Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center, Japan.
Department of Pathology, Saitama Cardiovascular and Respiratory Center, Japan.
Intern Med. 2021 Dec 1;60(23):3765-3772. doi: 10.2169/internalmedicine.5737-20. Epub 2021 May 22.
Fibrosing mediastinitis (FM) is a rare fibroinflammatory disease of the mediastinum with an etiology and clinical features that vary by world region. The characteristics of FM in Japan are still unknown. We herein report two Japanese patients with FM who were treated with corticosteroids and responded well. We also reviewed the Japanese literature on PubMed and summarized the characteristics of 27 Japanese FM patients, including our two patients. In Japan, the predominant cases were those without a specific cause, were diffusely distributed, and responded well to corticosteroid therapy.
纤维性纵隔炎(FM)是一种罕见的纵隔纤维炎性疾病,其病因和临床特征因世界区域而异。日本的 FM 特征尚不清楚。我们在此报告了两名接受皮质类固醇治疗且反应良好的日本 FM 患者。我们还在 PubMed 上检索了日本的文献,并总结了包括我们的两名患者在内的 27 名日本 FM 患者的特征。在日本,主要是那些没有特定病因、分布广泛且对皮质类固醇治疗反应良好的病例。