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三例非特异性间质性肺炎致死病例的临床特征

Clinical features of three fatal cases of non-specific interstitial pneumonia.

作者信息

Fujita J, Yamadori I, Bandoh S, Mizobuchi K, Suemitsu I, Nakamura Y, Ohtsuki Y, Takahara J

机构信息

First Department of Internal Medicine, Kagawa Medical University.

出版信息

Intern Med. 2000 May;39(5):407-11. doi: 10.2169/internalmedicine.39.407.

DOI:10.2169/internalmedicine.39.407
PMID:10830184
Abstract

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%.

摘要

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。

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State of the art in the diagnosis and management of interstitial lung disease.间质性肺疾病的诊断与管理的最新进展。
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Comparison of clinicoradiologic manifestation of nonspecific interstitial pneumonia and usual interstitial pneumonia/idiopathic pulmonary fibrosis: a report from NRITLD.非特异性间质性肺炎与普通间质性肺炎/特发性肺纤维化的临床影像学表现比较:NRITLD 研究报告。
Ann Thorac Med. 2008 Oct;3(4):140-5. doi: 10.4103/1817-1737.43081.
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Pulmonary function testing in idiopathic interstitial pneumonias.
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Non-specific interstitial pneumonia; as the first clinical presentation of various collagen vascular disorders.非特异性间质性肺炎;作为各种胶原血管疾病的首发临床表现。
Rheumatol Int. 2006 Apr;26(6):551-5. doi: 10.1007/s00296-005-0028-9. Epub 2005 Nov 8.