Elsevier

Clinics in Chest Medicine

Volume 37, Issue 3, September 2016, Pages 535-556
Clinics in Chest Medicine

Eosinophilic Lung Diseases

https://doi.org/10.1016/j.ccm.2016.04.015Get rights and content

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Key points

  • Eosinophilic lung diseases may present as eosinophilic pneumonia with chronic or acute onset, or as the more transient Löffler syndrome.

  • The diagnosis of eosinophilic pneumonia is based on both characteristic clinical-imaging features and the demonstration of alveolar eosinophilia of at least 25% eosinophils at bronchoalveolar lavage.

  • Peripheral blood eosinophilia is present in most eosinophilic lung disorders, but can be absent at presentation in idiopathic acute eosinophilic pneumonia and in

Definition

Eosinophilic lung diseases are a group of diffuse parenchymal lung diseases1, 2 characterized by the prominent infiltration of the lung interstitium and the alveolar spaces by polymorphonuclear eosinophils, with conservation of the lung architecture. As a corollary, a common denominator of eosinophilic lung diseases is represented by a dramatic response to systemic corticosteroid therapy and healing without any sequelae in most cases, despite frequent impressive impairment of lung function at

Recruitment of Eosinophils to the Lung

Blood and tissue eosinophilia have long been identified as major players in immunity against parasites and in the pathogenesis of allergic diseases.6 Following differentiation of precursor cells in the bone marrow under the action of several cytokines, including interleukin (IL)-5, IL-3, and granulocyte macrophage colony-stimulating factor (GM-CSF),7, 8, 9 eosinophils are recruited in the blood and tissue, including the lung in response to circulating IL-5, eotaxins, and the C-C chemokine

Idiopathic chronic eosinophilic pneumonia

First characterized by Carrington and colleagues,19 idiopathic chronic eosinophilic pneumonia (ICEP) is characterized by the onset over a few weeks of cough, dyspnea, malaise, and weight loss, with diffuse pulmonary infiltrates.

Idiopathic acute eosinophilic pneumonia

IAEP, first described by Badesch and colleagues48 and later individualized by Allen and colleagues,49 is both the most dramatic and the most frequently misdiagnosed of eosinophilic pneumonias, because it mimics infectious pneumonia or acute respiratory distress syndrome in previously healthy individuals. It differs from ICEP by its acute onset, the severity of hypoxemia, the usual lack of increased blood eosinophils at the onset of disease, and the absence of relapse.

Definition

EGPA (formerly, Churg-Strauss syndrome), described in 1951,92 mainly from autopsied cases, is a systemic disease associated with asthma, eosinophilia, and eosinophil-rich and granulomatous inflammation involving the respiratory tract and a small-vessel, necrotizing small to medium-sized vessel necrotizing vasculitis.93 EGPA is associated in approximately 40% of cases with antineutrophil cytoplasmic antibodies (ANCA) and therefore belongs to the pulmonary ANCA-associated vasculitides.

Epidemiology and Risk Factors

EGPA

Epidemiology and Pathogenesis

Allergic bronchopulmonary aspergillosis (ABPA) occurs almost exclusively in subjects with a prior history of chronic bronchial disease. It may occur in up to 1% to 2% of asthmatic adults and in up to 7% to 10% of patients with cystic fibrosis.180, 181 Isolated cases have been reported in patients with chronic obstructive pulmonary disease, and in workers in bagasse-containing sites in sugar-cane mills.182

Chronic bronchial disease is associated with viscid mucus, which with exposure to fungal

Idiopathic Hypereosinophilic Syndromes

The idiopathic hypereosinophilic syndromes, historically defined as a persistent eosinophilia greater than 1500/mm3 for longer than 6 months, without a known cause of eosinophilia, and with presumptive signs and symptoms of organ involvement,220 now encompass 2 variants221, 222: (1) the “lymphocytic variant” (approximately 30% of cases), resulting from clonal Th2 lymphocytes bearing an aberrant antigenic surface phenotype223; and (2) chronic eosinophilic leukemia or the “myeloproliferative

Practical approach to diagnosis and treatment

The diagnosis of eosinophilic lung diseases usually relies primarily on characteristic clinical-imaging features and the demonstration of alveolar eosinophilia, and lung biopsy is generally not necessary. Peripheral blood eosinophilia is an excellent diagnostic biomarker but may be absent at presentation, especially in IAEP and in patients who have already received corticosteroid treatment.

Defining the etiology of eosinophilic lung diseases has practical implications for therapeutic

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    Conflicts of Interest/Financial Support: Hospices Civils de Lyon, Université Lyon I.

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