Epidemiology, clinical management, and outcomes in patients with eosinophilic granulomatosis with polyangiitis in England: A retrospective observational cohort study.

Authors

Salman Siddiqui, Bo Ding, Paul Dolin, Chris Edmonds, Priya Jain, Jennifer Rowell, Lotte Westerink, Alessandra Lacetera, Pablo Suárez-Sánchez, Cono Ariti, Bélène Podmore, Alvaro Kitchin Velarde, Stephanie Y Chen

Published in

The journal of allergy and clinical immunology. Global. Volume 5. Issue 5. Pages 100740. Epub Jun 01, 2026.

Abstract

Data on the clinical burden of eosinophilic granulomatosis with polyangiitis (EGPA) are limited.
We sought to evaluate the epidemiology and clinical burden of EGPA in England using real-world evidence.
Patients diagnosed with EGPA between January 1, 2006, and February 28, 2019, who had ≥1 year of data before diagnosis (index date) were identified using the Clinical Practice Research Datalink Aurum database. Epidemiology, diagnosis, mortality, treatment, and clinical outcomes were assessed.
The incident and prevalent EGPA cohorts comprised 486 and 729 patients, respectively. The overall incidence and prevalence of EGPA were 3.04 (95% CI: 2.77-3.32) cases per million person-years and 2.7 (95% CI: 2.5-2.9) cases per 100,000 persons, respectively. Overall, 76.3% and 26.1% of patients had a Five Factor Score of 0 on the 1996 and 2009 versions. In the incident cohort (mean age 57.9 ± 15.2 years), most patients (97.1%) had ≥1 comorbidity; 79.8% had asthma coded. The median time from first major manifestation to EGPA diagnosis was 44.0 (Q1-Q3: 20.0-56.0) months. The death rate was 37.1 per 1000 person-years (95% CI: 30.1-45.2); the standardized mortality ratio for all-cause deaths was 2.3 (95% CI: 1.9-2.8). The 5-year survival rate was 82.3% (95% CI: 78.1%-85.7%). Most patients (86.2%) received oral glucocorticoids, of whom 27.0% successfully tapered. Six months post index date, 26.1% of patients had a new EGPA manifestation.
This study emphasizes the substantial clinical burden and reliance on glucocorticoids in EGPA, highlighting the need for improved diagnosis of this disorder.

PMID:
42405355
Bibliographic data and abstract were imported from PubMed on 06 Jul 2026.

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