Eosinophilic Cardiomyopathy in Eosinophilic Granulomatosis With Polyangiitis After Dupilumab Therapy

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Eosinophilic Granulomatosis With Polyangiitis (EGPA) and Eosinophilic Myocarditis (n=1). Intervention: Dupilumab. Primary outcome: Development of eosinophilic myocarditis.

Q: What does this research mean for the field?

Dupilumab therapy can be associated with the unmasking of underlying eosinophilic granulomatosis with polyangiitis manifesting as severe eosinophilic myocarditis. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Issey Agredano; Vincent Michiels; Jean‐François Argacha; Frederik H. Verbrugge; Kaoru Tanaka; Pierre Lefesvre; Bernard Cosyns

doi:10.1016/j.jaccas.2026.108880

June 1, 2026JACC Case ReportsOpen Access

Eosinophilic Cardiomyopathy in Eosinophilic Granulomatosis With Polyangiitis After Dupilumab Therapy

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Key result

Dupilumab therapy is linked to unmasking EGPA presenting as severe eosinophilic myocarditis.

n=1

Figures

Graphical abstract published by JACC Case Reports — Graphical abstract · Published by JACC Case Reports · CC BY-NC-ND

Why the study?

Dupilumab is associated with transient peripheral eosinophilia, but progression to eosinophilic granulomatosis with polyangiitis remains rare.

Key Points

To describe a case of severe eosinophilic myocarditis presenting in temporal association with dupilumab therapy for eosinophilic airway disease.
Evaluated a 64-year-old man presenting with acute chest pain, dyspnea, and systemic symptoms 3 months after initiating dupilumab.
Performed diagnostic workup comprising multimodality imaging, coronary angiography, and endomyocardial biopsy within 24 hours.
Treated the patient with high-dose corticosteroids and cyclophosphamide, followed by repeat coronary angiography and echocardiography.
Initial laboratory evaluation revealed marked peripheral eosinophilia of 25,000/μL, troponin T of 2,530 μg/L, and echocardiography showed reduced LVEF of 38%.
Coronary angiography showed an isolated stenosis of the mid left anterior descending artery, and endomyocardial biopsy confirmed eosinophilic myocarditis.
Immunosuppressive therapy led to rapid clinical improvement, recovery of LVEF to 50%, and complete resolution of coronary stenosis on repeat angiography.

Study Design

Type

Case Report (n=1)

Structured PICO

P

Population

A 64-year-old man with eosinophilic asthma and chronic rhinosinusitis who developed severe eosinophilic myocarditis in the context of EGPA 3 months after starting dupilumab therapy.

E

Exposure

Dupilumab therapy (triggering agent) subsequently managed with high-dose intravenous methylprednisolone (1 g/day for 2 days), oral prednisone (80 mg daily with tapering), and intravenous cyclophosphamide (15 mg/kg).

O

Outcome

Clinical response, recovery of left ventricular ejection fraction, and resolution of coronary stenosis following immunosuppressive therapy.

Dupilumab therapy may unmask or induce underlying eosinophilic granulomatosis with polyangiitis presenting as severe eosinophilic myocarditis, highlighting the need for vigilance and prompt immunosuppressive treatment.

Limitations

Causality remains uncertain and should be interpreted cautiously
Heterogeneity in reporting limits interpretation
Causality between dupilumab and EGPA remains uncertain and should be interpreted cautiously
Heterogeneity in reporting limits interpretation of dupilumab-associated EGPA cases

Implication

Requires vigilance for eosinophilic myocarditis on dupilumab; single case leaves open causality and needs confirmation.

Abstract

BACKGROUND: Dupilumab, an IL-4Rα antagonist used in type 2 inflammatory diseases, is associated with transient peripheral eosinophilia, yet progression to eosinophilic granulomatosis with polyangiitis (EGPA) remains rare. CASE SUMMARY: A 64-year-old man presented with acute chest pain, dyspnea, marked hypereosinophilia, elevated cardiac biomarkers, and systemic symptoms 3 months after dupilumab initiation. Echocardiography and cardiac magnetic resonance imaging demonstrated left ventricular dysfunction, and endomyocardial biopsy confirmed eosinophilic myocarditis. High-dose corticosteroids and cyclophosphamide led to rapid clinical improvement. DISCUSSION: This case shows severe eosinophilic myocarditis in the context of dupilumab therapy. Current literature suggests biologic therapy may unmask underlying EGPA rather than directly induce vasculitis, emphasizing the need for vigilance. TAKE-HOME MESSAGES: Dupilumab-associated eosinophilia warrants careful eosinophil monitoring. New systemic or cardiac symptoms should prompt evaluation for EGPA, using multimodality imaging with a low threshold for performing endomyocardial biopsy.

Discussion

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Cite This Study

Agredano et al. (2026) conducted a case report in Eosinophilic Granulomatosis With Polyangiitis (EGPA) and Eosinophilic Myocarditis (n=1). Dupilumab was evaluated on Development of eosinophilic myocarditis. Dupilumab therapy was temporally associated with the unmasking of underlying eosinophilic granulomatosis with polyangiitis presenting as severe eosinophilic myocarditis.

synapsesocial.com/papers/6a31570faf7cf7f8256b3cf5 https://doi.org/10.1016/j.jaccas.2026.108880

Topics

Myocarditis

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Eosinophilic Cardiomyopathy in Eosinophilic Granulomatosis With Polyangiitis After Dupilumab Therapy

Figures

Key Points

Study Design

Structured PICO

Limitations

Implication

Abstract

Discussion

Cite This Study

Topics

Eosinophilic Cardiomyopathy in Eosinophilic Granulomatosis With Polyangiitis After Dupilumab Therapy

Figures

Key Points

Study Design

Structured PICO

Limitations

Implication

Abstract

Discussion

Cite This Study

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Topics

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