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[肺气肿,你是说肺气肿吗?]

[Emphysema, did you say emphysema?].

作者信息

Benzaquen J, Pradelli J, Padovani B, Marquette C H, Leroy S

机构信息

Service de pneumologie, hôpital Pasteur, universite Côte-d'Azur, FHU OncoAge, centre hospitalier universitaire de Nice, 06000 Nice, France.

Service de pneumologie, hôpital Pasteur, universite Côte-d'Azur, FHU OncoAge, centre hospitalier universitaire de Nice, 06000 Nice, France.

出版信息

Rev Mal Respir. 2018 Jan;35(1):83-87. doi: 10.1016/j.rmr.2017.10.003. Epub 2018 Feb 3.

Abstract

INTRODUCTION

Chronic obstructive pulmonary disease (COPD) is a common condition that may initially look simple but may conceal other diseases capable of accelerating its natural history or even simulating it. We describe four cases presenting as COPD with emphysema that were reclassified on the basis of certain clinical characteristics and the radiological pattern.

CASE REPORTS

A 52 year old never smoking woman presenting with emphysema was eventually diagnosed as having lymphangioleiomyomatosis on the basis of an abdominal CT scan showing kidney angiomyolipomas. A 44 years old smoker presenting with rapidly evolving emphysema was eventually diagnosed as having Langerhans cell histiocytosis on the basis of a previous chest CT (four years earlier) showing cavitating nodules. An airport refueler, 73 years old, with severe emphysema despite never having smoked, was eventually diagnosed as suffering from alpha-1 antitrypsin deficiency. The last patient was a 54 year old man, a never smoker, who presented with severe airflow limitation and multilobar hyperlucency, with bronchiectasis in the same areas. He was eventually diagnosed as having a severe form of the Swyer-James-MacLeod syndrome.

CONCLUSION

These four case reports underline the importance of questioning the diagnosis of COPD when certain particular phenotypic characteristics are identified.

摘要

引言

慢性阻塞性肺疾病(COPD)是一种常见病症,其初期表现可能看似简单,但可能隐藏着其他能够加速其自然病程甚至模拟其症状的疾病。我们描述了四例表现为伴有肺气肿的COPD病例,这些病例根据某些临床特征和放射学表现进行了重新分类。

病例报告

一名52岁从不吸烟的女性,以肺气肿症状就诊,最终根据腹部CT扫描显示肾血管平滑肌脂肪瘤,被诊断为淋巴管平滑肌瘤病。一名44岁的吸烟者,以快速进展的肺气肿症状就诊,最终根据之前的胸部CT(四年前)显示有空洞结节,被诊断为朗格汉斯细胞组织细胞增多症。一名73岁的机场加油工,尽管从未吸烟,但患有严重肺气肿,最终被诊断为α-1抗胰蛋白酶缺乏症。最后一名患者是一名54岁从不吸烟的男性,表现为严重气流受限和多叶透亮度增加,相同区域存在支气管扩张。他最终被诊断为严重形式的斯怀尔-詹姆斯-麦克劳德综合征。

结论

这四例病例报告强调了在识别出某些特定表型特征时,对COPD诊断提出质疑的重要性。

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