SPRING Registry.

Objective: Bosentan (BOS) is approved for treating pulmonary arterial hypertension (PAH) and preventing digital ulcers (DU) in systemic sclerosis (SSc). Our study aimed to evaluate whether BOS prescribed for DU could reduce the incidence of PAH in a large SSc cohort from the SPRING registry.

Methods: Patients with SSc from the SPRING registry, meeting ACR/EULAR 2013 classification criteria with data on PAH onset, DU status, BOS exposure, and at least a one-year follow-up between 2015 and 2020, and no known PAH at baseline were included.

IP-to-MS: An Unbiased Workflow for Antigen Profiling.

Immunoprecipitation is among the most widely utilized methods in biomedical research, with applications that include the identification of antibody targets and associated proteins. The path to identifying these targets is not straightforward, however, and often requires the use of chemical cross-linking and/or gel electrophoresis to separate targets from an overabundance of immunoglobulin protein. Such experiments are labor intensive and often yield long lists of candidate antibody targets.

Evaluation of the Reliability of ChatGPT to Provide Guidance on Recombinant Zoster Vaccination for Patients With Rheumatic and Musculoskeletal Diseases.

Introduction: Large language models (LLMs) such as ChatGPT can potentially transform the delivery of health information. This study aims to evaluate the accuracy and completeness of ChatGPT in responding to questions on recombinant zoster vaccination (RZV) in patients with rheumatic and musculoskeletal diseases.

Methods: A cross-sectional study was conducted using 20 prompts based on information from the Centers for Disease Control and Prevention (CDC), the Advisory Committee on Immunization Practices (ACIP), and the American College of Rheumatology (ACR).

Larger sample sizes are needed when developing a clinical prediction model using machine learning in oncology: methodological systematic review.

Background: Having a sufficient sample size is crucial when developing a clinical prediction model. We reviewed details of sample size in studies developing prediction models for binary outcomes using machine learning (ML) methods within oncology and compared the sample size used to develop the models with the minimum required sample size needed when developing a regression-based model (N).

Methods: We searched the Medline (via OVID) database for studies developing a prediction model using ML methods published in December 2022.

Exploring the use and usefulness of living guidelines for consumers: international online survey of patients' and carers' views.

Background: Living guidelines contain continually updated, and potentially changing, clinical recommendations. The implications of living guidelines for consumers (e.g.

The structural organisation of pentraxin-3 and its interactions with heavy chains of inter-α-inhibitor regulate crosslinking of the hyaluronan matrix.

Pentraxin-3 (PTX3) is an octameric protein, comprised of eight identical protomers, that has diverse functions in reproductive biology, innate immunity and cancer. PTX3 interacts with the large polysaccharide hyaluronan (HA) to which heavy chains (HCs) of the inter-α-inhibitor (IαI) family of proteoglycans are covalently attached, playing a key role in the (non-covalent) crosslinking of HC•HA complexes. These interactions stabilise the cumulus matrix, essential for ovulation and fertilisation in mammals, and are also implicated in the formation of pathogenic matrices in the context of viral lung infections.

Anakinra improves retention rate of targeted treatments in Erdheim-Chester disease.

Objective: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis that exhibits a wide spectrum of clinical manifestations. The recent identification of activating mutations in the MAPK-ERK pathway in patients with ECD has led to the introduction of targeted therapies. The most commonly used targeted therapies are BRAF- and MEK-inhibitors, which are highly effective but also carry significant toxicity.

GTS-21 modulates rheumatoid arthritis Th17 and Th2 lymphocyte subset differentiation through the ɑ7nAch receptor.

Previous research has demonstrated ɑ7nAch receptor (ɑ7nAchR) agonists to provide benefit for rheumatoid arthritis (RA) patients. However, the immunological mechanism of action for these ɑ7nAchR agonists has not been elucidated. Herein, the effect of GTS-21, a selective ɑ7nAchR agonist, on the differentiation of Th17 and Th2 cells was assessed.

Translation, cross-cultural adaptation and psychometric evaluation of the Serbian Ankylosing Spondylitis Quality of Life (ASQoL) Questionnaire (refers to r-axSpA) and its relations with disease activity and functional status indices.

Objectives: To translate, cross-culturally adapt and validate the Serbian Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire, e.g. according to the new nomenclature Radiographic-Axial Spondyloarthritis (r-axSpA), and to relate it to disease activity and functional status domains.

Late-onset Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune rheumatic disease (ARD) that results from the dysregulation of multiple innate and adaptive immune pathways. Late-onset SLE (Lo-SLE) is the term used when the disease is first diagnosed after 50-65 years, though the standard age cut-off remains undefined. Defining "late-onset" as lupus with onset after 50 years is more biologically plausible as this roughly corresponds to the age of menopause.

Mutations disrupting the kinase domain of IKKα lead to immunodeficiency and immune dysregulation in humans.

IKKα, encoded by CHUK, is crucial in the non-canonical NF-κB pathway and part of the IKK complex activating the canonical pathway alongside IKKβ. The absence of IKKα causes fetal encasement syndrome in humans, fatal in utero, while an impaired IKKα-NIK interaction was reported in a single patient and causes combined immunodeficiency. Here, we describe compound heterozygous variants in the kinase domain of IKKα in a female patient with hypogammaglobulinemia, recurrent lung infections, and Hay-Wells syndrome-like features.

Assessing Clinical Improvement of Infants Hospitalized for Respiratory Syncytial Virus-Related Critical Illness.

Background: Pediatric respiratory syncytial virus (RSV)-related acute lower respiratory tract infection (LRTI) commonly requires hospitalization. The Clinical Progression Scale Pediatrics (CPS-Ped) measures level of respiratory support and degree of hypoxia across a range of disease severity, but it has not been applied in infants hospitalized with severe RSV-LRTI.

Methods: We analyzed data from a prospective surveillance registry of infants hospitalized for RSV-related complications across 39 U.

A Study on the Persistence of Immunity Following Two-Dose Varicella Vaccination With Varying Intervals in Chinese Children Aged 2-7 years.

Varicella, a highly contagious disease caused by the varicella-zoster virus (VZV), remains prevalent in China despite the introduction of the varicella vaccine in 1997. The current vaccination protocol in China involves a voluntary, self-funded single-dose regimen. This study aims to investigate the longevity of immune response in Chinese children following two-dose varicella vaccination administered at different intervals, with the objective of optimizing vaccination strategies.

Differences in myocardial involvement between new onset and longstanding systemic lupus erythematosus patients assessed by cardiovascular magnetic resonance.

Objectives: Subclinical myocardial involvement is common in systemic lupus erythematosus (SLE), but differences between new onset and longstanding SLE are not fully elucidated. This study compared myocardial involvement in new onset versus longstanding SLE using cardiovascular magnetic resonance (CMR).

Materials And Methods: We prospectively enrolled 24 drug-naïve new onset SLE patients, 27 longstanding SLE patients, and 20 healthy controls.