Clinical Manifestations and Long‐Term Outcomes of Eosinophilic Granulomatosis With Polyangiitis in North America

Objective To describe clinical manifestations and outcomes in patients with eosinophilic granulomatosis with polyangiitis (EGPA) in North America. Methods Analysis of patients aged 18 years or older who fulfilled the 1990 American College of Rheumatology Classification Criteria for EGPA enrolled in...

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Published inACR open rheumatology Vol. 3; no. 6; pp. 404 - 412
Main Authors Doubelt, Irena, Cuthbertson, David, Carette, Simon, Chung, Sharon A., Forbess, Lindsy J., Khalidi, Nader A., Koening, Curry L., Langford, Carol, McAlear, Carol A., Moreland, Larry W., Monach, Paul A., Seo, Philip, Specks, Ulrich, Spiera, Robert F., Springer, Jason M., Sreih, Antoine G., Warrington, Kenneth J., Merkel, Peter A., Pagnoux, Christian
Format Journal Article
LanguageEnglish
Published Atlanta John Wiley & Sons, Inc 01.06.2021
John Wiley and Sons Inc
Wiley
Subjects
Asthma
Consortia
Inflammatory diseases
Laboratories
Longitudinal studies
Medical prognosis
Mortality
Original
Patients
Vein & artery diseases
France
United States > US
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Summary:Objective To describe clinical manifestations and outcomes in patients with eosinophilic granulomatosis with polyangiitis (EGPA) in North America. Methods Analysis of patients aged 18 years or older who fulfilled the 1990 American College of Rheumatology Classification Criteria for EGPA enrolled in the Vasculitis Clinical Research Consortium from 2003 to 2019. Main clinical characteristics, treatments, outcomes, and accumulated damage were studied. Results The cohort included 354 patients; 59% female; age at diagnosis of 50.0 (±14) years; 39% were antineutrophil cytoplasm antibody (ANCA) positive. Time from diagnosis to last follow‐up was 7.0 (±6.2) years; 49.4% had one or more relapse. Patients positive for ANCA more commonly had neurological and kidney involvement when compared with patients negative for ANCA, who had more cardiac and lung manifestations. At last study visit, only 35 (12.6%) patients had been off all therapy for more than 2 years during their follow‐up. The overall mortality rate was 4.0% and did not differ by ANCA status or cyclophosphamide use. Scores on the Vasculitis Damage Index (VDI) for 134 patients with two or more visits and more than 1 year of follow‐up increased from 1.7 (±1.8) at enrollment (3.7 [±5.1] years after diagnosis) to 3.35 (±2.1) at last follow‐up (7.5 [±5.8] years after diagnosis), mainly represented by chronic asthma (67.5%), peripheral neuropathy (49.6%), and chronic sinusitis (31.3%). Longer duration of glucocorticoid use and relapse were associated with higher VDI scores. Conclusion This analysis describes the many clinical manifestations and varied outcomes of EGPA and highlights the ongoing need to attain more sustained, long‐term remission to limit the accrual of disease‐related damage.
Bibliography:The Vasculitis Clinical Research Consortium (VCRC) is part of the Rare Diseases Clinical Research Network, an initiative of the Office of Rare Diseases Research, National Center for Advancing Translational Science (NCATS). The VCRC is supported by funding from NCATS, the National Institute of Arthritis and Musculoskeletal and Skin Diseases (U54 AR057319), the National Center for Research Resources (U54 RR019497), and GlaxoSmithKline.
Nader A. Khalidi, MD: McMaster University and St. Joseph’s Healthcare, Hamilton, Ontario, Canada
Ulrich Specks, MD, Kenneth J. Warrington, MD: Mayo Clinic College of Medicine and Science, Rochester, Minnesota
Sharon A. Chung, MD, MAS: University of California, San Francisco
Larry W. Moreland, MD: University of Pittsburgh, Pittsburgh, Pennsylvania
Philip Seo, MD: Johns Hopkins University, Baltimore, Maryland
David Cuthbertson, MS: Health Informatics Institute, University of South Florida, Tampa
Paul A. Monach, MD: Division of Rheumatology, Veterans Affairs Boston Healthcare System, Boston, Massachusetts
Robert F. Spiera, MD: Hospital for Special Surgery, New York, New York
Jason M. Springer, MD, MS: University of Kansas, Kansas City.
Curry L. Koening, MD, MS: University of Utah Hospital, Salt Lake City
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Irena Doubelt, MD, Simon Carette, MD, MPhil, Christian Pagnoux, MD, MSc, MPH: Vasculitis Clinic, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada
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Carol A. McAlear, MA, Antoine G. Sreih, MD, Peter A. Merkel, MD, MPH: University of Pennsylvania, Philadelphia
Carol Langford, MD, MHS: Cleveland Clinic, Cleveland, Ohio
Lindsy J. Forbess, MD: Cedars Sinai Medical Center, Los Angeles, California
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Dr. Pagnoux reports receiving fees for serving on advisory boards from ChemoCentryx, GlaxoSmithKline, Sanofi, and Hoffman‐La Roche; he also reports receiving lecture fees and research grant support from Hoffman‐La Roche and GlaxoSmithKline. Peter A. Merkel reports receiving funds in the past 2 years for consulting from AbbVie, AstraZeneca, Biogen, Boeringher‐Ingelheim, Bristol‐Myers Squibb, Celgene, ChemoCentryx, CSL Behring, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx, Insmed, Jannsen, Kiniksa, Kyverna, Magenta, Novartis, Pfizer, Sparrow, Takeda, and Talaris; for research support from AstraZeneca, Boeringher‐Ingelheim, Bristol‐Myers Squibb, Celgene, ChemoCentryx, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx; and royalties from UpToDate.
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ISSN:2578-5745
2578-5745
DOI:10.1002/acr2.11263