title: [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.affiliation: Service de pneumologie, Centre national de référence de l'histiocytose langerhansienne, hôpital Saint-Louis, Assistance publique-Hôpitaux de Paris, 1, avenue Claude-Vellefaux, 75475 Paris cedex 10, France.
authors.forename: J
authors.initials: J
authors.lastname: Obert
authors.affiliation: Service de pneumologie, Centre national de référence de l'histiocytose langerhansienne, hôpital Saint-Louis, Assistance publique-Hôpitaux de Paris, 1, avenue Claude-Vellefaux, 75475 Paris cedex 10, France; U1153 CRESS, équipe de recherche en biostatistiques et épidémiologie clinique, université Paris Diderot, Sorbonne Paris Cité, 75013 Paris, France. Electronic address: abdellatif.tazi@sls.aphp.fr.
authors.forename: A
authors.initials: A
authors.lastname: Tazi
mesh_terms: D017668:Age of Onset; D018949:Antigens, CD1 / Q000032:analysis; D017338:Cladribine / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003948:Diagnostic Techniques, Respiratory System; D018450:Disease Progression; D004232:Diuretics / Q000627:therapeutic use; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use; D006644:Histiocytes / Q000737:chemistry / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D016040:Lung Transplantation; D020125:Mutation, Missense; D009857:Oncogenes / Q000235:genetics; D010102:Oxygen Inhalation Therapy; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D017354:Point Mutation; D011379:Prognosis; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics; D011859:Radiography; D012907:Smoking / Q000009:adverse effects; D016540:Smoking Cessation
chemical_list: D018949:Antigens, CD1; C419393:CD1a antigen; D004232:Diuretics; D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D017338:Cladribine; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf
keywords: BRAF; Cellules dendritiques; Dendritic cells; Diffuse cystic lung disease; High-resolution computed tomography; MAP kinases; Pneumopathies infiltrantes kystiques; Tomodensitométrie haute résolution