Delay and inequalities in the treatment of idiopathic pulmonary fibrosis: the case of two Nordic countries

Abstract Background Idiopathic pulmonary fibrosis (IPF) is characterized by progressive loss of lung function with high mortality within the first 5 years from diagnosis. In 2011–2014, two drugs, pirfenidone and nintedanib, have been approved worldwide for prevention of IPF progression. National IPF...

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Main Authors: Ida Pesonen, Lisa Carlson, Nicola Murgia, Riitta Kaarteenaho, Carl Magnus Sköld, Marjukka Myllärniemi, Giovanni Ferrara
Format: Article
Language:English
Published: PAGEPress Publications 2018-05-01
Series:Multidisciplinary Respiratory Medicine
Subjects:
Online Access:http://link.springer.com/article/10.1186/s40248-018-0126-7
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author Ida Pesonen
Lisa Carlson
Nicola Murgia
Riitta Kaarteenaho
Carl Magnus Sköld
Marjukka Myllärniemi
Giovanni Ferrara
author_facet Ida Pesonen
Lisa Carlson
Nicola Murgia
Riitta Kaarteenaho
Carl Magnus Sköld
Marjukka Myllärniemi
Giovanni Ferrara
author_sort Ida Pesonen
collection DOAJ
description Abstract Background Idiopathic pulmonary fibrosis (IPF) is characterized by progressive loss of lung function with high mortality within the first 5 years from diagnosis. In 2011–2014, two drugs, pirfenidone and nintedanib, have been approved worldwide for prevention of IPF progression. National IPF-registries have been established in both Finland and Sweden. Our study explored potential differences in the care of IPF in these two countries. Methods Patients included consecutively in the Finnish and Swedish IPF-registries from January 1, 2014 through December 31, 2016 were included in the study. Data on demographics and lung function at the time of inclusion were collected. Access to antifibrotic drugs and data on disease outcomes, mortality and the proportion of patients who underwent lung transplantation, was collected during a 3-year follow up. Results One-hundred and fifty-two patients from the Finnish and 160 patients from the Swedish IPF-cohorts were included in the study. At inclusion, Finnish patients were significantly older than the Swedish patients (74.6 years vs 72.5 years, p = 0.017). The proportion of non-smokers was significantly higher in the Finnish cohort (41.7% vs 26.9%, p = 0.007). Forced vital capacity (FVC), % of predicted (78.2 vs 71.7 for Finnish and Swedish patients, respectively, p = 0.01) and diffusion capacity for carbon monoxide (DLCO), % of predicted (53.3 vs 48.2 for Finnish and Swedish patients, respectively, p = 0.002) were significantly higher in the Finnish cohort compared to the Swedish cohort at the time of inclusion. During the 3-year follow up period, 45 (29.6%) Finnish and 111 (69.4%) Swedish patients, respectively, were initiated on treatment with an antifibrotic drug (pirfenidone or nintedanib) (p <  0.001). When comparing possible determinants of treatment, patients with higher FVC % were less likely to start antifibrotic drugs (OR 0.96, 95%CI 0.93–1.00, p <  0.024). To be resident in Sweden was the main determinant for receiving antifibrotic drugs (OR 5.48, 95%CI 2.65–11.33, p < 0.0001). No significant difference in number of deaths and lung transplantation during the follow up period was found. Conclusions This study highlights differences concerning how IPF patients are treated in Finland and Sweden. How these differences will influence the long-term outcome of these patients is unknown.
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spelling doaj.art-b5466693b9c645da8755df43b022b8652024-03-02T15:39:40ZengPAGEPress PublicationsMultidisciplinary Respiratory Medicine2049-69582018-05-011311610.1186/s40248-018-0126-7Delay and inequalities in the treatment of idiopathic pulmonary fibrosis: the case of two Nordic countriesIda Pesonen0Lisa Carlson1Nicola Murgia2Riitta Kaarteenaho3Carl Magnus Sköld4Marjukka Myllärniemi5Giovanni Ferrara6Department of Respiratory Medicine and Allergy, Karolinska University HospitalDepartment of Respiratory Medicine and Allergy, Karolinska University HospitalSection of Occupational Medicine, Respiratory Diseases and Toxicology, University of PerugiaResearch Unit of Internal Medicine, University of Oulu and Medical Research Center Oulu, Oulu University HospitalDepartment of Respiratory Medicine and Allergy, Karolinska University HospitalUniversity of Helsinki and Helsinki University Hospital, Heart and Lung CenterDepartment of Respiratory Medicine and Allergy, Karolinska University HospitalAbstract Background Idiopathic pulmonary fibrosis (IPF) is characterized by progressive loss of lung function with high mortality within the first 5 years from diagnosis. In 2011–2014, two drugs, pirfenidone and nintedanib, have been approved worldwide for prevention of IPF progression. National IPF-registries have been established in both Finland and Sweden. Our study explored potential differences in the care of IPF in these two countries. Methods Patients included consecutively in the Finnish and Swedish IPF-registries from January 1, 2014 through December 31, 2016 were included in the study. Data on demographics and lung function at the time of inclusion were collected. Access to antifibrotic drugs and data on disease outcomes, mortality and the proportion of patients who underwent lung transplantation, was collected during a 3-year follow up. Results One-hundred and fifty-two patients from the Finnish and 160 patients from the Swedish IPF-cohorts were included in the study. At inclusion, Finnish patients were significantly older than the Swedish patients (74.6 years vs 72.5 years, p = 0.017). The proportion of non-smokers was significantly higher in the Finnish cohort (41.7% vs 26.9%, p = 0.007). Forced vital capacity (FVC), % of predicted (78.2 vs 71.7 for Finnish and Swedish patients, respectively, p = 0.01) and diffusion capacity for carbon monoxide (DLCO), % of predicted (53.3 vs 48.2 for Finnish and Swedish patients, respectively, p = 0.002) were significantly higher in the Finnish cohort compared to the Swedish cohort at the time of inclusion. During the 3-year follow up period, 45 (29.6%) Finnish and 111 (69.4%) Swedish patients, respectively, were initiated on treatment with an antifibrotic drug (pirfenidone or nintedanib) (p <  0.001). When comparing possible determinants of treatment, patients with higher FVC % were less likely to start antifibrotic drugs (OR 0.96, 95%CI 0.93–1.00, p <  0.024). To be resident in Sweden was the main determinant for receiving antifibrotic drugs (OR 5.48, 95%CI 2.65–11.33, p < 0.0001). No significant difference in number of deaths and lung transplantation during the follow up period was found. Conclusions This study highlights differences concerning how IPF patients are treated in Finland and Sweden. How these differences will influence the long-term outcome of these patients is unknown.http://link.springer.com/article/10.1186/s40248-018-0126-7Idiopathic pulmonary fibrosisInterstitial lung diseaseNintedanibPirfenidoneAccess to healthcare
spellingShingle Ida Pesonen
Lisa Carlson
Nicola Murgia
Riitta Kaarteenaho
Carl Magnus Sköld
Marjukka Myllärniemi
Giovanni Ferrara
Delay and inequalities in the treatment of idiopathic pulmonary fibrosis: the case of two Nordic countries
Multidisciplinary Respiratory Medicine
Idiopathic pulmonary fibrosis
Interstitial lung disease
Nintedanib
Pirfenidone
Access to healthcare
title Delay and inequalities in the treatment of idiopathic pulmonary fibrosis: the case of two Nordic countries
title_full Delay and inequalities in the treatment of idiopathic pulmonary fibrosis: the case of two Nordic countries
title_fullStr Delay and inequalities in the treatment of idiopathic pulmonary fibrosis: the case of two Nordic countries
title_full_unstemmed Delay and inequalities in the treatment of idiopathic pulmonary fibrosis: the case of two Nordic countries
title_short Delay and inequalities in the treatment of idiopathic pulmonary fibrosis: the case of two Nordic countries
title_sort delay and inequalities in the treatment of idiopathic pulmonary fibrosis the case of two nordic countries
topic Idiopathic pulmonary fibrosis
Interstitial lung disease
Nintedanib
Pirfenidone
Access to healthcare
url http://link.springer.com/article/10.1186/s40248-018-0126-7
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