Department of Respiratory Medicine, Seirei Hamamatsu General Hospital, Japan.
Department of Pathology, Seirei Hamamatsu General Hospital, Japan.
Intern Med. 2024 Jan 1;63(1):119-124. doi: 10.2169/internalmedicine.1663-23. Epub 2023 May 24.
A 48-year-old woman was admitted to our hospital with acute respiratory failure. Chest computed tomography showed ground-glass opacity and patchy emphysematous lesions in both lungs. Corticosteroid therapy was effective; however, the disease worsened with the tapering of corticosteroids. Bronchoalveolar lavage revealed hemosiderin-laden macrophages, and video-assisted thoracic surgery showed diffuse interstitial fibrosis with diffuse alveolar hemorrhage (DAH). There was no evidence of vasculitis nor autoimmune diseases. This patient was diagnosed with idiopathic pulmonary hemosiderosis (IPH) that progressed to end-stage pulmonary fibrosis despite treatment. Autopsy demonstrated DAH with pulmonary fibrosis and emphysematous change, suggesting IPH-related pulmonary lesions.
一位 48 岁女性因急性呼吸衰竭入住我院。胸部 CT 显示双肺磨玻璃影和斑片状气肿性病变。皮质类固醇治疗有效；然而，随着皮质类固醇的逐渐减少，病情恶化。支气管肺泡灌洗显示含铁血黄素的巨噬细胞，视频辅助胸腔镜检查显示弥漫性间质纤维化伴弥漫性肺泡出血（DAH）。无脉管炎或自身免疫性疾病的证据。尽管进行了治疗，该患者仍被诊断为特发性肺含铁血黄素沉着症（IPH）进展为终末期肺纤维化。尸检显示 DAH 伴肺纤维化和肺气肿改变，提示与 IPH 相关的肺病变。
