Probable idiopathic hypereosinophilic syndrome: A case report of severe multi-organ eosinophilic involvement in a young male presenting with heart failure

Abstract

Hypereosinophilic syndrome (HES) is a disorder characterized by elevated levels of eosinophils, which may be associated with multi-organ involvement depending on severity. The recent diagnostic criteria for idiopathic HES require an elevated absolute eosinophil count (AEC) above 1500 cells/mcL with evidence of tissue damage. We present a case of a 37-year-old male firefighter with a purported history of eosinophilic bronchitis who was referred to the hospital with syncopal episodes and a persistent productive cough. The patient showed an AEC of 4500 cells/mcL on admission associated with high inflammatory markers. Cardiac imaging demonstrated acute myocarditis with heart failure and a reduced ejection fraction. Chest imaging was initially suggestive of community-acquired pneumonia. Workup was negative for a malignant etiology; infectious causes similarly were excluded. After a multidisciplinary evaluation, a diagnosis of idiopathic HES was made and steroids were instituted with rapid resolution of symptoms. Our case illustrates the importance of considering hypereosinophilia as a precipitating factor for acute heart failure in an otherwise healthy adult. An expeditious diagnosis can lead to early initiation of steroids to avoid progression toward multi-organ failure.

Keywords: Acute systolic heart failure; hypereosinophilia; idiopathic; idiopathic hypereosinophilic syndrome; myocarditis; pericardial effusion; reduce risk of death; urgent treatment.

Figure 1.

Chest X-ray images: increased interstitial thickening and hazy ground glass changes were noted. No pleural effusion or pneumothorax is present.

Figure 2.

Chest CT with IV contrast: axial scans shown with lung and soft tissue windows. Scans illustrate bilateral ground glass opacities (shown with arrows) with mediastinal lymphadenopathy. No pulmonary embolism is noted. CT: computerized tomography; IV: intravenous.

Figure 3.

Bronchoscopic images: (a) Mucosal edema and friability of right mainstem bronchus. (b) Image of distal bronchus intermedius. (c) Inflamed distal left lower lobe segmental bronchi with evident secretions.

Figure 4.

Cardiac MR images: thickening of myocardium observed on cardiac MRI. LV and RV are mildly dilated in size with severely reduced systolic functions: LVEF measuring at 30.2% and RVEF measuring at 31.2%. LGE of the LV was observed with mid-wall delayed enhancement in the basal inferoseptum, and subendocardial delayed enhancement in the mid-inferoseptum suggesting inflammation and/or necrosis. Moderate pericardial effusion was present as well as elevated T1, T2, and ECV, which was consistent with acute myocarditis. The mitral valve and tricuspid valve demonstrated regurgitation. ECV: extracellular volume; LGE: late gadolinium enhancement; LV: left ventricle; LVEF: left ventricular ejection fraction; RV: right ventricle; RVEF: right ventricular ejection fraction.