ArticleACR open rheumatology2021
Clinical Manifestations and Long-Term Outcomes of Eosinophilic Granulomatosis With Polyangiitis in North America.
Article in ACR open rheumatology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers, 2 of them syntheses that pooled it.
What it found
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
32 citing papers in PubMed, 2 syntheses or guidelines pooled it.
- Guideline
- Pooled it
- Long-Term Safety and Efficacy of Mepolizumab in Eosinophilic Granulomatosis With Polyangiitis.Arthritis & rheumatology (Hoboken, N.J.) · 2025Trial
- Advances in the treatment of eosinophilic granulomatosis with polyangiitis.Nature reviews. Rheumatology · 2026Review
- Chronic Rhinosinusitis and Nasal Polyps in Eosinophilic Granulomatosis With Polyangiitis: A Scoping Review.International forum of allergy & rhinology · 2026Article
- Epidemiology, clinical management, and outcomes in patients with eosinophilic granulomatosis with polyangiitis in England: A retrospective observational cohort study.The journal of allergy and clinical immunology. Global · 2026Article
- Review
- Association of rheumatoid factor with vasculitic manifestations and distinct clinical patterns in eosinophilic granulomatosis with polyangiitis: a multicenter observational study.Rheumatology international · 2026Observational
- Diagnosis and Treatment Pathway for Eosinophilic Granulomatosis with Polyangiitis in the Allergy Department.Diagnostics (Basel, Switzerland) · 2026Review
- Clinical characteristics and predictors of in-hospital mortality in patients with ANCA-associated vasculitis complicated by diffuse alveolar hemorrhage: a retrospective cohort study.Frontiers in medicine · 2026Article
- Glucocorticoid discontinuation in eosinophilic granulomatosis with polyangiitis treated with mepolizumab: associations with tapering patterns and initiation timing.Frontiers in immunology · 2026Article
- Challenges in the diagnosis, classification and prognosis of ANCA-associated vasculitis.Nature reviews. Rheumatology · 2025Review
- Patients' perspectives of eosinophilic granulomatosis with polyangiitis within the context of a clinical trial.EULAR rheumatology open · 2025Article
- The Way Back from Tetraplegia or, Rare Neurological Manifestations of Eosinophil Granulomatosis with Polyangiitis.Journal of clinical medicine · 2025Article
- High Disease Burden and Oral Corticosteroid Use in Patients with Hypereosinophilic Syndrome and Eosinophilic Granulomatosis with Polyangiitis: Country-Level Insights into Real-World Management in Europe.Journal of clinical medicine · 2025Article
- Two unique cases of eosinophilic granulomatosis with polyangiitis in childhood treated with anti-interleukin-5 therapy: infantile-onset and submandibular salivary gland involvement.Pediatric rheumatology online journal · 2025Article
- Solo Addition of Mepolizumab Turned Antineutrophil Cytoplasmic Antibody Negative and Achieved Glucocorticoid Discontinuation in a Patient with Eosinophilic Granulomatosis with Polyangiitis.Internal medicine (Tokyo, Japan) · 2025Article
- Eosinophilic granulomatosis with polyangiitis: Patient profiles from a large US allergy practice.The journal of allergy and clinical immunology. Global · 2025Article
- Review
- Clinical value at baseline and follow-up of myeloperoxidase-antibodies in ANCA-associated vasculitis.Frontiers in immunology · 2025Article
Corrections and comments
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Authors and funding
20 authors.
Funding
Abstract
objectiveTo describe clinical manifestations and outcomes in patients with eosinophilic granulomatosis with polyangiitis (EGPA) in North America.
methodsAnalysis 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.
resultsThe 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.
conclusionThis 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.
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Registered trials
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