대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Hypereosinophilic syndrome

Radiologic Findings

Chest radiograph shows multifocal ill-defined abnormality in the left lung.
Chest CT scans obtained 4 days later show multiple areas of ground-glass opacity in both lungs.

Follow-up; Eosinophil count in peripheral blood 21% (absolute count, 1400)
Core biopsy of the lung showed mature eosinophil infiltration without evidence of vasculitis

Brief Review

Hypereosinophilic syndrome (HES) is a rarely encountered, infiltrative disease of eosinophils affecting multiple organs, including the lung.

Criteria for the diagnosis include
Persistent eosinophilia of 1500 eosinophils/mm3 for longer than 6 months;
Absence of parasitic, allergic or other known causes of eosinophilia; and
Evidence of organ involvement.

Virtually any organ system can be involved in HES, but the most severe clinicopathologic involvement is of the heart and nervous system. There is widespread tissue infiltration with mature eosinophils that cause tissue damage, particularly endocardial damage leading to endocardial fibrosis.

Almost all patients have been men, typically young or middle-aged adult with progressive cardiopulmonary symptoms, skin rash, or myalgia together with systemic symptoms such as weight loss, weakness, fatigue, and fever.

Pulmonary involvement in HES occurs in up to 40% of patients and typically presents as cough or dyspnea.
Patients with HES and pulmonary infiltrates may be attributed to infection, infarction, congestive heart failure or HES-related pulmonary involvement. One half of these patients have significant pleural effusion, and probably due to congestive heart failure or embolic phenomenon.

The chest radiograph shows focal or diffuse, interstitial or alveolar, nonlobar infiltrates.
CT scan demonstrates nodules with or without a halo of ground-glass attenuation and focal areas of ground-glass attenuation in peripheral lung areas. Hilar lymphadenopathy has been noted, but the prevalence of intrathoracic lymphadenopathy has not been assessed.

Histopathology demonstrates striking infiltration of involved organs with eosinophils, associated with disruption of the architecture and areas of necrosis. BAL fluid eosinophils may suggest HES-related pulmonary involvement.

References

1. Kang EY, Shim JJ, Kim JS, Kim KI. Pulmonary involvement of idiopathic hypereosinophilic syndrome: CT findings in five patients. J Comput Assist Tomogr 1997; 21: 612-615
2. Winn RE, Kollef MH, Meyer JI. Pulmonary involvement in the hypereosinophilic syndrome. Chest 1994;105:656-60
3. Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine 1975;54:1-27
4. Slabbynck H, lmpens N, Naegels S, Dewaele M, Schandevyl W. Idiopathic hypereosinophilic syndrome - related pulmonary involvement diagnosed by bronchoalveolar lavage. Chest 1992;101:1178-80
5. Fauci AS, Harley JB, Robers WC, Ferrans VJ, Gralinick HR, Bjornson BH. The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic, and therapeutic considerations. Ann Intern Med 1982;97:78-92
6. Kim Y, Lee KS, Choi D-C, Primack SL, Im J-G. The spectrum of eosinophilic lung disease: radiologic findings. J Comput Assist Tomogr 1997;21:920-930

Keywords

Lung, Eosinophilic lung disease,