Murohashi Kota, Hara Yu, Aoki Ayako, Matsumura Masahito, Kataoka Toshiro, Okudela Koji, Kaneko Takeshi
Department of Pulmonology, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
Department of Pathology, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
Respir Med Case Rep. 2020 Feb 7;29:101022. doi: 10.1016/j.rmcr.2020.101022. eCollection 2020.
An 83-year-old man with a history of interstitial lung disease (ILD) presented with a 1-week history of progressive dyspnea. Computed tomography of the chest revealed right lung-predominant, diffuse, ground glass opacities superimposed upon reticular opacities. Despite methylprednisolone pulse therapy under a diagnosis of acute exacerbation (AE) of ILD, lung involvement and renal dysfunction worsened and disseminated intravascular coagulation developed. The patient died on day 5 of hospitalization. Pathological examination at autopsy revealed diffuse alveolar hemorrhage (DAH) superimposed upon organizing diffuse alveolar damage and usual interstitial pneumonia. We reached a final diagnosis of DAH-predominant AE of idiopathic pulmonary fibrosis (IPF). Abundant expression of the oxidative stress marker hemeoxygenase-1 (HO-1) was observed in alveolar macrophages. These suggest that HO-1 expression in the lungs may offer a useful biomarker for this atypical histological subtype of AE of IPF.
一名83岁有间质性肺疾病（ILD）病史的男性，出现进行性呼吸困难1周。胸部计算机断层扫描显示以右肺为主的弥漫性磨玻璃影叠加在网状影上。尽管在诊断为ILD急性加重（AE）的情况下给予了甲泼尼龙冲击治疗，但肺部受累及肾功能障碍仍恶化，并发生了弥散性血管内凝血。患者于住院第5天死亡。尸检病理检查显示弥漫性肺泡出血（DAH）叠加在机化性弥漫性肺泡损伤和普通间质性肺炎之上。我们最终诊断为特发性肺纤维化（IPF）以DAH为主的AE。在肺泡巨噬细胞中观察到氧化应激标志物血红素加氧酶-1（HO-1）的大量表达。这些提示肺部HO-1表达可能为IPF这种非典型组织学亚型的AE提供有用的生物标志物。
