Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

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...

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Main Authors: Stephen C Juvet, David Hwang, Gregory P Downey
Format: Article
Language:English
Published: Wiley 2010-01-01
Series:Canadian Respiratory Journal
Online Access:http://dx.doi.org/10.1155/2010/216240
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author Stephen C Juvet
David Hwang
Gregory P Downey
author_facet Stephen C Juvet
David Hwang
Gregory P Downey
author_sort Stephen C Juvet
collection DOAJ
description 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.
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institution Kabale University
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series Canadian Respiratory Journal
spelling doaj-art-5745c68703c44666825af868f27192922025-02-03T06:47:24ZengWileyCanadian Respiratory Journal1198-22412010-01-01173e55e6210.1155/2010/216240Rare Lung Diseases III: Pulmonary Langerhans’ Cell HistiocytosisStephen C Juvet0David Hwang1Gregory P Downey2Division of Respirology and Clinician-Scientist Training Program, Department of Medicine, University Health Network, Toronto, Ontario, CanadaDepartment of Laboratory Medicine and Pathobiology, and Toronto General Hospital/University Health Network, Toronto, Ontario, CanadaDepartments of Medicine, Pediatrics, and Immunology, National Jewish Health and University of Colorado, Aurora, Colorado, USAPulmonary 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.http://dx.doi.org/10.1155/2010/216240
spellingShingle Stephen C Juvet
David Hwang
Gregory P Downey
Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis
Canadian Respiratory Journal
title Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis
title_full Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis
title_fullStr Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis
title_full_unstemmed Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis
title_short Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis
title_sort rare lung diseases iii pulmonary langerhans cell histiocytosis
url http://dx.doi.org/10.1155/2010/216240
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AT davidhwang rarelungdiseasesiiipulmonarylangerhanscellhistiocytosis
AT gregorypdowney rarelungdiseasesiiipulmonarylangerhanscellhistiocytosis