Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease associated with vascular inflammation and multisystem organ damage. The literature reporting EGPA incidence or prevalence is limited. We performed a systematic literature review and meta-analysis to describe the incidence, prevalence, and disease burden associated with EGPA. Real-world, observational, English-language studies in MEDLINE, MEDLINE In-Process, and Embase up to 6 June, 2019, were included. A single investigator screened all identified titles/abstracts and extracted data; an additional, independent investigator repeated the screening and validated the extracted data. A random-effects meta-analysis was conducted to generate pooled estimates for EGPA incidence and prevalence. Data from 100 eligible publications were extracted (32 with incidence/prevalence data, 65 with morbidity/healthcare resource data; 3 with both types of data). Significant evidence of between-study heterogeneity for reported incidence (p = 0.0013–0.0016) and prevalence (p = 0.0001–0.0006) estimates was observed. Global and European pooled estimates (95% confidence interval) of EGPA incidence were 1.22 (0.93, 1.60) and 1.07 (0.94, 1.35) cases per million person-years, respectively; global and European pooled estimates (95% confidence interval) for EGPA prevalence were 15.27 (11.89, 19.61) and 12.13 (6.98, 21.06) cases per million individuals, respectively. The proportions of patients experiencing relapses, or who had nasal polyps or severe asthma, varied considerably across studies. EGPA healthcare resource use was high, with inpatient admissions and emergency department visits reported for 17–42% and 25–42% of patients, respectively. Our results indicate that although global and European EGPA incidence and prevalence is low, the associated disease burden is substantial.

Key points • We performed a systematic literature review and meta-analysis of real-world, observational studies describing the incidence, prevalence, and disease burden associated with eosinophilic granulomatosis with polyangiitis (EGPA). • Based on meta-analysis data from 35 eligible studies reporting incidence and prevalence, the incidence and prevalence of EGPA were low (globally 1.22 cases per million person-years and 15.27 cases per million individuals, respectively). • Among the 49 studies with morbidity and/or healthcare resource data, most reported a large proportion of patients with EGPA relapses and comorbidities of nasal polyps and severe asthma. • Healthcare resource use was also high among patients with EGPA in these studies, with inpatient admissions and emergency department visits reported for 17–42% and 25–42% of patients, respectively. Taken together, these data indicate the substantial disease burden associated with EGPA.

中文翻译：

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嗜酸性肉芽肿性多血管炎相关疾病负担：系统文献回顾和荟萃分析

摘要

嗜酸性肉芽肿伴多血管炎（EGPA）是一种与血管炎症和多系统器官损伤相关的罕见疾病。报道 EGPA 发病率或流行率的文献是有限的。我们进行了系统的文献回顾和荟萃分析，以描述与 EGPA 相关的发病率、患病率和疾病负担。包括截至 2019 年 6 月 6 日在 MEDLINE、MEDLINE In-Process 和 Embase 中的真实世界、观察性英语语言研究。一名调查员筛选所有已确定的标题/摘要并提取数据；另一位独立调查员重复了筛选并验证了提取的数据。进行随机效应荟萃分析以生成 EGPA 发病率和患病率的汇总估计值。提取了 100 份符合条件的出版物的数据（32 份有发病率/患病率数据，65 具有发病率/医疗保健资源数据；3 两种类型的数据）。观察到报告的发病率（p = 0.0013-0.0016）和患病率（p = 0.0001-0.0006）估计值的研究间异质性的重要证据。EGPA 发病率的全球和欧洲汇总估计（95% 置信区间）分别为每百万人年 1.22（0.93, 1.60）和 1.07（0.94, 1.35）例；EGPA 患病率的全球和欧洲汇总估计值（95% 置信区间）分别为每百万人 15.27（11.89、19.61）和 12.13（6.98、21.06）例。在不同研究中，复发、鼻息肉或严重哮喘患者的比例差异很大。EGPA 医疗资源使用率很高，据报道，住院患者和急诊科就诊率分别为 17-42% 和 25-42%。

要点 •我们对描述与嗜酸性肉芽肿伴多血管炎(EGPA) 相关的发病率、患病率和疾病负担的真实世界观察性研究进行了系统的文献回顾和荟萃分析。 •根据来自 35 项报告发病率和患病率的合格研究的荟萃分析数据，EGPA 的发病率和患病率很低（全球分别为每百万人年 1.22 例和每百万人 15.27 例）。 •在具有发病率和/或医疗资源数据的 49 项研究中，大多数报告了大部分 EGPA 复发和鼻息肉和严重哮喘合并症的患者。 •在这些研究中，EGPA 患者的医疗资源使用率也很高，据报道，分别有 17-42% 和 25-42% 的患者住院和急诊就诊。总之，这些数据表明与 EGPA 相关的大量疾病负担。