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Author Spotlight: Investigating the Pathophysiology of Eosinophilic Esophagitis
Published on: May 10, 2024
Isolated CNS involvement in eosinophilic granulomatosis with polyangiitis treated with mepolizumab: A case report
Hyoshin Son1, Woo-Jin Lee1, Jangsup Moon2
1Department of Neurology, Seoul National University Hospital, Seoul, South Korea.
Abstract:
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis disease involving small-sized vessels. The literature has reported involvement of the central nervous system (CNS) in 5% cases, and isolated CNS involvement is extremely rare. Due to its rarity and scarcity of clinical data, standardized treatment of isolated CNS involvement in EGPA is unclear. Mepolizumab, an anti-interleukin-5 monoclonal antibody, was previously introduced to treat EGPA with longer remission period, more patients showing remission, and reduction in prednisolone dose of those who already taking prednisolone compared to placebo. We describe a case of isolated CNS involvement in EGPA, confirmed by brain biopsy and treated with mepolizumab, which was refractory to conventional immunotherapy.
Insights
Eosinophilic granulomatosis with polyangiitis (EGPA) rarely affects the central nervous system (CNS). This study details a successful mepolizumab treatment for isolated CNS EGPA refractory to standard therapies.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis affecting small vessels.
- Central nervous system (CNS) involvement in EGPA is uncommon, with isolated CNS EGPA being extremely rare.
- Standardized treatment for isolated CNS EGPA remains unclear due to limited clinical data.
Observation:
- This report describes a unique case of EGPA presenting solely with CNS involvement.
- Diagnosis was confirmed through brain biopsy.
- The patient's condition was refractory to conventional immunotherapy.
Findings:
- Mepolizumab, an anti-interleukin-5 monoclonal antibody, was administered to the patient.
- Mepolizumab demonstrated efficacy in treating this refractory case of isolated CNS EGPA.
- This suggests a potential therapeutic role for mepolizumab in such rare presentations.
Implications:
- This case highlights mepolizumab as a viable treatment option for refractory isolated CNS EGPA.
- Further research into targeted therapies like mepolizumab is warranted for rare vasculitic presentations.
- Understanding treatment responses in rare EGPA subtypes can improve patient outcomes.
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