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间质肺异常:在向进展性肺纤维化迈进的道路上设置障碍。

Interstitial lung abnormalities: erecting fences in the path towards advanced pulmonary fibrosis.

出版信息

Thorax. 2019 May;74(5):506-511. doi: 10.1136/thoraxjnl-2018-212446. Epub 2019 Feb 5.

Abstract

Interstitial lung abnormalities, when present in members of undiagnosed family members recruited on the basis of familial interstitial pneumonia, or in undiagnosed research participants, have been associated with a syndrome that includes distinct sets of imaging abnormalities, restrictive physiological and exercise impairments, and an increased prevalence of histopathological findings, and genetic predictors, that have been noted in patients with idiopathic pulmonary fibrosis. Recent longitudinal studies have demonstrated that qualitative and quantitative assessments of interstitial abnormalities are associated with accelerated lung function decline, an increased rate of clinical diagnoses of interstitial lung disease and an increased rate of mortality. In this perspective, in addition to reviewing the prior information, four major efforts that could help the field of early pulmonary fibrosis detection move forward are discussed. These efforts include: (1) developing standards for characterising and reporting imaging findings from patients with existing CTs; (2) developing consensus statements on when undiagnosed and asymptomatic imaging abnormalities should be considered a disease; (3) identifying populations for which screening efforts might be beneficial; and (4) considering approaches to developing effective secondary prevention trials.

摘要

间质性肺异常,当存在于家族性间质性肺炎基础上招募的未确诊家族成员或未确诊的研究参与者中时,与一种综合征相关,该综合征包括不同的影像学异常、限制性生理和运动障碍,以及更高的组织病理学发现和遗传预测因子的患病率,这些在特发性肺纤维化患者中已经注意到。最近的纵向研究表明,间质异常的定性和定量评估与肺功能下降加速、间质性肺疾病临床诊断率增加和死亡率增加有关。在这种观点下,除了回顾先前的信息外,还讨论了有助于早期肺纤维化检测领域前进的四项主要努力。这些努力包括:(1)制定用于描述和报告现有 CT 患者的影像学发现的标准;(2)制定关于何时将未确诊和无症状的影像学异常视为疾病的共识声明;(3)确定可能受益于筛查的人群;(4)考虑开发有效的二级预防试验的方法。

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