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Overview of Eosinophilic Pulmonary Diseases

By Joyce Lee, MD, MAS, Assistant Professor, Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado Denver

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Eosinophilic pulmonary diseases are a heterogeneous group of disorders characterized by the accumulation of eosinophils in alveolar spaces, the interstitium, or both. Peripheral blood eosinophilia is also common. Known causes of eosinophilic pulmonary disease include

  • Infections (especially helminthic infections)

  • Drug-induced pneumonitis (eg, therapeutic drugs, such as antibiotics, phenytoin, or l-tryptophan)

  • Inhaled toxins (eg, recreational drugs, such as cocaine)

  • Systemic disorders (eg, eosinophilic granulomatosis with polyangiitis [formerly Churg-Strauss syndrome])

  • Allergic bronchopulmonary aspergillosis

Often the cause is unknown.

The two primary eosinophilic pulmonary diseases of unknown etiology are

Hypereosinophilic syndrome, a systemic disease affecting multiple organs, is discussed elsewhere.

Löffler syndrome, a syndrome of fleeting pulmonary findings and peripheral blood eosinophilia, is another eosinophilic pulmonary disease.


  • Chest-x-ray

  • Demonstrating eosinophilia in peripheral blood, bronchoalveolar lavage fluid, or lung tissue

Diagnosis is based on demonstration of opacities on chest x-ray and identification of eosinophilia (> 450/μL) in peripheral blood, bronchoalveolar lavage fluid, or lung biopsy tissue. However, pulmonary eosinophilia may occur in the absence of peripheral eosinophilia. Pulmonary opacities on chest x-ray associated with blood eosinophilia are sometimes called PIE (pulmonary infiltrates with eosinophilia) syndrome.

Eosinophils are primarily tissue-dwelling and are several hundred–fold more abundant in tissues than in blood. Consequently, blood eosinophil numbers do not necessarily indicate the extent of eosinophilic involvement in affected tissues. Eosinophils are most numerous in tissues with a mucosal epithelial interface with the environment, such as the respiratory, GI, and lower GU tracts. Eosinophils are not present in the lungs of healthy people, so their presence in tissue or bronchoalveolar lavage fluid (> 5% of differential count) identifies a pathologic process.

Eosinophils are exquisitely sensitive to corticosteroids and completely disappear from the bloodstream within a few hours after administration of corticosteroids. This rapid disappearance from the blood may obscure the diagnosis in patients who receive corticosteroids before the diagnostic assessment is instituted.