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Canadian Respiratory Journal
Volume 2016, Article ID 3740902, 5 pages
http://dx.doi.org/10.1155/2016/3740902
Clinico-Pathologic Conferences

Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

1Division of Respirology, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada K1H 8L6
2Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada K1H 8L6
3Department of Pathology and Laboratory Medicine, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada K1H 8L6

Received 23 November 2014; Accepted 6 June 2015

Copyright © 2016 K. Earlam et al. 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.

Abstract

Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement.