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特发性肺纤维化诊断和分类的更新。

Update on the diagnosis and classification of ILD.

机构信息

Department of Medicine, University of British Columbia, Vancouver, Canada.

出版信息

Curr Opin Pulm Med. 2013 Sep;19(5):453-9. doi: 10.1097/MCP.0b013e328363f48d.

DOI:10.1097/MCP.0b013e328363f48d
PMID:23872860
Abstract

PURPOSE OF REVIEW

The purpose of this review is to provide an update on the diagnosis and classification of interstitial lung disease (ILD), with a specific focus on newly described ILD subtypes and phenotypes. In addition, the strengths and limitations of the current approach to ILD diagnosis and management are discussed.

RECENT FINDINGS

Idiopathic pleuroparenchymal fibroelastosis and acute fibrinous and organizing pneumonia are new entities that have been described in small case series. Undifferentiated connective tissue disease-associated ILD, smoking-related interstitial fibrosis, familial ILD, unclassifiable ILD, and subclinical ILD have also been better characterized in recent publications. New data regarding these conditions are summarized in this review. The multidisciplinary approach to ILD is reviewed, and complementary classification schemes are described that may help direct the management and improve prognostication of some ILDs.

SUMMARY

ILDs are a large and heterogeneous group of diseases with several newly characterized subtypes and phenotypes. The current approach to ILD classification has limitations in some patients that can be minimized by considering complementary classification schemes.

摘要

目的综述

本文旨在更新间质性肺疾病(ILD)的诊断和分类,重点介绍新描述的ILD 亚型和表型。此外,还讨论了目前ILD 诊断和管理方法的优缺点。

最近的发现

特发性胸膜肺弹力纤维增生症和急性纤维性机化性肺炎是在小病例系列中描述的新实体。未分化结缔组织病相关间质性肺病、与吸烟相关的间质性纤维化、家族性间质性肺病、无法分类的间质性肺病和亚临床间质性肺病在最近的出版物中也得到了更好的描述。本文总结了这些疾病的新数据。本文回顾了ILD 的多学科方法,并描述了补充分类方案,这些方案可能有助于指导某些ILD 的管理和改善预后。

总结

ILD 是一组异质性较大的疾病,有几种新描述的亚型和表型。目前的ILD 分类方法在某些患者中存在局限性,通过考虑补充分类方案可以最小化这些局限性。

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