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Published in: Respiratory Research 1/2019

Open Access 01-12-2019 | Idiopathic Pulmonary Fibrosis | Research

Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry

Authors: Estrella Fernández-Fabrellas, María Molina-Molina, Joan B. Soriano, José Antonio Rodríguez Portal, Julio Ancochea, Claudia Valenzuela, Antoni Xaubet, on behalf of the SEPAR-IPF National Registry

Published in: Respiratory Research | Issue 1/2019

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Abstract

Background

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).

Methods

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.

Results

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.

Conclusions

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.
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Metadata
Title
Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry
Authors
Estrella Fernández-Fabrellas
María Molina-Molina
Joan B. Soriano
José Antonio Rodríguez Portal
Julio Ancochea
Claudia Valenzuela
Antoni Xaubet
on behalf of the SEPAR-IPF National Registry
Publication date
01-12-2019
Publisher
BioMed Central
Published in
Respiratory Research / Issue 1/2019
Electronic ISSN: 1465-993X
DOI
https://doi.org/10.1186/s12931-019-1084-0

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