Clinical Features Suggestive of Underlying Eosinophilic Granulomatosis With Polyangiitis Before Dupilumab Therapy in Patients With Chronic Rhinosinusitis With Nasal Polyps
- 1Department of Otolaryngology, Head and Neck Surgery, Asahikawa Medical University, Asahikawa, JPN
- 2Medical Oncology, Dana-Farber Cancer Institute, Boston, USA
Abstract
Dupilumab is effective for eosinophilic chronic rhinosinusitis (ECRS); however, eosinophilic granulomatosis with polyangiitis (EGPA) may become clinically apparent after treatment initiation. We retrospectively reviewed three patients with chronic rhinosinusitis with nasal polyps who developed systemic manifestations leading to a diagnosis of EGPA shortly after dupilumab initiation. All three patients had bronchial asthma and peripheral eosinophilia before treatment. EGPA developed within two weeks to two months after dupilumab initiation, with manifestations including fever, arthralgia, peripheral neuropathy, weight loss, and hypertrophic pachymeningitis. Two patients achieved remission with systemic corticosteroids and mepolizumab, whereas one patient with pre-existing myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity had a fatal outcome despite intensive immunosuppressive therapy. Their pretreatment courses suggested that systemic eosinophilic disease may already have been evolving before biologic therapy, particularly in the patient with pre-existing MPO-ANCA positivity. Notably, the timing at which EGPA criteria were fulfilled differed between Japan's Ministry of Health, Labour and Welfare (MHLW) diagnostic criteria and the European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) classification criteria in these patients, highlighting that reliance on a single set of criteria may delay recognition of evolving EGPA. These cases emphasize the importance of evaluating unexplained marked eosinophilia, ANCA positivity, and systemic symptoms before initiating dupilumab and of distinguishing transient treatment-associated eosinophilia from emerging systemic vasculitis during follow-up.
Related
- Eosinophilic Granulomatosis with Polyangiitis Complicated by Eosinophilic Chronic Rhinosinusitis Successfully Treated with Combination of Mepolizumab and Dupilumab
- When Eosinophilic Granulomatosis With Polyangiitis (EGPA) Looks Like Granulomatosis With Polyangiitis (GPA): Navigating a Complex Case of Anti-neutrophil Cytoplasmic Antibodies (ANCA) Vasculitis
- Eosinophilic Granulomatosis with Polyangiitis Presenting with an Acute Hypersensitivity Pneumonitis-like CT Pattern: A Case Report
- Severe Cardiomyopathy Following Takotsubo Syndrome in a Patient Suffering from Eosinophilic Granulomatosis With Polyangiitis: A Case Report
- Anti-programmed Death-ligand 1 Antibody-associated Eosinophilic Granulomatosis with Polyangiitis in Non-small Cell Lung Cancer
BibTeX
@article{hashimoto2026clinical,
title = {{Clinical Features Suggestive of Underlying Eosinophilic Granulomatosis With Polyangiitis Before Dupilumab Therapy in Patients With Chronic Rhinosinusitis With Nasal Polyps}},
author = {Ruka Hashimoto and Takumi Kumai and Michihisa Kono and Kenzo Ohara and Miki Takahara},
journal = {Cureus},
year = {2026},
month = oct,
doi = {10.7759/cureus.117710},
url = {https://doi.org/10.7759/cureus.117710},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Clinical Features Suggestive of Underlying Eosinophilic Granulomatosis With Polyangiitis Before Dupilumab Therapy in Patients With Chronic Rhinosinusitis With Nasal Polyps
AU - Ruka Hashimoto
AU - Takumi Kumai
AU - Michihisa Kono
AU - Kenzo Ohara
AU - Miki Takahara
PY - 2026
DA - 2026/10/09
JO - Cureus
DO - 10.7759/cureus.117710
UR - https://doi.org/10.7759/cureus.117710
AB - Dupilumab is effective for eosinophilic chronic rhinosinusitis (ECRS); however, eosinophilic granulomatosis with polyangiitis (EGPA) may become clinically apparent after treatment initiation. We retrospectively reviewed three patients with chronic rhinosinusitis with nasal polyps who developed systemic manifestations leading to a diagnosis of EGPA shortly after dupilumab initiation. All three patients had bronchial asthma and peripheral eosinophilia before treatment. EGPA developed within two weeks to two months after dupilumab initiation, with manifestations including fever, arthralgia, peripheral neuropathy, weight loss, and hypertrophic pachymeningitis. Two patients achieved remission with systemic corticosteroids and mepolizumab, whereas one patient with pre-existing myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity had a fatal outcome despite intensive immunosuppressive therapy. Their pretreatment courses suggested that systemic eosinophilic disease may already have been evolving before biologic therapy, particularly in the patient with pre-existing MPO-ANCA positivity. Notably, the timing at which EGPA criteria were fulfilled differed between Japan's Ministry of Health, Labour and Welfare (MHLW) diagnostic criteria and the European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) classification criteria in these patients, highlighting that reliance on a single set of criteria may delay recognition of evolving EGPA. These cases emphasize the importance of evaluating unexplained marked eosinophilia, ANCA positivity, and systemic symptoms before initiating dupilumab and of distinguishing transient treatment-associated eosinophilia from emerging systemic vasculitis during follow-up.
SN - 2168-8184
ER -