Systematic literature review and meta-analysis of the epidemiology and clinical burden of eosinophilic granulomatosis with polyangiitis.

Abstract

Objective: To conduct a systematic literature review and meta-analysis for the incidence, prevalence, and mortality of eosinophilic granulomatosis with polyangiitis (EGPA) and describe the clinical burden of EGPA.

Methods: Searches were conducted in Ovid MEDLINE and Ovid Embase from January 2019 to June 2023 to identify relevant studies. Data were extracted, and the quality was assessed. Frequentist (random effects) meta-analyses were conducted to estimate EGPA incidence, prevalence, and mortality.

Results: Data from 69 unique studies were included. The pooled estimates for EGPA incidence, prevalence, and mortality were 2.15 [95% confidence interval (CI): 0.99, 4.68] cases per million person-years, 34.44 (95% CI: 22.92, 51.76) per million people and 10.63 (95% CI: 7.20, 15.71) per 1000 person-years, respectively. Asthma was the most frequently observed clinical manifestation in patients with EGPA [average of 85.5% (range 42.3-100%)]. Other organs commonly affected included the ear, nose and throat, lungs, nervous system, and heart, and constitutional symptoms were frequently reported.

Conclusions: This study confirms that EGPA is a rare disease and is associated with a clear mortality burden. EGPA is a clinically diverse disease; patients experience substantial clinical burden, including treatment-related morbidity and mortality, and there is an unmet need to alleviate this burden.