대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Idiopathic Hypereosinophilic Syndrome

Radiologic Findings

Chest radiograph shows multiple and variable sized pulmonary nodules in both lungs. CT scans show multiple nodules with or without a halo of ground-glass attenuation in the peripheral lungs. Multiple small low attenuation nodules are also seen in the liver. On laboratory examinations, the number of eosinophil increased markedly in peripheral blood (50%) and
BAL fluid (35%). Histopathologic examination in the lung and liver revealed infiltration of eosinophils in both the organs.

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

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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,