Discontinuation of Oral Glucocorticoids and Achievement of Remission in Patients With Eosinophilic Granulomatosis With Polyangiitis Treated With Benralizumab or Mepolizumab.

Objective: In the phase 3 head-to-head MANDARA study (NCT04157348), benralizumab demonstrated noninferiority to mepolizumab in inducing remission (defined as Birmingham Vasculitis Activity Score [BVAS] of 0 and oral glucocorticoid [OGC] dosage ≤4 mg/day at weeks 36 and 48) in patients with eosinophilic granulomatosis with polyangiitis (EGPA). This analysis investigated a more stringent definition of remission that included discontinuation of OGCs and being relapse-free.

Methods: Patients aged ≥18 years with documented relapsing or refractory EGPA receiving OGCs at ≥7.

Two-year efficacy and safety of anti-interleukin-5/receptor therapy for eosinophilic granulomatosis with polyangiitis.

Objectives: To summarise the efficacy and safety of 2 years of anti-interleukin-5/receptor (anti-IL-5/R) therapy in patients with eosinophilic granulomatosis with polyangiitis (EGPA).

Methods: Patients were randomised 1:1 to receive benralizumab or mepolizumab every 4 weeks during the 52-week double-blind period of the MANDARA trial. Patients entered an open-label extension (OLE) in which they continued benralizumab (benralizumab/benralizumab) or switched from mepolizumab to benralizumab (mepolizumab/benralizumab).

A Randomized, Double-Blind, Placebo-Controlled Trial of Abatacept for the Treatment of Relapsing, Nonsevere Granulomatosis With Polyangiitis.

Objective: To compare the efficacy of abatacept to placebo for the treatment of relapsing, nonsevere granulomatosis with polyangiitis (GPA).

Methods: In this multicenter trial, eligible patients with relapsing, nonsevere GPA were randomized to receive abatacept 125 mg subcutaneously once a week or placebo, both together with prednisone 30 mg/day (or equivalent), tapered and discontinued at week 12. Patients already taking methotrexate, azathioprine, mycophenolate, or leflunomide continued this medication at a stable dose.

Aging in Granulomatosis with Polyangiitis and Microscopic Polyangiitis: From Pathophysiology to Clinical Management.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) predominantly affect individuals aged 55-75 years, with granulomatosis with polyangiitis (GPA) being diagnosed most often between 55 and 65 years and microscopic polyangiitis (MPA) between 65 and 75 years. Owing to the general increase in life expectancy, the average age at diagnosis increases, encompassing also those over 75 years old. Unfortunately, the exclusion of these older patients from many clinical trials has limited our understanding of the progression of these diseases in older subjects.

Data-Driven Clustering Approach to Identify Different Phenotypes of Primary Central Nervous System Vasculitis.

Background: To determine whether hierarchical unsupervised cluster analysis identifies a phenotypic distinction in adult patients with primary CNS vasculitis (PCNSV).

Methods: An agglomerative hierarchical cluster analysis based on the Ward method was conducted, including 153 patients with complete baseline phenotypic characterization in the COVAC' registry.

Results: The hierarchical analysis identified two main clusters.

Patients with vasculitis present a poor oral health: results of the online cross-sectional survey from Canada (VASC-TOOTH Survey).

To describe oral health complications and related quality of life in patients with vasculitis. Survey to assess clinical variables, pertinent to oral and dental health in patients with vasculitis and using the Oral Health Impact Profile 14 (OHIP14) questionnaire. 226 patients answered the survey.

Efficacy and safety of avacopan in patients aged 65 years and older with ANCA-associated vasculitis: a post hoc analysis of data from the ADVOCATE trial.

Objectives: To evaluate the efficacy and safety of avacopan in patients aged ≥65 years with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) in the phase 3 ADVOCATE trial of avacopan vs a prednisone taper, plus either rituximab or cyclophosphamide.

Methods: In this descriptive, post hoc analysis, patients receiving avacopan or a prednisone taper were stratified by age. Key efficacy outcomes included the rate of remission at week 26 and sustained remission at week 52.

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.

A study of implementation factors for a novel approach to clinical trials: constructs for consideration in the coordination of direct-to-patient online-based medical research.

Background: Traditional medical research infrastructures relying on the Centers of Excellence (CoE) model (an infrastructure or shared facility providing high standards of research excellence and resources to advance scientific knowledge) are often limited by geographic reach regarding patient accessibility, presenting challenges for study recruitment and accrual. Thus, the development of novel, patient-centered (PC) strategies (e.g.

Social determinants of health as barriers to care for vasculitis: perspectives of patients and healthcare providers.

Objective: This study examined patient and healthcare provider (HCP) perspectives on the impact of unmet social needs on healthcare barriers for patients with vasculitis.

Methods: Two surveys were developed to gather perspectives from patients with vasculitis, and HCPs specializing in vasculitis care. The patient survey also included a 20-question social needs assessment.

Glucocorticoid Minimization in Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: An International Survey of Clinicians.

Rationale & Objective: Research in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) has focused on reducing treatment toxicities, notably through reduction of exposure to glucocorticoids. Glucocorticoid-sparing therapies such as avacopan are not widely available in many countries, and patients are exposed to high glucocorticoid doses. There is little data concerning what clinicians should accept as the lowest glucocorticoid dosing that can be used in induction therapy for AAV.

Optic nerve sheath measurement to monitor disease activity in giant cell arteritis: a pilot study.

Introduction/objectives: Optic nerve sheath (ONS) enhancement using magnetic resonance imaging of the orbits was observed in patients with giant cell arteritis (GCA). We previously showed that ONS diameter (ONSD) by bedside ultrasound is increased in patient with active GCA. This study aims to assess whether ONSD decreases with clinical remission in patients with GCA.

Signal Regulatory Protein α Expression in Systemic Vasculitis.

Objective: Signal regulatory protein α (SIRPα) is found primarily on myeloid cells, including macrophages and neutrophils; binds to CD47; and regulates phagocytosis, antigen presentation, cellular fusion, cell proliferation, and migration. Therefore, SIRPα may be involved in the pathogenesis of autoimmune diseases, including systemic vasculitis. This study aimed to assess SIRPα expression in tissue samples from patients with vasculitis.

Antiplatelet therapy to prevent ischemic events in giant cell arteritis: protocol for a systematic review and meta-analysis.

Background: Giant cell arteritis (GCA) is the most common systemic vasculitis in adults. Presenting features include new-onset headaches, constitutional symptoms, jaw claudication, polymyalgia rheumatica, and visual symptoms. Arterial inflammation with subsequent stenosis and occlusion may cause tissue ischemia, leading to blindness, strokes, and myocardial infarction.

Assessment of damage in Takayasu's arteritis.

Objectives: To evaluate damage and clinical characteristics associated with damage in Takayasu's arteritis (TAK).

Methods: Patients with TAK enrolled in a multicentre, prospective, observational study underwent standardized damage assessment every 6 months using the Vasculitis Damage Index (VDI) and the Large-Vessel Vasculitis Index of Damage (LVVID).

Results: The study included 236 patients with TAK: 92% female, 81% Caucasian; median (25th, 75th percentile) disease duration = 2.

Aneurysmal Disease in Patients With Takayasu Arteritis.

Objective: Takayasu arteritis (TA) leads to stenotic disease. Aneurysmal lesions are rarer. This study assessed the main characteristics of aneurysmal disease in a Canadian cohort of patients with TA.