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Canadian Respiratory Journal
Volume 17, Issue 3, Pages e55-e62

Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

Stephen C Juvet,1 David Hwang,2 and Gregory P Downey3

1Division of Respirology and Clinician-Scientist Training Program, Department of Medicine, University Health Network, Toronto, Ontario, Canada
2Department of Laboratory Medicine and Pathobiology, and Toronto General Hospital/University Health Network, Toronto, Ontario, Canada
3Departments of Medicine, Pediatrics, and Immunology, National Jewish Health and University of Colorado, Aurora, Colorado, USA

Copyright © 2010 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Pulmonary Langerhans’ cell histiocytosis (PLCH) is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. The current review discusses the presenting features and relevant diagnostic testing and treatment options for PLCH in the context of a clinical case. While the focus of the present article is adult PLCH and its pulmonary manifestations, it is important for clinicians to distinguish the adult and pediatric forms of the disease, as well as to be alert for possible extrapulmonary complications. A major theme of the current series of articles on rare lung diseases has been the translation of insights gained from fundamental research to the clinic. Accordingly, the understanding of dendritic cell biology in this disease has led to important advances in the care of patients with PLCH.