Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of pulmonary disorders characterised by cysts within the lung parenchyma. In some cases, a characteristic HRCT image indicates a diagnosis. There are a large number of cystic lung diseases, which have been divided into six groups according to their pathogenesis: neoplastic, congenital/genetic, lymphoproliferative, infectious, associated with ILD and other causes. In this chapter, the main characteristics of DCLDs are described, along with more detailed descriptions of ultra-rare cystic disorders such as lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and Birt–Hogg–Dubé syndrome.
Diffuse cystic lung diseases including lymphangioleiomyomatosis / D. Elia, A. Caminati, L. Tescaro, R. Cassandro, S. Harari (ERS MONOGRAPH). - In: Rare Diseases of the Respiratory System / [a cura di] Thomas O.F. Wagner, Marc Humbert, Marlies Wijsenbeek, Michael Kreuter and Helge Hebestreit. - Sheffield : European Respiratory Society Publications, 2023. - ISBN 9781849841665. - pp. 69-84 [10.1183/2312508X.10017622]
Diffuse cystic lung diseases including lymphangioleiomyomatosis
L. Tescaro;S. Harari
Ultimo
2023
Abstract
Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of pulmonary disorders characterised by cysts within the lung parenchyma. In some cases, a characteristic HRCT image indicates a diagnosis. There are a large number of cystic lung diseases, which have been divided into six groups according to their pathogenesis: neoplastic, congenital/genetic, lymphoproliferative, infectious, associated with ILD and other causes. In this chapter, the main characteristics of DCLDs are described, along with more detailed descriptions of ultra-rare cystic disorders such as lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and Birt–Hogg–Dubé syndrome.File | Dimensione | Formato | |
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