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Improved long-term prognosis of eosinophilic granulomatosis with polyangiitis: retrospective analysis of 87 patients after biologic therapy introduction in Japan
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  • Published: 21 February 2026

Improved long-term prognosis of eosinophilic granulomatosis with polyangiitis: retrospective analysis of 87 patients after biologic therapy introduction in Japan

  • Yuga Yamashita1,2,
  • Nami Masumoto1,
  • Sachiko Takaoka1,
  • Takuya Nakashima1,
  • Kaho Matsunaga1,
  • Yuka Kodama1,
  • Kosuke Terada1,
  • Hinako Masumitsu1,
  • Atsushi Miyasaka1,
  • Tatsuya Muraoka1,
  • Takeshi Kaneko2 &
  • …
  • Naomi Tsurikisawa1,2 

Scientific Reports , Article number:  (2026) Cite this article

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Subjects

  • Cardiology
  • Diseases
  • Immunology
  • Medical research

Abstract

Five-year survival in eosinophilic granulomatosis with polyangiitis (EGPA) exceeds 90%, but long-term prognosis is poor. We examined prognosis in 87 Japanese EGPA patients, seen between April 2018 and December 2024, after mepolizumab introduction. Primary outcomes were survival rate, relapse rate, and associated clinical factors. The 5-, 10-, and 20-year survival rates were 95.0%, 91.4%, and 85.2%, respectively. Nine patients died (mean age at death, 74.8 ± 6.6 years), with six deaths due to aspiration or bacterial pneumonia; none was due to active vasculitis. Older age at onset (p = 0.003) and BVAS at diagnosis (p = 0.011) were associated with poorer survival, but myocardial involvement (p = 0.43) and FFS2009 (p = 0.41) were not. Mepolizumab was administered to 69% of patients, many with cardiac involvement and frequent relapses (defined as disease occurrence at least once every 2 years after initial remission). Frequent relapses were associated with myocardial involvement (p = 0.021) and mepolizumab administration (p = 0.032). Long-term prognosis appeared favorable, with no deaths attributed to active vasculitis. Survival was comparable between the mepolizumab-treated and untreated groups, which included patients with severe and less severe disease, respectively; therefore, an independent effect of mepolizumab on prognosis could not be confirmed.

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Data availability

The datasets used and/or analyzed during this study are available from the corresponding author upon reasonable request.

Abbreviations

ACR:

American College of Rheumatology

ANCA:

antineutrophil cytoplasmic autoantibody

BVAS:

Birmingham Vasculitis Activity Score

EGPA:

eosinophilic granulomatosis with polyangiitis

EULAR:

European Alliance of Associations for Rheumatology

FFS:

five-factor score

IVIG:

intravenous immunoglobulin

MPO:

myeloperoxidase

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Acknowledgements

No grants or industrial support were involved in the funding of this study.

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This work was not funded by any grant or other external source.

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Authors and Affiliations

  1. Department of Respirology, National Hospital Organization Yokohama Medical Center, 3-60-2 Harajuku, Totsuka-ku, Yokohama, 245-8575, Japan

    Yuga Yamashita, Nami Masumoto, Sachiko Takaoka, Takuya Nakashima, Kaho Matsunaga, Yuka Kodama, Kosuke Terada, Hinako Masumitsu, Atsushi Miyasaka, Tatsuya Muraoka & Naomi Tsurikisawa

  2. Department of Pulmonology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Kanagawa, Japan

    Yuga Yamashita, Takeshi Kaneko & Naomi Tsurikisawa

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Contributions

Y.Y.: examination of patients, analysis of data, construction of figures and tables, and writing of main documentN.M.: examination of patients, analysis of data, and contributions to the discussionS.T, T.N., K.M, Y.K., K.T., H.M., A.M., and T.M.: examination of patients and contributions to the discussionT.K.: contributions to the discussionN.T.: examination of patients, analysis of data, construction of figures and tables, discussion and summary of paperAll authors have read and approved the final manuscript.

Corresponding author

Correspondence to Naomi Tsurikisawa.

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The authors declare no competing interests.

Ethics approval and consent to participate

The Ethics Committee of our hospital approved the study, and written informed consent was obtained from all patients or their legal representatives. Human Subject Protection Committee approvals at Hiratsuka City Hospital (30 − 013) and the National Hospital Organization Yokohama Medical Center (2022-06) were obtained for the retrospective review of existing medical records.

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Yamashita, Y., Masumoto, N., Takaoka, S. et al. Improved long-term prognosis of eosinophilic granulomatosis with polyangiitis: retrospective analysis of 87 patients after biologic therapy introduction in Japan. Sci Rep (2026). https://doi.org/10.1038/s41598-026-40518-2

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  • Received: 23 December 2025

  • Accepted: 13 February 2026

  • Published: 21 February 2026

  • DOI: https://doi.org/10.1038/s41598-026-40518-2

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Keywords

  • Biological product
  • Churg–Strauss Syndrome
  • Eosinophilic granulomatosis with polyangiitis
  • Immunosuppressive agent
  • Prognosis
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