Case Report: From misdiagnosis to successful treatment using mepolizumab in ANCA-negative severe EGPA—clinical lessons in eosinophil-targeted therapy - Scorecard - MDSpire

Case Report: From misdiagnosis to successful treatment using mepolizumab in ANCA-negative severe EGPA—clinical lessons in eosinophil-targeted therapy

  • By

  • Ju-Zhang Li

  • Si-Yao Guan

  • Qiao-Zhen Wu

  • Jiang-Nan Zheng

  • July 8, 2026

  • 0 min

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Clinical Scorecard: Clinical Insights: Transitioning from Misdiagnosis to Effective Management of ANCA-Negative Severe EGPA with Mepolizumab—Lessons in Eosinophil-Directed Therapy

At a Glance

CategoryDetail
ConditionEosinophilic Granulomatosis with Polyangiitis (EGPA)
Key MechanismsInvolves eosinophilia and systemic necrotizing vasculitis affecting multiple organs.
Target PopulationPatients with ANCA-negative severe EGPA, particularly those with multi-organ involvement.
Care SettingClinical management of severe systemic vasculitis.

Key Highlights

  • Mepolizumab effectively induced and maintained remission in a patient with ANCA-negative severe EGPA.
  • Initial treatment with omalizumab may mask disease progression in undiagnosed EGPA.
  • Mepolizumab dosing interval extended to 8 weeks showed sustained remission.
  • ANCA-negative EGPA often misdiagnosed as refractory asthma.
  • The patient exhibited multi-organ involvement including myocarditis and pericardial effusion.

Guideline-Based Recommendations

Diagnosis

  • Utilize the 2022 ACR/EULAR classification criteria for diagnosing EGPA.

Management

  • Consider mepolizumab for treatment in patients with severe EGPA.

Monitoring & Follow-up

  • Regularly assess eosinophil levels and monitor for disease progression.

Risks

  • Be cautious of initiating biologic therapy in patients with asthma without ruling out EGPA.

Patient & Prescribing Data

33-year-old female with a history of allergic rhinitis and asthma.

Mepolizumab was initiated after failure of omalizumab, leading to symptom resolution.

Clinical Best Practices

  • Evaluate for EGPA in patients with asthma and eosinophilia before starting biologic therapy.
  • Monitor for potential complications such as myocarditis in patients with severe EGPA.

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