ACR Meeting Abstracts

ACR Meeting Abstracts

  • Home
  • Meetings Archive
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018 ACR/ARHP Annual Meeting
    • 2017 ACR/ARHP Annual Meeting
    • 2017 ACR/ARHP PRSYM
    • 2016-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • Register
    • View and print all favorites
    • Clear all your favorites
  • Meeting Resource Center

Abstract Number: 2638

The Cumulative Burden of Damage for Patients with Eosinophilic Granulomatosis with Polyangiitis

Irena Doubelt1, David Cuthbertson 2, Gunnar Tomasson 3, Simon Carette 1, Nader A. Khalidi 4, Curry L. Koening 5, Carol Langford 6, Carol A. McAlear 7, Larry W. Moreland 8, Paul Monach 9, Philip Seo 10, Ulrich Specks 11, Antoine Sreih 12, Kalen Young 13, Steven Ytterberg 11, Peter A. Merkel 14, Christian Pagnoux 1 and VCRC Vasculitis Clinical Research Consortium 15, 1Mount Sinai Hospital and University Health Network, Toronto, ON, Canada, 2University of South Florida, Tampa, FL, 3Faculty of Medicine, University of Iceland and Landpitali University Hospital, Reykjavik, Iceland, 4McMaster University, Hamilton, ON, Canada, 5University of Utah Hospital, Salt Lake City, UT, 6Cleveland Clinic, Cleveland, OH, 7University of Pennsylvania - VCRC Project Manager, Philadelphia, PA, 8University of Pittsburgh, Pittsburgh, PA, 9Brigham and Women's Hospital, Boston, MA, 10Johns Hopkins Medicine, Baltimore, MD, 11Mayo Clinic College of Medicine, Rochester, MN, 12University of Pennsylvania, Philadelphia, PA, 13Vasculitis Foundation, Kansas City, MO, 14Univeristy of Pennsylvania, Philadelphia, PA, 15University of Pennsylvania, Division of Rheumatology, philadelphia

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: ANCA and asthma, Churg-Strauss syndrome, patient outcomes, Vasculitis

  • Tweet
  • Email
  • Print
Save to PDF
Session Information

Date: Tuesday, November 12, 2019

Session Title: Vasculitis – ANCA-Associated Poster I

Session Type: Poster Session (Tuesday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by asthma and other manifestations of vasculitis, some of which can be life-threatening, cause major organ damage, or become chronic. Data on damage in EGPA is limited, mainly due to the rarity of the disease. This study aimed at describing damage in patients with EGPA enrolled in the Vasculitis Clinical Research Consortium (VCRC).

Methods: Retrospective analysis of damage in patients with EGPA participating in the VCRC Longitudinal Study (LS) or One-Time DNA (OT) studies from 2003-2019. Patients were ≥18 years of age at enrollment and fulfilled the modified American College of Rheumatology 1990 criteria. Data elements included demographics, ANCA status, use of cyclophosphamide (CYC), cumulative duration of glucocorticoid (GC) use, relapses, all items of the Vascular Damage Index (VDI). VDI is a validated, cumulative damage score, which includes 64 items, grouped by organ or system and each scoring 1 point (range, 0-64; by design, VDI is 0 at diagnosis). VDI scores and items were analyzed at enrollment (for all patients with a diagnosis made >3 months prior to enrollment, and at the farthest visit from diagnosis for patients with a follow-up >1-year post-diagnosis. For the last VDI score, associations of age, sex, CYC use, duration of GC use, and relapses with accrual of damage were explored.

Results: The cohort included 354 patients, of which 336 had a follow-up post-diagnosis >3 months. Mean age at diagnosis for the latter was 50.0 (SD ±14.1) years; 197 (58.6%) were female. Of those tested for ANCA, 132 (42.7%) were positive. A total of 112 (41.9%) LS patients received CYC at some point during their disease; mean duration of GC use was 13.7 (±20.7) months. With a mean follow-up from diagnosis for all 336 patients of 7.2 (±6.1) years, 173 (51.4%) had at least one relapse. VDI at enrollment (for the 284 patients enrolled >3 months’ post-diagnosis; mean 4.2 (±5.5) years from diagnosis), and last follow-up visit for patients with >1 year of follow-up post-diagnosis (n=268) were 2.61 (±2.17) and 3.19 (± 2.24), respectively. VDI score distributions and items for the latter are showed in Figures 1 and 2. Univariate analysis of data from the 128 LS patients with a follow-up post-diagnosis >1 year identified male sex, older age, longer duration of GC use, and having relapsed as significantly associated with higher VDI at last visit. Multivariate Poisson regression analysis retained only male sex (mean VDI of 3.80 ±0.31 in male vs. 3.0 ±0.23 in female patients; P< 0.01) and longer duration of GC use (increase of the VDI by 0.5% for each additional month of GC use; P< 0.01) as being associated with a higher last VDI.

Conclusion: Patients with EGPA accumulate substantial permanent damage from the disease and treatment, even several years after diagnosis. Strategies to attain sustained, long-term limitation of damage should be important aspects of the management of patients with EGPA.  Identification of more effective and safer treatments for EGPA are needed, especially to limit the use of GC.


fig1

Figure 1: Distribution of vasculitis damage index -VDI- scores for patients with eosinophilic granulomatosis with polyangiitis in the Vasculitis Clinical Research Consortium at enrollment, and last follow-up -for the patients with a follow-up post-diagnosis >1 year-.


fig2

Figure 2: Main items of damage at last follow-up for patients with eosinophilic granulomatosis with polyangiitis in the Vasculitis Clinical Research Consortium. 268 patients with >1 year of follow-up post-diagnosis -mean, 7.0 ± 6.2 years-.


Disclosure: I. Doubelt, None; D. Cuthbertson, None; G. Tomasson, None; S. Carette, None; N. Khalidi, None; C. Koening, None; C. Langford, Bristol-Myers Squibb, 2, GlaxoSmithKline,, 2, ChemoCentryx, 2, Genentech, 2, Bristol-Myers Squibb, 5, 9, Abbvie, 9, AstraZeneca, 9; C. McAlear, None; L. Moreland, None; P. Monach, None; P. Seo, None; U. Specks, None; A. Sreih, Bristol-Meyers Squibb, 3, Bristol-Myers Squibb, 3; K. Young, None; S. Ytterberg, None; P. Merkel, AbbVie, 5, AstraZeneca, 2, 5, Biogen, 5, Boeringher-Ingelheim, 2, 5, Bristol-Myers Squibb, 2, 5, Celgene, 2, 5, ChemoCentryx, 2, 5, CSL Behring, 5, Genentech/Roche, 2, 5, Genzyme/Sanofi, 5, GlaxoSmithKline, 2, 5, InflaRx, 5, Insmed, 5, Jannsen, 5, Kiniksa, 5, Kypha, 2, TerumoBCT, 2, UpToDate, 7; C. Pagnoux, ChemoCentryx, 5, Chemocentryx, 5, Genetech/Roche, 5, Genzyme/Sanofi, 5, GlaxoSmithKline, 5, Hoffman-La Roche, 2, 5, 8, Hoffman-LaRoche, 2, 5, 8, Sanofi, 5; V. Vasculitis Clinical Research Consortium, None.

To cite this abstract in AMA style:

Doubelt I, Cuthbertson D, Tomasson G, Carette S, Khalidi N, Koening C, Langford C, McAlear C, Moreland L, Monach P, Seo P, Specks U, Sreih A, Young K, Ytterberg S, Merkel P, Pagnoux C, Vasculitis Clinical Research Consortium V. The Cumulative Burden of Damage for Patients with Eosinophilic Granulomatosis with Polyangiitis [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/the-cumulative-burden-of-damage-for-patients-with-eosinophilic-granulomatosis-with-polyangiitis/. Accessed April 13, 2021.
  • Tweet
  • Email
  • Print
Save to PDF

« Back to 2019 ACR/ARP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-cumulative-burden-of-damage-for-patients-with-eosinophilic-granulomatosis-with-polyangiitis/

Advanced Search

Your Favorites

You can save and print a list of your favorite abstracts by clicking the “Favorite” button at the bottom of any abstract. View your favorites »

ACR Convergence: Where Rheumatology Meets. All Virtual. November 5-9.

ACR Pediatric Rheumatology Symposium 2020

© COPYRIGHT 2021 AMERICAN COLLEGE OF RHEUMATOLOGY

Wiley

  • Home
  • Meetings Archive
  • Advanced Search
  • Meeting Resource Center
  • Online Journal
  • Privacy Policy
  • Permissions Policies
loading Cancel
Post was not sent - check your email addresses!
Email check failed, please try again
Sorry, your blog cannot share posts by email.
This site uses cookies: Find out more.