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Eosinophilic Pneumonia

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Orphan Lung Diseases

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Abstract

Eosinophilic pneumonia may manifest as chronic or transient infiltrates with only mild symptoms, slowly progressive chronic idiopathic eosinophilic pneumonia, or as the frequently severe acute eosinophilic pneumonia that may present with respiratory failure. When present, blood eosinophilia greater than 1 × 109 eosinophils/L (and preferably greater than 1.5 × 109/L) is of considerable help for suggesting the diagnosis, however, it may be absent, as in the early phase of idiopathic acute eosinophilic pneumonia or when patients are already taking corticosteroids. On bronchoalveolar lavage, high eosinophilia (>25%, and preferably >40% of differential cell count) is considered diagnostic of eosinophilic pneumonia in a compatible setting, obviating the need for video-assisted thoracic surgical lung biopsy, which is now performed only on very rare occasions when inconsistency between clinical, biological, and imaging features is present. Inquiry as to drug intake, including illicit drugs, must be meticulous (www.pneumotox.com) and any suspected drug should be withdrawn. Laboratory investigations for parasitic causes must take into account the travel history or residence and the epidemiology of parasites. In patients with associated extrathoracic manifestations, the diagnoses of eosinophilic granulomatosis with polyangiitis, idiopathic eosinophilic vasculitis, and hypereosinophilic syndrome should be raised. The presence of airflow obstruction can be found in hypereosinophilic asthma, allergic bronchopulmonary aspergillosis, idiopathic chronic eosinophilic pneumonia, and eosinophilic granulomatosis with polyangiitis, or in the syndrome of hypereosinophilic obliterative bronchiolitis. Corticosteroids remain the cornerstone of symptomatic treatment for eosinophilic pneumonias, with a generally dramatic response. Relapses are common with tapering or stopping treatment, especially in idiopathic chronic eosinophilic pneumonia. Cyclophosphamide is necessary only in patients with eosinophilic granulomatosis with polyangiitis and poor-prognostic factors. Imatinib is very effective in the treatment of the clonal variant of hypereosinophilic syndrome. Evidence is accumulating for the efficacy of anti-interleukin-5 and anti-interleukin-5-receptor monoclonal antibodies, which are increasingly used in many conditions within the spectrum of eosinophilic disorders.

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  1. Department of Respiratory Medicine, National Coordinating Reference Centre for Rare Pulmonary Diseases (OrphaLung), Louis Pradel Hospital, University of Lyon, Lyon, France

    Vincent Cottin

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  1. Department of Respiratory Medicine, National Coordinating Reference Centre for Rare Pulmonary Diseases (OrphaLung), Louis Pradel Hospital, University of Lyon, Lyon, France

    Vincent Cottin

  2. Fondazione Policlinico A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Roma, Italy

    Luca Richeldi

  3. National Jewish Health, Denver, CO, USA

    Kevin Brown

  4. Division of Pulmonary, Critical Care, and Sleep Medicine Department of Internal Medicine, University of Cincinnati, Cincinnati, OH, USA

    Francis X. McCormack

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Cottin, V. (2023). Eosinophilic Pneumonia. In: Cottin, V., Richeldi, L., Brown, K., McCormack, F.X. (eds) Orphan Lung Diseases. Springer, Cham. https://doi.org/10.1007/978-3-031-12950-6_17

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