Diffuse Cystic Lung Disease Part II
Diffuse Cystic Lung Disease Part II
复制标题
DOI：
10.1164/rccm.201411-2096ci
复制
发表时间：
2015-07-01
影响因子：
24.7
通讯作者：
McCormack, Francis X.
中科院分区：
文献类型：
--
作者：
Gupta, Nishant;Vassallo, Robert;McCormack, Francis X.
弥漫性囊性肺疾病具有广泛的鉴别诊断。从气道阻塞到肺重塑的各种病理生理过程都可能导致肺部多灶性囊肿的发展。尽管淋巴管平滑肌瘤病和肺朗格汉斯细胞组织细胞增多症在临床上可能更常见，但诸如Birt-Hogg-Dube综合征、淋巴细胞性间质性肺炎、滤泡性细支气管炎和轻链沉积病等疾病也越来越多地被认识到。获得准确的诊断可能具有挑战性，并且管理方法高度依赖于疾病。独特的影像学特征、基因检测、血清研究和临床特征提供了宝贵的线索，帮助临床医生区分各种病因，但通常需要活检才能明确诊断。在本综述的第二部分中，我们概述了由淋巴增殖性疾病、基因突变或肺部发育异常引起的弥漫性囊性肺疾病，并提供了一种帮助诊断和治疗的方法。
The diffuse cystic lung diseases have a broad differential diagnosis. A wide variety of pathophysiological processes spanning the spectrum from airway obstruction to lung remodeling can lead to multifocal cyst development in the lung. Although lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are perhaps more frequently seen in the clinic, disorders such as Birt-Hogg-Dube syndrome, lymphocytic interstitial pneumonia, follicular bronchiolitis, and light-chain deposition disease are increasingly being recognized. Obtaining an accurate diagnosis can be challenging, and management approaches are highly disease dependent. Unique imaging features, genetic tests, serum studies, and clinical features provide invaluable clues that help clinicians distinguish among the various etiologies, but biopsy is often required for definitive diagnosis. In part II of this review, we present an overview of the diffuse cystic lung diseases caused by lymphoproliferative disorders, genetic mutations, or aberrant lung development and provide an approach to aid in their diagnosis and management.