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Challenges in pulmonary fibrosis · 3: Cystic lung disease

Department of Medicine, National Jewish Medical and Research Center and Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver, Colorado, USA

Correspondence to:
Correspondence to:
Dr Kevin K Brown
National Jewish Medical and Research Center, 1400 Jackson Street, F107, Denver, Colorado 80206, USA; brownk{at}njc.org

Cystic lung disease is a frequently encountered problem caused by a diverse group of diseases. Distinguishing true cystic lung disease from other entities, such as cavitary lung disease and emphysema, is important given the differing prognostic implications. In this paper the features of the cystic lung diseases are reviewed and contrasted with their mimics, and the clinical and radiographic features of both diffuse (pulmonary Langerhans’ cell histiocytosis and lymphangioleiomyomatosis) and focal or multifocal cystic lung disease are discussed.

Abbreviations: AML, angiomyolipoma; CCAM, congenital cystic adenomatoid malformation; DIP, desquamative interstitial pneumonia; HRCT, high resolution computed tomography; LAM, lymphangioleiomyomatosis; LIP, lymphocytic interstitial pneumonia; PLCH, pulmonary Langerhans’ cell histiocytosis; TSC, tuberous sclerosis complex