Dotake Yoichi, Tanaka Kentaro, Fujisaki Shiro, Shimobaba Kenichi, Kuroiwa Hirotoshi, Satomura Midori, Matsuyama Hiromi, Takagi Koichi, Mitsuyama Hideo, Inoue Hiromasa
Department of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima University Kagoshima Japan.
Respirol Case Rep. 2025 May 20;13(5):e70219. doi: 10.1002/rcr2.70219. eCollection 2025 May.
Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterised by asthma, eosinophilia, and systemic inflammation, often involving the lungs. We present the case of a 47-year-old woman with EGPA who developed progressive ground-glass opacities and a crazy-paving pattern on chest computed tomography (CT). Bronchoalveolar lavage revealed milky fluid, and transbronchial lung biopsy showed periodic acid-Schiff (PAS)-positive eosinophilic granular material. Elevated anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibodies confirmed a diagnosis of autoimmune pulmonary alveolar proteinosis (aPAP). Corticosteroid tapering initially led to EGPA relapse, which was successfully controlled with mepolizumab, enabling further steroid reduction. Following this, the radiological findings of aPAP showed gradual improvement. In rare cases, it is known that autoimmune diseases such as vasculitis can be complicated by aPAP. This case highlights the importance of individualised immunomodulatory treatment and close imaging follow-up in patients with overlapping autoimmune conditions.
嗜酸性肉芽肿性多血管炎(EGPA)是一种抗中性粒细胞胞浆抗体(ANCA)相关性血管炎,其特征为哮喘、嗜酸性粒细胞增多和全身炎症,常累及肺部。我们报告一例47岁患有EGPA的女性病例,其胸部计算机断层扫描(CT)显示磨玻璃影进行性加重及铺路石征。支气管肺泡灌洗显示为乳状液体,经支气管肺活检显示过碘酸雪夫(PAS)染色阳性的嗜酸性颗粒物质。抗粒细胞-巨噬细胞集落刺激因子(GM-CSF)抗体升高确诊为自身免疫性肺泡蛋白沉积症(aPAP)。最初逐渐减少皮质类固醇导致EGPA复发,使用美泊利单抗成功控制复发,从而能够进一步减少类固醇用量。此后,aPAP的影像学表现逐渐改善。已知在罕见情况下,血管炎等自身免疫性疾病可并发aPAP。该病例强调了在重叠自身免疫性疾病患者中进行个体化免疫调节治疗和密切影像学随访的重要性。