par Taton, Olivier 
;Bondue, Benjamin ;Roufosse, Florence
Référence ERS Monograph, 2022, page (153-176)
Publication Publié, 2022-02-01
;Bondue, Benjamin ;Roufosse, Florence Référence ERS Monograph, 2022, page (153-176)
Publication Publié, 2022-02-01
Article révisé par les pairs
| Résumé : | HES are a heterogeneous group of rare disorders characterised by hypereosinophilia and eosinophil-mediated organ damage and/or dysfunction. Although specific disease variants primarily involving myeloid or lymphoid cells exist, underlying pathogenic mechanisms remain unknown in most patients. Respiratory symptoms are frequent in HES, and the lungs are among the most commonly affected organs, especially in idiopathic and FIP1L1-PDGFRA+ myeloid variants. Pulmonary complications may occur in isolation, especially at presentation, or as part of a systemic disease affecting other organs. They vary in nature, and commonly consist of asthma and/or interstitial disease with presence of eosinophils both in the airway lining and parenchyma. Demonstration of eosinophils in BAL fluid and/or lung tissue is an important step in establishing the direct role of eosinophils in lung disease. Treatment should aim to reduce blood and tissue eosinophilia to reverse and prevent further damage. Although corticosteroids are often rapidly effective, targeted treatment options should be preferred, namely the tyrosine kinase inhibitor imatinib mesylate as first-line therapy for clonal eosinophilic variants and monoclonal antibodies targeting the IL-5 pathway as maintenance therapy for patients with non-clonal eosinophils. |
