title: [Adult pulmonary Langerhans' cell histiocytosis: approach to the reality of the Spanish population].
abstract: OBJECTIVE
  Pulmonary Langerhans cell histiocytosis (PLCH) is a rare respiratory disease closely associated with smoking. The aim of the study is to know the reality of PLCH in the Catalonian population.
  
  
  METHODS
  We included all patients diagnosed of PLCH in 8 different Catalonian hospitals from April 2011 to December 2012. Epidemiologic, clinical, radiological, functional, diagnostic and therapeutic variables were collected, both at the beginning and at the last visit of the process.
  
  
  RESULTS
  Thirty two patients were included. Overall, patients were middle-aged (38 years), with no gender predominance. Most of them were smokers (71.9%). The main pulmonary function values (forced vital capacity 75.9%, forced expiratory volume in one second 68.9%, diffusing capacity of the lung for carbon monoxide 59.6%) and radiological findings (cysts 89.7%, nodules 60.3%) showed no significant changes during evolution. However, the disease progressed with a worsening lung function in 34.4% of cases. The analysis of the functional outcome (forced vital capacity, forced expiratory volume in one second, and diffusing capacity of the lung for carbon monoxide) did not reveal significant differences according to the persistence or not of smoking (P<0.05).
  
  
  CONCLUSIONS
  Our patients with PLCH had similar clinical and radiological characteristics to those described in other populations. The results observed regarding the relationship with smoking suggest that there may be other factors beyond smoking, which influence on the evolution of the disease. It therefore seems advisable to design prospective studies to investigate this aspect.
authors.affiliation: Servicio de Neumología, Hospital de la Santa Creu i Sant Pau, Institut d'Investigacions Biomèdiques (IIB-Sant Pau), Barcelona, España. Electronic address: dcastillo@santpau.cat.
authors.forename: Diego
authors.initials: D
authors.lastname: Castillo
authors.affiliation: Servicio de Neumología, Hospital de la Santa Creu i Sant Pau, Institut d'Investigacions Biomèdiques (IIB-Sant Pau), Barcelona, España.
authors.forename: Iñigo
authors.initials: I
authors.lastname: Martin-Arroyo
authors.affiliation: Servicio de Neumología, Hospital Universitari Parc Taulí, Sabadell, España.
authors.forename: Amalia
authors.initials: A
authors.lastname: Moreno
authors.affiliation: Servicio de Neumología, Servei de Pneumologia, Parc Salut Mar, Institut Municipal d'investigació mèdica (IMIM), Departamento de Ciencias Experimentales y de la Salud, Universitat Pompeu Fabra, Barcelona, España; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Bunyola, Islas Baleares, España.
authors.forename: Eva
authors.initials: E
authors.lastname: Balcells
authors.affiliation: Servicio de Neumología, Hospital Universitario Vall d'Hebron, Barcelona, España; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Bunyola, Islas Baleares, España.
authors.forename: Ana
authors.initials: A
authors.lastname: Villar
authors.affiliation: Unidad de Intersticio Pulmonar, Servicio de Neumología, Hospital Universitario de Bellvitge, Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), Hospitalet de Llobregat, España.
authors.forename: Vanesa
authors.initials: V
authors.lastname: Vicens-Zygmunt
authors.affiliation: Servicio de Neumología, Hospital Universitari Dr.Trueta, Institut d'Investigació Biomèdica de Girona Dr. Josep Trueta (IDIBGI), Girona, España; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Bunyola, Islas Baleares, España.
authors.forename: Montserrat
authors.initials: M
authors.lastname: Vendrell
authors.affiliation: Servicio de Neumología, Hospital Universitari Dr.Trueta, Institut d'Investigació Biomèdica de Girona Dr. Josep Trueta (IDIBGI), Girona, España.
authors.forename: Sonia
authors.initials: S
authors.lastname: Belda
authors.affiliation: Servicio de Neumología, Hospital Universitari Germans Trias i Pujol, Badalona, España.
authors.forename: Karina
authors.initials: K
authors.lastname: Portillo
authors.affiliation: Servicio de Neumología, Hospital Universitari Arnau de Vilanova, Lleida, España.
authors.forename: Dalila
authors.initials: D
authors.lastname: Kettou
authors.affiliation: Grupo de investigación en Enfermedades Respiratorias Crónicas. Institut d'Investigacions Biomèdiques (IIB-Sant Pau), Barcelona, España.
authors.forename: Francisco
authors.initials: F
authors.lastname: Aparicio
authors.affiliation: Unidad de Intersticio Pulmonar, Servicio de Neumología, Hospital Universitario de Bellvitge, Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), Hospitalet de Llobregat, España; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Bunyola, Islas Baleares, España.
authors.forename: Maria
authors.initials: M
authors.lastname: Molina-Molina
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018450:Disease Progression; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012307:Risk Factors; D013030:Spain / Q000453:epidemiology
keywords: Adult; Adultos; Case series; España; Histiocitosis células Langerhans; Langerhans’ cell Histiocytosis; Lung; Pulmón; Serie de casos; Spain