Abstract 17864: Unusual Manifestation of Eosinophilic Granulomatosis with Polyangitis: Diffuse Intracardiac Thrombi Mimicking Hypereosinophilic Syndrome and Antineutorophil Cytoplasmic Antibody-negative Angionecrotic Glomerulonephritis
Eosinophilic Granulomatosis with Polyangitis (EGPA), formerly known as Churg-Strauss syndrome, is a systemic small vessel vasculitis with eosinophilia. Cardiac involvement is especially important as it accounts for about half of the mortality and thus requires additive treatment. But its distinction from other cardiac conditions is often difficult. Positive antineutrophil cytoplasmic antibody (ANCA) test helps identify EGPA, and is most often associated with renal involvement.
Here we experienced a patient with ANCA-negative EGPA who showed diffuse intracardiac thrombi mimicking Hypereosinophilic syndrome (HES) and angionecrotic glomerulonephritis.
A 59-year-old housewife with a history of asthma was admitted due to persistent fever. Laboratory test revealed eosinophilia (3.6х109/L), elevation of NT-pro-BNP (8750 pg/mL) and troponin I (1.88 ng/mL). ANCA was negative. The patient also had overt proteinuria and hematuria. Echocardiography and Cardiac Magnetic Resonance imaging (CMR) each showed abnormal structures suggestive of thrombi along the left ventricle walls, which closely resembled cardiac manifestation of HES. On CMR, left ventricular endocardium was enhanced by T2 weighted and late gadolinium enhanced imaging. Coronary angiography was almost normal, and no etiology that can induce eosinophilia nor other organ involvement consistent with EGPA was found. Pathological examination of the kidney, on the other hand, demonstrated angionecrosis and crescent formation of the glomerulus. There were also eosinophil invasion at the sub-capsule area of the kidney. These findings finally led to the diagnosis of EGPA. Immunosuppressive agent was added to the patient besides corticosteroids.
EGPA can show diffuse intracardiac thrombi mimicking HES, and ANCA-negative EGPA can show angionecrotic glomerulonephritis. Identification of these unusual organ involvements is significant for proper and effective therapeutic intervention.
Author Disclosures: Y. Saito: None. S. Okada: None. K. Ozawa: None. H. Takaoka: None. N. Funabashi: None. Y. Kobayashi: None.
- © 2014 by American Heart Association, Inc.