title: Diffuse Cystic Lung Diseases: Diagnostic Considerations.
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.
authors.affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Kai-Feng
authors.initials: KF
authors.lastname: Xu
authors.affiliation: Department of Pathology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Ruie
authors.initials: R
authors.lastname: Feng
authors.affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Han
authors.initials: H
authors.lastname: Cui
authors.affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Xinlun
authors.initials: X
authors.lastname: Tian
authors.affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Hanping
authors.initials: H
authors.lastname: Wang
authors.affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Zhao
authors.affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Hui
authors.initials: H
authors.lastname: Huang
authors.affiliation: Department of Radiology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Weihong
authors.initials: W
authors.lastname: Zhang
authors.affiliation: Developmental Pediatrician, PECAT, Children's Hospital Westmead, Sydney, New South Wales, Australia.
authors.forename: Bee Hong
authors.initials: BH
authors.lastname: Lo
mesh_terms: D000367:Age Factors; D000686:Amyloidosis / Q000175:diagnosis; D058249:Birt-Hogg-Dube Syndrome / Q000175:diagnosis; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis*; D017563:Lung Diseases, Interstitial / Q000175:diagnosis; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis; D012737:Sex Factors; D012859:Sjogren's Syndrome / Q000175:diagnosis