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Multicenter Study
. 2025 Jul;42(7):3369-3385.
doi: 10.1007/s12325-025-03197-5. Epub 2025 May 20.

Reality of Patient-Reported Symptoms in 200 Patients with Eosinophilic Granulomatosis with Polyangiitis: A Cross-Sectional Survey (The KUNPU Study)

Koichi Amano  1 , Keita Ono  2 , Kazuya Sumi  2 , Hitomi Uchimura  2 , Hayato Oka  2 , Naoyuki Makita  2 , Masami Taniguchi  3
Affiliations
Multicenter Study

Reality of Patient-Reported Symptoms in 200 Patients with Eosinophilic Granulomatosis with Polyangiitis: A Cross-Sectional Survey (The KUNPU Study)

Koichi Amano et al. Adv Ther. 2025 Jul.

Abstract

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of antineutrophil cytoplasmic antibody-associated vasculitis characterized by inflammation of small- and medium-sized vessels, causing symptoms in multiple organs. The symptoms and daily life problems reported by patients with EGPA themselves are largely unknown. We conducted a cross-sectional survey to investigate the reality of EGPA-related symptoms in patients with EGPA.

Methods: Specialists and specialized facilities with experience in treating patients with EGPA cooperated in the survey; specialists from 28 facilities across Japan participated. Patients diagnosed with EGPA by their physician and treated for ≥ 1 year who agreed to answer the online questions were enrolled and completed the survey between March and June 2024. Patients answered questions about their general symptoms, asthma symptoms, and quality of life.

Results: We analyzed valid responses from 200 patients (61.0% female/38.5% male/0.5% prefer not to answer) with EGPA. The mean age was 57.9 years and 34.5% were ≥ 65 years old. Patients were treated at rheumatology departments (48.0%), respiratory/allergy departments (48.0%), and other departments (4.0%). Basic treatments included oral glucocorticoids (63.0%) and anti-interleukin-5/receptor α biologics (61.0%). Symptoms in > 50.0% of patients (past month) were pain/numbness (73.5%), fatigue/malaise (68.0%), asthmatic symptoms (56.0%), nasal/paranasal symptoms (55.0%), and joint/muscle pain (54.5%). Pain/numbness was considered the most painful symptom (29.5%). Nearly all patients experienced symptoms affecting two or more organs/systems. Patients reported that EGPA symptoms had detrimental impacts on physical and mental health; 67.0% of patients thought they were not understood by others because their disease is invisible, and symptoms frequently affected their daily life (61.5%), work (53.0%), sleep (49.5%), and social life (36.5%).

Conclusion: This is the largest survey of patients with EGPA. We have revealed the reality of patients' perceptions of EGPA-related symptoms. These results are expected to contribute to improvement in patient-centered EGPA management.

Trial registration: jRCT1050230186.

Keywords: EGPA; Eosinophilic granulomatosis with polyangiitis; Patient-reported outcomes; Quality of life.

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Conflict of interest statement

Declarations. Conflict of Interest: Koichi Amano has received honoraria from AbbVie, AstraZeneca, Chugai Pharmaceutical, Eisai, Eli Lilly, GSK, and Pfizer. Keita Ono, Kazuya Sumi, Hitomi Uchimura, Hayato Oka, and Naoyuki Makita are employees of AstraZeneca K.K. Masami Taniguchi has received grants from GSK and honoraria from AstraZeneca, GSK, and Sanofi. Ethical Approval: This study was conducted in adherence to the Declaration of Helsinki, Ethical Guidelines for Medical and Biological Research Involving Human Subjects, and Japanese laws and regulations. The KUNPU study was approved by the Takahashi Pediatric Clinic Institutional Review Board (approval number LNW00196, January 2024) and registered at jRCT (jRCT1050230186). All participants gave electronic informed consent prior to registration in the KUNPU study, and medical data were collected and stored in compliance with the relevant laws/regulations concerning data protection and the Personal Information Protection Act.

Figures

Fig. 1
Fig. 1
Patient disposition. aMultiple reasons were possible. FAS full analysis set
Fig. 2
Fig. 2
Prevalence and severity of EGPA-related symptoms (a), number of symptoms (b), and most painful symptoms (c) experienced by the patients in the past month. EGPA eosinophilic granulomatosis with polyangiitis
Fig. 3
Fig. 3
Impact of EGPA symptoms on aspects of physical and mental health. EGPA eosinophilic granulomatosis with polyangiitis
See this image and copyright information in PMC

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