Lipsi Roberto, Coppola Angelo, Carriera Lorenzo, Moretti Riccardo, Dodaj Meridiana
Department of Pulmonology and Sub-Intensive Respiratory Unit Ospedale Santa Maria della Misericordia Perugia Italy.
UOC Pneumologia, Ospedale San Filippo Neri-ASL Roma 1 Rome Italy.
Respirol Case Rep. 2025 Jul 9;13(7):e70270. doi: 10.1002/rcr2.70270. eCollection 2025 Jul.
Hypereosinophilic Syndrome (HES) represents a heterogeneous and complex group of disorders characterised by persistent blood and tissue eosinophilia, leading to progressive tissue damage and organ dysfunction. This spectrum includes both hematologic variants and non-hematologic forms, either secondary to identifiable causes or idiopathic in nature. In this article, we describe a rare clinical presentation of HES manifesting primarily with pulmonary involvement, diagnosed as Non-Specific Interstitial Pneumonia (NSIP). Remarkably, the interstitial lung disease showed near-complete reversibility following targeted inhibition of the IL-5 pathway with mepolizumab, highlighting the potential role of Th2-driven eosinophilic inflammation in the pathogenesis of certain forms of interstitial lung disease.
高嗜酸性粒细胞综合征（HES）是一组异质性且复杂的疾病，其特征为血液和组织中嗜酸性粒细胞持续增多，导致进行性组织损伤和器官功能障碍。这一谱系包括血液学变异型和非血液学形式，可继发于可识别的病因或本质上为特发性。在本文中，我们描述了一例主要表现为肺部受累的HES罕见临床病例，诊断为非特异性间质性肺炎（NSIP）。值得注意的是，在用美泊利单抗靶向抑制IL-5通路后，间质性肺病显示出近乎完全的可逆性，突出了Th2驱动的嗜酸性粒细胞炎症在某些形式的间质性肺病发病机制中的潜在作用。
