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

Stay the Course: How to Maintain Remission in Vasculitis

Jason Liebowitz, MD, FACR  |  September 9, 2019

MADRID—During the 2019 European Congress of Rheumatology (EULAR), held June 12–15, speakers addressed the complexity of vasculitis in a Challenges in Clinical Practice session titled, How to Maintain Remission in Vasculitis.

Although vasculitis appears in many varieties, one commonality exists among these related yet distinct conditions: When there is vital organ or systemic involvement, disease progression can be rapid and life threatening. Example: The five-year survival rate for patients in the French Vasculitis Study Group with systemic necrotizing vasculitides, including polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), was only 72% through 1980. However, these rates have improved over the years, with 94% of patients in this cohort who were diagnosed after 2010 surviving five years.1 Additionally, research in the U.S. has shown declining in-hospital mortality and mortality due to end-stage renal disease in GPA since the 1990s.2,3

ad goes here:advert-1
ADVERTISEMENT
SCROLL TO CONTINUE

Thus, rheumatologists are left with the difficult task of correctly identifying and treating systemic vasculitis while avoiding the long-term side effects of treatment, such as infection, osteoporosis and negative consequences of corticosteroid therapy. They must also evaluate how best to maintain disease remission in patients.

Treatment Options
Loïc Guillevin, MD, professor of medicine and therapeutics, Hôpital Cochin, Université Paris Descartes, France, began the session. He sought to answer several important questions: What is the ideal induction treatment for anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis, and what is the effect on maintenance treatment? Do patients with ANCA-associated vasculitis need maintenance treatment, and if so, what treatment? How long should patients with these conditions be treated, and what should long-term treatment entail?

ad goes here:advert-2
ADVERTISEMENT
SCROLL TO CONTINUE

Traditionally, remission in ANCA-associated vasculitis has been achieved with the use of cyclophosphamide and corticosteroids. More recently, rituximab, along with corticosteroids, has become a potential treatment option. In GPA without renal involvement, methotrexate or azathioprine are viable treatment strategies. In some cases, such as EGPA without systemic involvement or signs of poor prognosis, corticosteroids can be used as monotherapy. Prof. Guillevin noted that although remission can be initially achieved with azathioprine, methotrexate or corticosteroid monotherapy, relapse rates are high when treatment is stopped early. Therefore, when immunosuppressants are prescribed to induce remission, a maintenance agent is compulsory.

It’s an exciting time in the area of vasculitis treatment & research, & the next frontier in the field may be devising the best methods to induce & monitor for disease remission.

With regard to cyclophosphamide, the Cyclophosphamide vs. Azathioprine for Early Remission Phase of Vasculitis (CYCAZAREM) trial demonstrated that in patients with generalized vasculitis, withdrawal of cyclophosphamide and substitution of azathioprine after remission did not increase the rate of relapse. This finding indicates that extended treatment with cyclophosphamide is not necessary for maintenance of remission.4 With regard to methotrexate and azathioprine for maintenance of remission in ANCA-associated vasculitis, the two medications were shown to be similar in efficacy in the Wegener’s Granulomatosis–Entretien (WEGENT) trial. But relapse-free survival at 36 months was only 69% in the methotrexate group and 64% in the azathioprine group.5

Page: 1 2 3 | Single Page
Share: 

Filed under:ConditionsEULAR/OtherMeeting ReportsVasculitis Tagged with:EULARgiant cell arteritis (GCA)RemissionrituximabVasculitis

Related Articles

    Maintenance of Remission in ANCA-Associated Vasculitis

    September 5, 2012

    Relapses are common, but difficult to predict and prevent.

    Two Inflammatory Conditions—Polymyalgia Rheumatica and Giant Cell Arteritis—Share Clinical Connection

    March 1, 2013

    Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) have common clinical and epidemiologic links, but they need not occur synchronously

    Updates on Giant Cell Arteritis

    March 19, 2018

    SAN DIEGO—Recent research tells us more about giant cell arteritis (GCA) to help rheumatologists more accurately diagnose and effectively treat patients with this type of vasculitis. On Nov. 6 at the ACR/ARHP Annual Meeting, three experts explored the latest findings on GCA pathogenesis, diagnostic approaches, imaging modalities and growing treatment options. GCA: What’s Really Happening?…

    Case Report: Giant Cell Arteritis-Related Stroke

    September 10, 2023

    Thromboembolic events are major contributors to the morbidity and mortality of patients with giant cell arteritis (GCA), but little is known about how GCA may increase the risk of ischemic strokes. GCA-related stroke is described as an ischemic cerebral infarct occurring within three to four weeks of GCA diagnosis and treatment. It occurs in 3–7%…

  • 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