Clinical Manifestations and Long‐Term Outcomes of Eosinophilic Granulomatosis With Polyangiitis in North America

Abstract

Objective To describe clinical manifestations and outcomes in patients with eosinophilic granulomatosis with polyangiitis (EGPA) in North America. Methods Analysis of patients aged 18 years or older who fulfilled the 1990 American College of Rheumatology Classification Criteria for EGPA enrolled in the Vasculitis Clinical Research Consortium from 2003 to 2019. Main clinical characteristics, treatments, outcomes, and accumulated damage were studied. Results The cohort included 354 patients; 59% female; age at diagnosis of 50.0 (±14) years; 39% were antineutrophil cytoplasm antibody (ANCA) positive. Time from diagnosis to last follow‐up was 7.0 (±6.2) years; 49.4% had one or more relapse. Patients positive for ANCA more commonly had neurological and kidney involvement when compared with patients negative for ANCA, who had more cardiac and lung manifestations. At last study visit, only 35 (12.6%) patients had been off all therapy for more than 2 years during their follow‐up. The overall mortality rate was 4.0% and did not differ by ANCA status or cyclophosphamide use. Scores on the Vasculitis Damage Index (VDI) for 134 patients with two or more visits and more than 1 year of follow‐up increased from 1.7 (±1.8) at enrollment (3.7 [±5.1] years after diagnosis) to 3.35 (±2.1) at last follow‐up (7.5 [±5.8] years after diagnosis), mainly represented by chronic asthma (67.5%), peripheral neuropathy (49.6%), and chronic sinusitis (31.3%). Longer duration of glucocorticoid use and relapse were associated with higher VDI scores. Conclusion This analysis describes the many clinical manifestations and varied outcomes of EGPA and highlights the ongoing need to attain more sustained, long‐term remission to limit the accrual of disease‐related damage.

Clinical Characteristics of EGPA Patients in Comparison to GPA Subgroup with Increased Blood Eosinophilia from POLVAS Registry

“…As expected, other reports on large EGPA cohorts support our observations regarding most demographic and clinical characteristics [ 4 , 7 , 19 – 24 ], except for the gender ratio. In our EGPA group, females were more prevalent, which stays in line with reports published by Tsurkisawa et al [ 19 ], Doubelt et al [ 21 ], and Sokołowska et al [ 9 ]. On the other hand, Comarmond et al [ 4 ], Samson et al [ 20 ], and Solans-Laqué et al [ 23 ] reported higher or equal morbidity in males.…”

“…As expected, other reports on large EGPA cohorts support our observations regarding most demographic and clinical characteristics [4,7,[19][20][21][22][23][24], except for the gender ratio. In our EGPA group, females were more prevalent, which stays in line with reports published by Tsurkisawa et al [19], Doubelt et al [21], and Sokołowska et al [9]. On the other hand, Comarmond et al [4], Samson et al [20], and Solans-Laqué et al [23] reported higher or equal morbidity in males.…”

Prevalence, incidence and healthcare burden of eosinophilic granulomatosis with polyangiitis in the UK

“…Prior to diagnosis patients most commonly experienced respiratory-related symptoms and over 80% had comorbid asthma. This is consistent with the commonly reported pattern of disease development leading to EGPA where the development of asthma typically pre-dates the development of hypereosinophilia and vasculitis by several years [5,7].…”

Burden of eosinophilic granulomatosis with polyangiitis in Europe

“…This is lower than reported in a US cohort study for which the mean (SD) number of manifestations through to the end of the study was 6.6 (2.3), over a longer mean duration of follow-up of 7 years. The nature of the clinical manifestations was similar between the two studies with lung, ear/nose/throat, and constitutional symptoms being the most frequently reported manifestations [4]. Similarly, vasculitic manifestations such as renal manifestations and purpura (19% and 16% in the current study, respectively) were moderately common, in keeping with other reports [4].…”