University of Oulu

Kaunisto J, Salomaa E-R, Hodgson U,et al. Demographics and survival ofpatients with idiopathic pulmonary fibrosis in the FinnishIPF registry.ERJ Open Res2019; 5: 00170-2018 [https://doi.org/10.1183/23120541.00170-2018].

Demographics and survival of patients with idiopathic pulmonary fibrosis in the FinnishIPF registry

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Author: Kaunisto, Jaana1,2; Salomaa, Eija-Riitta2; Hodgson, Ulla3,4;
Organizations: 1Division of Medicine, Dept of Pulmonary Diseases, Turku University Hospital, Turku, Finland
2Dept of Pulmonary Diseases and Clinical Allergology, University of Turku, Turku, Finland
3Heart and Lung Center, Helsinki University Hospital, Helsinki, Finland
4Dept of Medicine, University of Helsinki, Helsinki, Finland
5Respiratory Medicine, Research Unit of Internal Medicine, University of Oulu, Oulu, Finland
6Medical Research Center Oulu, Oulu University Hospital, Oulu, Finland
7Dept of Respiratory Medicine, Seinäjoki Central Hospital, Seinäjoki, Finland
8Faculty of Medicine and Health Technology, University of Tampere, Tampere, Finland
9Research Programs Unit, Individrug Research Program, Medical Faculty, University of Helsinki, Helsinki, Finland
10Dept of Biostatistics, University of Turku, Turku, Finland
Format: article
Version: published version
Access: open
Online Access: PDF Full Text (PDF, 0.5 MB)
Persistent link: http://urn.fi/urn:nbn:fi-fe202003319856
Language: English
Published: European Respiratory Society, 2019
Publish Date: 2020-03-31
Description:

Abstract

Idiopathic pulmonary fibrosis (IPF) is characterised by unpredictable disease course and poor survival. After the introduction of novel antifibrotic drugs, the prognosis of patients with IPF is probably changing.

FinnishIPF, a nationwide registry of carefully characterised patients, was initiated in Finland in 2011. For the data analysis, we included 453 incident IPF patients diagnosed during 2011–2015. In this study, we describe the demographics and prognosis of these real-life patients.

The median overall survival time of registered IPF patients was 4.5 years. The transplant-free survival at 1, 2, 3, 4 and 5 years was 95%, 83%, 70%, 58% and 45%, respectively. Smoking did not have any effect on survival. 117 (26%) patients received pirfenidone or nintedanib. Patients who received ≥6 months of treatment had better survival compared with those who did not receive treatment but this difference disappeared after age adjustment. The transplantation rate was 3%.

Although IPF is diagnosed in Finland at a older age, the prognosis is better than expected due to a relatively well preserved lung function at diagnosis. Age and pulmonary function were identified as independent predictors of survival in the entire IPF patient population as well as in patients who had received antifibrotic treatment.

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Series: ERJ open research
ISSN: 2312-0541
ISSN-E: 2312-0541
ISSN-L: 2312-0541
Volume: 5
Issue: 3
Article number: 00170-2018
DOI: 10.1183/23120541.00170-2018
OADOI: https://oadoi.org/10.1183/23120541.00170-2018
Type of Publication: A1 Journal article – refereed
Field of Science: 3121 General medicine, internal medicine and other clinical medicine
Subjects:
Funding: We thank the Sigrid Jusélius Foundation, The Academy of Finland, The Research Foundation of the Pulmonary Diseases, Väinö and Laina Kivi Foundation, Jalmari and Rauha Ahokas Foundation, The Paulo Foundation and The Finnish Anti-Tuberculosis Association Foundation.
Copyright information: © ERS 2019. This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.
  https://creativecommons.org/licenses/by-nc/4.0/