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Semin Respir Crit Care Med 2016; 37(03): 457-467
DOI: 10.1055/s-0036-1580690
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Diffuse Cystic Lung Diseases: Diagnostic Considerations

Kai-Feng Xu
1   Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
,
Ruie Feng
2   Department of Pathology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
,
Han Cui
1   Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
,
Xinlun Tian
1   Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
,
Hanping Wang
1   Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
,
Jing Zhao
1   Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
,
Hui Huang
1   Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
,
Weihong Zhang
3   Department of Radiology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
,
Bee Hong Lo
4   Developmental Pediatrician, PECAT, Children's Hospital Westmead, Sydney, New South Wales, Australia
› Author Affiliations
Further Information

Publication History

Publication Date:
27 May 2016 (online)

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Abstract

Diffuse cystic lung disease (DCLD) is a group of heterogeneous diseases that present as diffuse cystic changes in the lung on computed tomography of the chest. Most DCLD diseases are rare, although they might resemble common diseases such as emphysema and bronchiectasis. Main causes of DCLD include lymphangioleiomyomatosis, Birt–Hogg–Dubé syndrome, pulmonary Langerhans cell histiocytosis, lymphoid interstitial pneumonia, amyloidosis, light-chain deposition disease, Sjögren syndrome, and primary or metastatic neoplasm. We discuss clinical factors that are helpful in the differential diagnosis of DCLDsuch as sex and age, symptoms and signs, extrapulmonary presentations, cigarette smoking, and family history. Investigations for DCLD include high-resolution computed tomography, biochemical and histopathological studies, genetic tests, pulmonary function tests, and bronchoscopic and video-assisted thoracoscopic biopsies. A proposed diagnostic algorithm would enhance ease of diagnosing most cases of DCLD.

Keywords

diffuse cystic lung disease - high-resolution computed tomography - genetic test - lymphangioleiomyomatosis - pulmonary Langerhans cell histiocytosis - Sjögren syndrome - Birt–Hogg–Dubé syndrome - lymphoid interstitial pneumonia

Note

Kai-Feng Xu and Ruie Feng contributed to this work equally.


 

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