Antineutrophil Cytoplasmic Antibody Negative Eosinophilic Granulomatosis With Polyangiitis Presents as Acute Decompensated Heart Failure

Strategic Insights -

A 60-year-old man presented with progressive dyspnea, and echocardiography demonstrated left ventricular hypokinesis with an ejection fraction of 40%. He was later diagnosed with antineutrophil cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis (EGPA), a chronic inflammatory condition characterized by eosinophilic infiltration of multiple organ systems. The patient's cardiac involvement was effectively treated with induction and maintenance immunosuppression, highlighting the importance of further evaluating cardiac involvement in EGPA. This case report underscores the need for prompt recognition and treatment of EGPA, particularly in its cardiac-predominant presentation.

Key Takeaways:

  • EGPA is a chronic inflammatory condition characterized by eosinophilic infiltration of multiple organ systems, including the heart, respiratory system, peripheral nerves, integumentary system, and gastrointestinal tract.
  • Cardiac involvement is a common presentation of EGPA, particularly in the absence of antineutrophil cytoplasmic antibodies (ANCA).
  • Induction and maintenance immunosuppression with medication such as oral prednisone, intravenous cyclophosphamide, and oral azathioprine can effectively treat cardiac involvement in EGPA.
  • The patient's cardiac function was severely compromised, with left ventricular hypokinesis and an ejection fraction of 40% at presentation.
  • Cardiac thrombi were detected, and endomyocardial biopsy revealed eosinophilic infiltration, necrosis, and fibrosis.
  • The patient's treatment course and outcome emphasize the importance of prompt recognition and treatment of EGPA, particularly in its cardiac-predominant presentation.

Statistics:

  • The patient's initial ejection fraction was 40%, which decreased to 20% over the next 3 months.
  • The patient was treated with oral prednisone for 6 months, followed by maintenance immunosuppression with oral azathioprine and prednisone.
  • Cardiac thrombi were detected in 25% of patients with EGPA.

Sources:

  • Antineutrophil Cytoplasmic Antibody Negative Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) Presenting as Acute Decompensated Heart Failure, JACC Case Reports, 2025;30(31):105332.
  • Pacific Northwest University of Health Sciences.