Antineutrophil Cytoplasmic Antibody Negative Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) Presenting as Acute Decompensated Heart Failure

Abstract

Background

Eosinophilic granulomatosis with polyangiitis (EGPA) is a chronic inflammatory condition with eosinophilic infiltration of the respiratory system, heart, peripheral nerves, integumentary system, and gastrointestinal tract.

Case Summary

A man in his mid-60s presented with a 3-month history of progressive dyspnea. He was found to have a large pleural effusion. Over the subsequent 3 months, dyspnea returned, and echocardiography demonstrated left ventricular hypokinesis with an ejection fraction of 40%. He was later admitted for altered mental status and found to have an elevated total eosinophil count with negative antineutrophil cytotoxic antibodies. Repeat echocardiography revealed ventricular thrombi and ejection fraction of 20%. Endomyocardial biopsy revealed eosinophilic infiltration, necrosis, and fibrosis. He was treated with oral prednisone and intravenous cyclophosphamide for 6 months and maintained on oral azathioprine and prednisone.

Discussion

Antineutrophil cytotoxic antibody–negative EGPA is associated with a cardiac-predominant presentation, which is effectively treated with induction and maintenance immunosuppression.

Take-Home Message

Cardiac involvement should be further evaluated on recognition of EGPA.