Fujita J, Yamadori I, Bandoh S, Mizobuchi K, Suemitsu I, Nakamura Y, Ohtsuki Y, Takahara J
First Department of Internal Medicine, Kagawa Medical University.
Intern Med. 2000 May;39(5):407-11. doi: 10.2169/internalmedicine.39.407.
We describe the clinical courses of the 3 fatal patients (2 females and 1 male) with idiopathic non-specific interstitial pneumonia (NSIP) among 24 patients with NSIP. Lung biopsies were diagnosed to be NSIP group II in all patients. The clinical courses from onset to death of these 3 patients were 41 months, 46 months, and 91 months. A follow-up chest CT demonstrated no apparent honey-comb formation. We found that i) about 20% of patients with NSIP died of respiratory failure, ii) in the chest CT findings, apparent honey-comb formation was rare even just before death, iii) prediction of the prognosis based on the histological findings was difficult. This is the first report to describe the clinical features of deceased patients with idiopathic NSIP; the incidence of fatal cases was considered to range from 10 to 20%.
我们描述了24例非特异性间质性肺炎(NSIP)患者中3例(2例女性,1例男性)特发性NSIP致命患者的临床病程。所有患者经肺活检均诊断为II型NSIP。这3例患者从发病到死亡的临床病程分别为41个月、46个月和91个月。随访胸部CT显示无明显蜂窝状形成。我们发现,i)约20%的NSIP患者死于呼吸衰竭,ii)在胸部CT表现中,即使在临死前明显的蜂窝状形成也很罕见,iii)基于组织学表现预测预后很困难。这是第一份描述特发性NSIP死亡患者临床特征的报告;致命病例的发生率被认为在10%至20%之间。