Diego Castillo1, Iñigo Martin-Arroyo2, Amalia Moreno3, Eva Balcells4, Ana Villar5, Vanesa Vicens-Zygmunt6, Montserrat Vendrell7, Sonia Belda8, Karina Portillo9, Dalila Kettou10, Francisco Aparicio11, Maria Molina-Molina12. 1. 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. 2. Servicio de Neumología, Hospital de la Santa Creu i Sant Pau, Institut d'Investigacions Biomèdiques (IIB-Sant Pau), Barcelona, España. 3. Servicio de Neumología, Hospital Universitari Parc Taulí, Sabadell, España. 4. 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. 5. Servicio de Neumología, Hospital Universitario Vall d'Hebron, Barcelona, España; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Bunyola, Islas Baleares, España. 6. Unidad de Intersticio Pulmonar, Servicio de Neumología, Hospital Universitario de Bellvitge, Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), Hospitalet de Llobregat, España. 7. 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. 8. Servicio de Neumología, Hospital Universitari Dr.Trueta, Institut d'Investigació Biomèdica de Girona Dr. Josep Trueta (IDIBGI), Girona, España. 9. Servicio de Neumología, Hospital Universitari Germans Trias i Pujol, Badalona, España. 10. Servicio de Neumología, Hospital Universitari Arnau de Vilanova, Lleida, España. 11. Grupo de investigación en Enfermedades Respiratorias Crónicas. Institut d'Investigacions Biomèdiques (IIB-Sant Pau), Barcelona, España. 12. 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.
Abstract
BACKGROUND AND 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. MATERIAL AND 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). CONCLUSION: 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.
BACKGROUND AND 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. MATERIAL AND 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). CONCLUSION: 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.