AB1238 DIVERSE CLINICAL MANIFESTATIONS OF EGPA: A SYSTEMATIC REVIEW
Authors: J. Rowell, S. Lucas, P. Dolin
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Eosinophilic granulomatosis with polyangiitis (EGPA), previously referred to as Churg-Strauss syndrome, is a rare, systemic necrotising vasculitis associated with blood and tissue eosinophilia, extravascular granulomas and asthma. It is the rarest of the antineutrophil cytoplasmic antibody-associated vasculitides. EGPA is highly heterogenous, and patients experience a wide variety of clinical manifestations that impact multiple organ systems simultaneously. <h3>Objectives:</h3> To describe and quantify the burden of the clinical manifestations of EGPA from contemporary literature. <h3>Methods:</h3> A comprehensive search strategy was conducted in Ovid MEDLINE and Ovid Embase from January 2019 to June 2023 without language restrictions. In addition, hand searches were conducted for relevant conference proceedings and systematic literature review bibliographies from 2020 to 2023. Records were independently screened by two reviewers at title/abstract and full-text level against pre-specified PICOS criteria. The Joanna Briggs Institute critical appraisal tools [1] were used to conduct risk of bias assessments on each full-text study. This review has been registered with PROSPERO (registration number: CRD42023432025).[2] <h3>Results:</h3> From a total of 1,815 records, 90 publications were included, which reported 69 unique studies. Asthma was the most frequently observed clinical manifestation; an average of 85.5% of patients with EGPA were reported as having asthma (range 42.3–100%; n = 20 studies). Two studies defined asthma severity based on the Global Initiative for Asthma (GINA) steps and reported on average 56.3% of patients (pooled n = 174) had severe asthma (GINA step 5), while in one study only 20% patients had mild asthma (GINA steps 1 and 2). A high proportion of patients were also affected by ear, nose and throat manifestations (average of 75.8% of patients, range 34.2–100%; n = 19 studies) and other lung manifestations (average of 74.2% of patients, range 14.8–100%; n = 20 studies). Cardiac manifestations were observed in the highest number of studies (n = 25), affecting an average of 28% of patients with EGPA. In general, ophthalmological and haematological manifestations were the least common complications of EGPA (an average of &lt;10% of patients), although rates of thrombosis as a specific symptom were reported by an average of 14% of patients. <h3>Conclusion:</h3> This review confirms and updates the clinically diverse nature of EGPA, indicative of the substantial burden of this disease and unmet need for treatments to alleviate the burden. For certain manifestations, such as asthma and cardiac sequelae, there is a much greater associated burden of disease. Early diagnosis of EGPA as the underlying cause of these manifestations, and therefore early therapeutic intervention, has the potential to reduce the cumulative burden of organ damage. <h3>REFERENCES:</h3> [1] Aromataris E, Munn Z. JBI manual for evidence synthesis [Internet]. Adelaide: Joanna Briggs Institute, 2020 [accessed 25.7.22]. [2] National Institute for Health and Care Research. Epidemiology of eosinophilic granulomatosis with polyangiitis (EGPA): a systematic review (PROSPERO registration number: CRD42023432025). 2023. Available from: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=432025 [accessed 15 November 2023]. <h3>Acknowledgements:</h3> The authors thank Victoria Richardsdóttir Fife and Bianca Kennedy Hall from AstraZeneca for project support, and Sukhvir Rai and Kerrie Ford of Health Economics and Outcomes Research Ltd (Cardiff, UK) for medical writing support, which was funded by AstraZeneca (Cambridge, UK) in accordance with Good Publication Practice (GPP 2022) guidelines (https://www.ismpp.org/gpp-2022). <h3>Disclosure of Interests:</h3> Jennifer Rowell owns share options in AstraZeneca and Roche, employee of AstraZeneca, but previously employed by Roche, Amgen and QLT Phototherapeutics, Sara Lucas an employee of Health Economics and Outcomes Research Ltd. Health Economics and Outcomes Research Ltd. received funding for data analysis and medical writing support from AstraZeneca for this work, Paul Dolin contractor for AstraZeneca.