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Lookup NU author(s): Professor Christopher WardORCiD
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© 2021, Irish Medical Association. All rights reserved.Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, small-to-medium vessel vasculitis presenting most commonly with upper and lower airway symptoms and a peripheral blood eosinophilia (PBE)1. EGPA is highly variable in clinical expression and can be diagnostically challenging as the syndrome slowly evolves over time. Methods The aim of this study was to determine the American College of Rheumatology diagnostic (ACR) criteria score2 in a cohort of patients with EGPA and to describe their treatment and clinical outcomes. Results The mean age at diagnosis was 53 ± 12.2 years with an average time in clinic of 1.1 years prior to diagnosis. All patients had ≥4 ACR criteria. All 15 had sinusitis and 14 (93%) lung infiltrates, asthma and >10% PBE. 7 patients (47%) had mono/polyneuropathy and two (13%) had a positive biopsy. One patient had a stroke. 9 patients (60%) remained in remission with a prednisolone/ methotrexate combination, two (13%) prednisolone alone, two patients (13%) with azathioprine, one patient required prednisolone and mepolizumab to attain control and one unstable patient on prednisolone /methotrexate due to start mepolizumab repatriated to eastern Europe. Conclusion Clinicians should be aware of the possibility of EGPA in a patient with unstable adult-onset asthma and sinusitis and significant PBE.
Author(s): McDermott CM, Harrison MJ, Ward C, Doyle D, O'regan AW, Rutherford RM
Publication type: Article
Publication status: Published
Journal: Irish Medical Journal
Year: 2021
Volume: 114
Issue: 10
Print publication date: 01/11/2021
Acceptance date: 02/04/2020
ISSN (print): 0332-3102
Publisher: Irish Medical Association
URL: https://imj.ie/eosinophilic-granulomatosis-with-polyangiitis/
