Diffuse cystic lung diseases including lymphangioleiomyomatosis

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.