[Pulmonary manifestations of Langerhans cell histiocytosis].

Abstract

Pulmonary Langerhans cell histiocytosis is a rare diffuse cystic interstitial pneumonia of unknown etiology that occurs selectively in young smokers of both genders. The multicenter studies conducted by the reference center have better defined the short and medium terms natural history of the disease and the clinical management of patients. A substantial proportion of patients experience a dramatic decline in their lung function soon after diagnosis. Importantly, smoking cessation is associated with a decreased risk of subsequent deterioration. Cladribine, a purine analogue, chemotherapy may dramatically improve lung function in patients with progressive pulmonary Langerhans cell histiocytosis, but this treatment should be used only in the setting of clinical research. Specific pulmonary hypertension therapies (anti-endothelin receptors, inhibitors of phosphodiesterases) may be used with caution in specialized centres for patients with severe pulmonary hypertension, and seem to be well tolerated. The recent identification of the V600E mutation of the BRAF oncogene in approximately half of the Langerhans cell histiocytosis lesions, including pulmonary granulomas, represents an important step forward in the understanding of the pathogenesis of Langerhans cell histiocytosis. Potentially it opens the way to targeted therapies.

• Authors: J Obert, A Tazi
• Journal: Revue des maladies respiratoires (Rev Mal Respir) Vol. 32 Issue 8 Pg. 850-66 (Oct 2015) ISSN: 1776-2588 [Electronic] France
• Vernacular Title: Manifestations pulmonaires de l'histiocytose langerhansienne.
• PMID: 26003197 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2015 SPLF. Published by Elsevier Masson SAS. All rights reserved.

Chemical References

• Antigens, CD1
• CD1a antigen
• Diuretics
• Endothelin Receptor Antagonists
• Phosphodiesterase Inhibitors
• Cladribine
• BRAF protein, human
• Proto-Oncogene Proteins B-raf

Topics

• Age of Onset
• Antigens, CD1 (analysis)
• Cladribine (therapeutic use)
• Combined Modality Therapy
• Diagnostic Techniques, Respiratory System
• Disease Progression
• Diuretics (therapeutic use)
• Endothelin Receptor Antagonists (therapeutic use)
• Histiocytes (chemistry, pathology)
• Histiocytosis, Langerhans-Cell (complications, drug therapy, epidemiology, genetics)
• Humans
• Hypertension, Pulmonary (drug therapy, etiology, physiopathology)
• Lung Diseases, Interstitial (diagnostic imaging, drug therapy, etiology, physiopathology)
• Lung Transplantation
• Mutation, Missense
• Oncogenes (genetics)
• Oxygen Inhalation Therapy
• Phosphodiesterase Inhibitors (therapeutic use)
• Point Mutation
• Prognosis
• Proto-Oncogene Proteins B-raf (genetics)
• Radiography
• Smoking (adverse effects)
• Smoking Cessation