title: Multiple cystic lung disease.
abstract: Multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. High-resolution computed tomography of the chest helps to define the morphological aspects and distribution of lung cysts, as well as associated findings. The combination of appearance upon imaging and clinical features, together with extrapulmonary manifestations, when present, permits confident and accurate diagnosis of the majority of these diseases without recourse to open-lung biopsy. The main diseases in this group that are discussed in this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and folliculin gene-associated syndrome (Birt-Hogg-Dubé); other rare causes of cystic lung disease, including cystic metastasis of sarcoma, are also discussed. Disease progression is unpredictable, and understanding of the complications of cystic lung disease and their appearance during evolution of the disease are essential for management. Correlation of disease evolution and clinical context with chest imaging findings provides important clues for defining the underlying nature of cystic lung disease, and guides diagnostic evaluation and management.
authors.affiliation: Dept of Radiology, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Flavia Angélica
authors.initials: FA
authors.lastname: Ferreira Francisco
authors.affiliation: Dept of Radiology, Medical School of Rio Preto and Ultra X, São José do Rio Preto, Brazil.
authors.forename: Arthur
authors.initials: A
authors.lastname: Soares Souza
authors.affiliation: Dept of Radiology, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Gláucia
authors.initials: G
authors.lastname: Zanetti
authors.affiliation: Dept of Radiology, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil edmarchiori@gmail.com.
authors.forename: Edson
authors.initials: E
authors.lastname: Marchiori
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D001706:Biopsy; D058249:Birt-Hogg-Dube Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D008297:Male; D011020:Pneumonia, Pneumocystis / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D011237:Predictive Value of Tests; D011379:Prognosis; D012307:Risk Factors; D014057:Tomography, X-Ray Computed