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.

Mycophenolate mofetil plus methotrexate versus cyclophosphamide with sequential azathioprine for treatment of Takayasu arteritis.

Objectives: Study the efficacy and safety of mycophenolate mofetil (MMF) combined with methotrexate (MTX) compared to cyclophosphamide (CYC) followed by azathioprine (AZA) to treat active Takayasu arteritis (TAK).

Methods: Adults with active TAK were randomised in a 2:1 ratio to receive oral MMF plus MTX or intravenous CYC followed by oral AZA. All subjects also received high-dose oral glucocorticoids with a predefined taper.

Longitudinal changes on cranial magnetic resonance imaging in relapsing giant cell arteritis.

Objective: There is a need for better tools to monitor disease activity in giant cell arteritis (GCA). Prior studies demonstrated that vascular enhancement on cranial vessel wall magnetic resonance imaging (vw-MRI) decreases with treatment of GCA, but whether enhancement increases during relapse is not well known. This study examined changes on vw-MRI during relapse of cranial GCA.

Treatment With Avacopan in ANCA-Associated Vasculitis With Kidney Involvement.

Introduction: Kidney disease impacts long-term outcomes of patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). This analysis evaluated the effect of avacopan in a subgroup of patients with GPA or MPA and kidney involvement at baseline from the ADVOCATE trial.

Methods: The analysis included a study population of 268 patients (of 330 patients, 81.

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 glossary of signs and symptoms of giant cell arteritis.

Objectives: This study seeks to create consensus-based definitions of signs and symptoms of giant cell arteritis (GCA) for use by health care professionals, primarily in research settings.

Methods: Core definitions of signs and symptoms of GCA were extracted from 11 randomised controlled trials of GCA previously reviewed in a systematic literature review conducted in the context of the development of response criteria for GCA. This information was supplemented by definitions from other sources, such as rheumatology textbooks.

A fair machine learning model to predict flares of systemic lupus erythematosus.

Objective: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that disproportionately affects women and racial/ethnic minority groups. Predicting disease flares is essential for improving patient outcomes, yet few studies integrate both clinical and social determinants of health (SDoH). We therefore developed FLAME (re achine learning prediction of SL), a machine learning pipeline that uses electronic health records (EHRs) and contextual-level SDoH to predict 3-month flare risk, emphasizing explainability and fairness.

Performance in adults of the EULAR/PRINTO/PRES (Ankara 2008) classification criteria for IgA vasculitis.

Objective: To examine the performance in adults of the European Alliance of Associations for Rheumatology (EULAR)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 classification criteria for IgA vasculitis (IgAV).

Methods: The EULAR/PReS/Ankara 2008 classification criteria for IgAV were applied to patients enrolled in an international observational cohort which included patients with IgAV and comparators with other forms of small-vessel and medium-vessel vasculitis. After the initial assessment of the performance of the criteria, possible revisions to increase the performance were tested.

'Am I doing this right?' Physician perceptions of the global assessment in clinical trials of systemic sclerosis.

Objectives: Physician global assessments (PhyGAs) are commonly performed in randomised controlled trials (RCTs) in systemic sclerosis (SSc). However, there is no single PhyGA applied across RCTs. We performed an exploratory qualitative study to explore perceptions of the PhyGA, its role in RCTs and how physicians perform their own assessment.

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.

Advances in the treatment of ANCA-associated vasculitis.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) consists of a group of small-vessel vasculitides that often present with organ-threatening or life-threatening manifestations. Current immunosuppressive treatments have improved survival and rates of remission, but are not curative, have frequent toxicities, and do not effectively prevent relapse. Clinical trials have established the role of rituximab, an anti-CD20 B cell-depleting monoclonal antibody, in both the remission-induction and maintenance phases of the disease and demonstrated that glucocorticoid doses can be substantially reduced from historical dosing levels without affecting treatment efficacy.

Clinical Manifestations and Treatment in Patients With Relapsing Polychondritis: A Multicenter Observational Cohort Study.

Objective: Relapsing polychondritis (RP) is a rare, heterogeneous, multisystem disease lacking standard treatment guidelines. This study describes clinical manifestations in association with approaches to treatment.

Methods: Adults with physician-diagnosed RP were recruited into a multicenter observational cohort study.

Frailty and Associated Outcomes in Patients With Vasculitis.

Objective: To describe the frequency and outcomes associated with self-reported frailty in patients with vasculitis.

Methods: VascStrong is a longitudinal study using the Vasculitis Patient-Powered Research Network, an internet-based cohort of patients with vasculitis. Data collected included patient global assessment (PtGA) and several domains of the Patient-Reported Outcomes Measurement Information System (PROMIS).

Safety of Avacopan for the Treatment of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Combined Data From Three Clinical Trials.

Objective: This study aimed to report the safety of avacopan, an oral selective complement C5a receptor antagonist, using pooled data from clinical trials in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (granulomatosis with polyangiitis [GPA] or microscopic polyangiitis [MPA]).

Methods: Data were included from two phase 2 (CLEAR [NCT01363388] and CLASSIC [NCT02222155]) and one phase 3 (ADVOCATE [NCT02994927]) double-blind randomized controlled trials comparing the safety and efficacy of avacopan with active non-avacopan control regimens to induce remission in patients with GPA or MPA. In CLEAR and ADVOCATE, avacopan-treated patients received either no or lower doses of study-supplied prednisone than the control groups; in CLASSIC, all groups received the same dose of study-supplied prednisone.

A Phase 3 Trial of Upadacitinib for Giant-Cell Arteritis.

Background: Giant-cell arteritis is a systemic vasculitis with limited treatment options. The efficacy and safety of upadacitinib - a selective Janus kinase (JAK) inhibitor that blocks the signaling of several cytokines, including interleukin-6 and interferon-γ - are unknown in patients with giant-cell arteritis.

Methods: We randomly assigned patients with new-onset or relapsing giant-cell arteritis, in a 2:1:1 ratio, to receive upadacitinib at a dose of 15 mg or 7.

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.

Long-Term Safety and Efficacy of Mepolizumab in Eosinophilic Granulomatosis With Polyangiitis.

Objective: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, relapsing, inflammatory disease. Management of EGPA predominantly relies on oral corticosteroids (OCS), which are associated with many adverse effects. The phase 3 MIRRA trial demonstrated efficacy and safety of mepolizumab, anti-interleukin-5 biologic, for EGPA.

Treatment With Avacopan in Patients With Respiratory Tract Manifestations of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Objective: The ADVOCATE trial demonstrated that treatment of active granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) with avacopan was noninferior in achieving remission at week 26 and superior for sustained remission at week 52 compared with a prednisone taper. This analysis of ADVOCATE evaluated the efficacy and safety of avacopan in patients with ear, nose, throat (ENT), or lung manifestations.

Methods: This post hoc analysis included patients enrolled in ADVOCATE with ENT or lung manifestations at baseline.

The effects of plasma exchange and glucocorticoids on early kidney function among patients with ANCA-associated vasculitis in the PEXIVAS trial.

Therapeutic plasma exchange (PLEX) is an adjunctive treatment for patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and kidney involvement. Little is known about the effect of PLEX on early changes in kidney function. This post-hoc analysis of the PEXIVAS trial investigated the effects of PLEX on changes in kidney function within 12 months.

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.

Vessel wall MRI in giant cell arteritis: standardized protocol and scoring approach developed by an international working group.

Objectives: There are an increasing number of centres performing research on high-resolution vessel wall magnetic resonance imaging (VW-MRI) in GCA. However, harmonized approaches to VW-MRI in GCA are lacking and are essential to performing multicentre studies. Using a data-driven, consensus-based approach, an international expert group developed a standardized MRI protocol and scoring system to advance multi-centred research in cranial GCA.

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.