Video: Every Case Tells a Story| Webinar: ACR/CHEST ILD Guidelines in Practice

An official publication of the ACR and the ARP serving rheumatologists and rheumatology professionals

  • Conditions
    • Axial Spondyloarthritis
    • Gout and Crystalline Arthritis
    • Myositis
    • Osteoarthritis and Bone Disorders
    • Pain Syndromes
    • Pediatric Conditions
    • Psoriatic Arthritis
    • Rheumatoid Arthritis
    • Sjögren’s Disease
    • Systemic Lupus Erythematosus
    • Systemic Sclerosis
    • Vasculitis
    • Other Rheumatic Conditions
  • FocusRheum
    • ANCA-Associated Vasculitis
    • Axial Spondyloarthritis
    • Gout
    • Psoriatic Arthritis
    • Rheumatoid Arthritis
    • Systemic Lupus Erythematosus
  • Guidance
    • Clinical Criteria/Guidelines
    • Ethics
    • Legal Updates
    • Legislation & Advocacy
    • Meeting Reports
      • ACR Convergence
      • Other ACR meetings
      • EULAR/Other
    • Research Rheum
  • Drug Updates
    • Analgesics
    • Biologics/DMARDs
  • Practice Support
    • Billing/Coding
    • EMRs
    • Facility
    • Insurance
    • QA/QI
    • Technology
    • Workforce
  • Opinion
    • Patient Perspective
    • Profiles
    • Rheuminations
      • Video
    • Speak Out Rheum
  • Career
    • ACR ExamRheum
    • Awards
    • Career Development
  • ACR
    • ACR Home
    • ACR Convergence
    • ACR Guidelines
    • Journals
      • ACR Open Rheumatology
      • Arthritis & Rheumatology
      • Arthritis Care & Research
    • From the College
    • Events/CME
    • President’s Perspective
  • Search

2024 Research Updates in ANCA-Associated & IgA Vasculitis

Philip Seo, MD, MHS  |  Issue: October 2024  |  October 7, 2024

4. EGPA & interleukin-5 blockade.

Abstract O-046: Wechsler et al.1

Mepolizumab is the only agent approved by the U.S. Food & Drug Administration (FDA) for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA). MANDARA was a phase 3, randomized controlled trial that randomized 140 patients with relapsing or refractory EGPA to one year of treatment with either benralizumab 30 mg or mepolizumab 300 mg. Both drugs were administered subcutaneously on a monthly basis.

MANDARA demonstrated that benralizumab was non-inferior to mepolizumab for achieving remission at weeks 36 and 48 (59% vs. 56%). However, MANDARA defined remission as a Birmingham Vasculitis Activity Score (BVAS) of 0 and an oral glucocorticoid dose of no more than 4 mg daily. The present study is a post hoc analysis that endeavored to determine if the results might have differed if a more stringent definition of complete remission had been used (i.e., BVAS of 0 and no oral glucocorticoids at weeks 36 and 48). Sustained complete remission was defined as patients who achieved complete remission by week 48 and remained in complete remission by the end of week 52.

ad goes here:advert-1
ADVERTISEMENT
SCROLL TO CONTINUE

Using these new definitions, a greater proportion of patients treated with benralizumab achieved both complete remission (23.5% versus 11.1%) and sustained complete remission (35.7% versus 22.9%) compared to patients treated with mepolizumab.

Mechanistically, benralizumab is more efficient at eliminating eosinophils than mepolizumab. It is, therefore, not surprising that benralizumab was also more efficient at achieving a glucocorticoid-free remission. Importantly, this post hoc analysis highlights that most patients with EGPA treated with IL-5 blockade (either benralizumab or mepolizumab) will continue to require chronic therapy with glucocorticoids, albeit at low doses.

ad goes here:advert-2
ADVERTISEMENT
SCROLL TO CONTINUE

5. Rituximab in Adult-Onset IgA Vasculitis.

Abstract O-084: Triviol et al.1

IgA vasculitis (formerly Henoch Schonlein purpura) is widely considered refractory to immunosuppressive therapies, including cyclophosphamide. Multiple attempts have been made to explore the utility of rituximab for the treatment of IgA vasculitis, with mixed results.

This retrospective study reviewed the kidney response of patients with IgA vasculitis treated with rituximab. Approximately two-thirds of patients had relapsing disease. Most patients also received treatment with glucocorticoids and 15% also received cyclophosphamide.

At six months, 85% of patients were in remission, defined as a BVAS less than three, and 89% of patients with glomerulonephritis demonstrated evidence of improvement. Relapse occurred in 29% of patients a median of 13 months following treatment with rituximab.

Page: 1 2 3 4 | Single Page
Share: 

Filed under:Biologics/DMARDsConditionsDrug UpdatesMeeting ReportsResearch ReviewsResearch RheumVasculitis Tagged with:AAVAAV FocusRheumabataceptANCA-Associated VasculitisbenralizumabhypogammaglobulinemiaIgA vasculitismepolizumabrituximab

Related Articles

    Top Research in ANCA-Associated Vasculitis Presented at ACR Convergence 2023

    November 21, 2023

    SAN DIEGO—Vasculitis expert and former editor of The Rheumatologist, Dr. Philip Seo gives us his picks for the 10 most important abstracts in ANCA-associated vasculitis to come out of ACR Convergence 2023.

    Vasculitis Guidelines in Focus, Part 2: ANCA-Associated Vasculitis

    September 9, 2021

    Sharon Chung, MD, MAS, discusses specific recommendations for the treatment and management of ANCA-associated vasculitis from the latest ACR Guideline.

    Figure 2: Renal Biopsy

    The Classification & Diagnosis of Granulomatosis with Polyangiitis

    August 16, 2018

    Based on the classification system developed by the Chapel Hill Consensus Conference, anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis is defined as a necrotizing vasculitis involving small vessels that is associated with myeloperoxidase (MPO) ANCA or proteinase 3 (PR3) ANCA and displays minimal immune deposits. The mechanism behind the pathogenesis of ANCA-associated vasculitis is not fully…

    Maintenance of Remission in ANCA-Associated Vasculitis

    September 5, 2012

    Relapses are common, but difficult to predict and prevent.

  • About Us
  • Meet the Editors
  • Issue Archives
  • Contribute
  • Advertise
  • Contact Us
  • Copyright © 2025 by John Wiley & Sons, Inc. All rights reserved, including rights for text and data mining and training of artificial technologies or similar technologies. ISSN 1931-3268 (print). ISSN 1931-3209 (online).
  • DEI Statement
  • Privacy Policy
  • Terms of Use
  • Cookie Preferences