Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain

Little is known on the characteristics of patients diagnosed with idiopathic pulmonary fibrosis (IPF) in Spain. We aimed to characterize the demographic and clinical profile of IPF patients included in the IPF National Registry of the Spanish Respiratory Society (SEPAR). This is a prospective, obser...

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Detalles Bibliográficos
Autores: Fernández-Fabrellas, Estrella, Molina Molina, Maria|||0000-0002-1852-1723, Soriano, Joan B.|||0000-0001-9740-2994, Rodríguez Portal, José Antonio, Ancochea, Julio|||0000-0001-7451-4133, Valenzuela, Claudia|||0000-0002-0856-6290, Xaubet, Antoni, Aburto, Myriam|||0000-0001-8644-1015, Alfageme, Inmaculada|||0000-0002-5106-1669, Bollo de Miguel, Elena, Cano, Esteban, Casanova Espinosa, Álvaro, Castillo, Diego|||0000-0002-4862-3595, Figuerola Mendal, J.A., García Sevila, Raquel|||0000-0002-4218-793X, Gaudó Navarro, Javier I., Gómez-Carrera, Luis, Saez González, Jesus Miguel, Herrera Lara, Susana, Laporta Hernández, Rosalía, Marín González, M., Nieto Barbero, Asunción, Portillo, Karina|||0000-0002-7804-1051, Romero, Ana Dolores, Sánchez Simón-Talero, Rafaela, Sancho Chust, José N.|||0000-0002-3028-0715, Sellares, Jacobo, Soler-Sempere, Maria J.|||0000-0002-6607-2037, Sauleda, Jaume|||0000-0002-9806-3370, Tomás López, Laura, Villanueva, Manuel Angel|||0000-0003-3256-9579
Tipo de recurso: artículo
Fecha de publicación:2019
País:España
Institución:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:285821
Acceso en línea:https://ddd.uab.cat/record/285821
https://dx.doi.org/urn:doi:10.1186/s12931-019-1084-0
Access Level:acceso abierto
Palabra clave:Anti-fibrotic treatment
Idiopathic pulmonary fibrosis
National registry
SEPAR
Spain
Descripción
Sumario:Little is known on the characteristics of patients diagnosed with idiopathic pulmonary fibrosis (IPF) in Spain. We aimed to characterize the demographic and clinical profile of IPF patients included in the IPF National Registry of the Spanish Respiratory Society (SEPAR). This is a prospective, observational, multicentre and nationwide study that involved 608 IPF patients included in the SEPAR IPF Registry up to June 27th, 2017, and who received any treatment for their disease. IPF patients were predominantly males, ex-smokers, and aged in their 70s, similar to other registries. Upon inclusion, mean ± SD predicted forced vital capacity was 77.6% ± 19.4, diffusing capacity for carbon monoxide was 48.5% ± 17.7, and the 6-min walk distance was 423.5 m ± 110.4. The diagnosis was mainly established on results from the high-resolution computed tomography in the proper clinical context (55.0% of patients), while 21.2% of patients required invasive procedures (surgical lung biopsy) for definitive diagnosis. Anti-fibrotic treatment was prescribed in 69.4% of cases, 51.5% pirfenidone and 17.9% nintedanib, overall with a good safety profile. The SEPAR IPF Registry should help to further characterize current characteristics and future trends of IPF patients in Spain and compare/pool them with other registries and cohorts.