[Adult pulmonary Langerhans cell histiocytosis].
There is a strong relationship between tobacco smoking and pulmonary Langerhans cell histiocytosis (LCH) in adults. Definitive diagnosis relies on lung histology. In certain cases, the diagnosis can be retained on a typical nodulo-cystic pattern on lung high-resolution computed tomography (HRCT) and appropriate clinical setting. The main differential diagnoses to consider vary according to nodulo-cystic or only cystic pattern on lung HRCT and the clinical context. The natural history of the disease is better known and regular evaluation of respiratory function is essential during the follow-up of the patients. The disease may regress or remain stable and mild without treatment in a significant number of cases. Smoking cessation is the essential first step because it reduces the risk of early disease progression. Cladribine is the only treatment that has shown improvement in respiratory function. Its efficacy and tolerance will be clarified in the near future. Therapies targeting mutations in the MAPK pathway represent new therapeutic options that need to be appropriately evaluated pulmonary LCH.
Assistance publique-Hôpitaux de Paris, hôpital Saint-Louis, centre national de référence de l'histiocytose Langerhansienne, service de pneumologie, 75475 Paris cedex 10, France.
Gwenaël
G
Lorillon
Assistance publique-Hôpitaux de Paris, hôpital Saint-Louis, service de pathologie, Inserm UMR_S1165, 75475 Paris cedex 10, France.
Véronique
V
Meignin
Assistance publique-Hôpitaux de Paris, hôpital Saint-Louis, centre national de référence de l'histiocytose Langerhansienne, service de pneumologie, 75475 Paris cedex 10, France; Université Paris-Diderot, Sorbonne Paris Cité, Inserm UMR 1153 CRESS, équipe de recherche en biostatistiques etépidémiologie clinique, 75013 Paris, France. Electronic address: abdellatif.tazi@aphp.fr.
Abdellatif
A
Tazi
D000328:Adult; D017668:Age of Onset; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D006801:Humans; D008297:Male; D013902:Radiography, Thoracic