Antineutrophil Cytoplasmic Antibody-Negative Eosinophilic Granulomatosis With Polyangiitis in a Pediatric Patient

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare pediatric vasculitis that can be difficult to diagnose, especially when antineutrophil cytoplasmic antibody (ANCA) is negative. We describe the case of a 10-year-old girl with severe asthma, recurrent leukocytoclastic vasculitis, peripheral eosinophilia, and pulmonary nodules. Infectious and immunologic workup was unremarkable, and skin biopsy confirmed eosinophilic vasculitis. She was diagnosed with ANCA-negative EGPA based on the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology criteria and started on high-dose corticosteroids with plans for mepolizumab maintenance. This case highlights the diagnostic challenges in pediatric EGPA, the masking effects of biologic therapy, and the importance of multidisciplinary evaluation for early recognition and optimal management.

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Publication Details

Journal
Cureus
Published
2026-03-13
DOI
https://doi.org/10.7759/cureus.105160
Primary Topic
Vasculitis and related conditions
Type
article
Field-Weighted Citation Impact
0.00
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article

Antineutrophil Cytoplasmic Antibody-Negative Eosinophilic Granulomatosis With Polyangiitis in a Pediatric Patient

Joseph Odeyemi, Apeksha Sathyaprasad
Cureus
Vasculitis and related conditions
article

Antineutrophil Cytoplasmic Antibody-Negative Eosinophilic Granulomatosis With Polyangiitis in a Pediatric Patient

Joseph Odeyemi, Apeksha Sathyaprasad
article en

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare pediatric vasculitis that can be difficult to diagnose, especially when antineutrophil cytoplasmic antibody (ANCA) is negative. We describe the case of a 10-year-old girl with severe asthma, recurrent leukocytoclastic vasculitis, peripheral eosinophilia, and pulmonary nodules. Infectious and immunologic workup was unremarkable, and skin biopsy confirmed eosinophilic vasculitis. She was diagnosed with ANCA-negative EGPA based on the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology criteria and started on high-dose corticosteroids with plans for mepolizumab maintenance. This case highlights the diagnostic challenges in pediatric EGPA, the masking effects of biologic therapy, and the importance of multidisciplinary evaluation for early recognition and optimal management.

Cureus
Good health and well-being
Openalex Percentile: Top 23%
Vasculitis and related conditions
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