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- Title
Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry.
- Authors
Fernández-Fabrellas, Estrella; Molina-Molina, María; Soriano, Joan B.; Portal, José Antonio Rodríguez; Ancochea, Julio; Valenzuela, Claudia; Xaubet, Antoni; SEPAR-IPF National Registry
- Abstract
<bold>Background: </bold>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).<bold>Methods: </bold>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.<bold>Results: </bold>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.<bold>Conclusions: </bold>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.
- Subjects
SPAIN; IDIOPATHIC pulmonary fibrosis; CARBON monoxide; THERAPEUTICS; COMPARATIVE studies; LONGITUDINAL method; RESEARCH methodology; MEDICAL cooperation; MEDICAL societies; NONSTEROIDAL anti-inflammatory agents; PYRIDINE; RESEARCH; EVALUATION research; ACQUISITION of data; RETROSPECTIVE studies
- Publication
Respiratory Research, 2019, Vol 20, Issue 1, pN.PAG
- ISSN
1465-9921
- Publication type
journal article
- DOI
10.1186/s12931-019-1084-0