Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis

Abstract Background The CAnadian REgistry for Pulmonary Fibrosis (CARE-PF) is a multi-center, prospective registry designed to study the natural history of fibrotic interstitial lung disease (ILD) in adults. The aim of this cross-sectional sub-study was to describe the baseline characteristics, risk...

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Main Authors: J. H. Fisher, M. Kolb, M. Algamdi, J. Morisset, K. A. Johannson, S. Shapera, P. Wilcox, T. To, M. Sadatsafavi, H. Manganas, N. Khalil, N. Hambly, A. J. Halayko, A. S. Gershon, C. D. Fell, G. Cox, C. J. Ryerson
Format: Article
Language:English
Published: BMC 2019-11-01
Series:BMC Pulmonary Medicine
Subjects:
Online Access:http://link.springer.com/article/10.1186/s12890-019-0986-4
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author J. H. Fisher
M. Kolb
M. Algamdi
J. Morisset
K. A. Johannson
S. Shapera
P. Wilcox
T. To
M. Sadatsafavi
H. Manganas
N. Khalil
N. Hambly
A. J. Halayko
A. S. Gershon
C. D. Fell
G. Cox
C. J. Ryerson
author_facet J. H. Fisher
M. Kolb
M. Algamdi
J. Morisset
K. A. Johannson
S. Shapera
P. Wilcox
T. To
M. Sadatsafavi
H. Manganas
N. Khalil
N. Hambly
A. J. Halayko
A. S. Gershon
C. D. Fell
G. Cox
C. J. Ryerson
author_sort J. H. Fisher
collection DOAJ
description Abstract Background The CAnadian REgistry for Pulmonary Fibrosis (CARE-PF) is a multi-center, prospective registry designed to study the natural history of fibrotic interstitial lung disease (ILD) in adults. The aim of this cross-sectional sub-study was to describe the baseline characteristics, risk factors, and comorbidities of patients enrolled in CARE-PF to date. Methods Patients completed study questionnaires and clinical measurements at enrollment and each follow-up visit. Environmental exposures were assessed by patient self-report and comorbidities by the Charlson Comorbidity Index (CCI). Baseline characteristics, exposures, and comorbidities were described for the overall study population and for incident cases, and were compared across ILD subtypes. Results The full cohort included 1285 patients with ILD (961 incident cases (74.8%)). Diagnoses included connective tissue disease-associated ILD (33.3%), idiopathic pulmonary fibrosis (IPF) (24.7%), unclassifiable ILD (22.3%), chronic hypersensitivity pneumonitis (HP) (7.5%), sarcoidosis (3.2%), non-IPF idiopathic interstitial pneumonias (3.0%, including idiopathic nonspecific interstitial pneumonia (NSIP) in 0.9%), and other ILDs (6.0%). Patient-reported exposures were most frequent amongst chronic HP, but common across all ILD subtypes. The CCI was ≤2 in 81% of patients, with a narrow distribution and range of values. Conclusions CTD-ILD, IPF, and unclassifiable ILD made up 80% of ILD diagnoses at ILD referral centers in Canada, while idiopathic NSIP was rare when adhering to recommended diagnostic criteria. CCI had a very narrow distribution across our cohort suggesting it may be a poor discriminator in assessing the impact of comorbidities on patients with ILD.
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spelling doaj.art-286eca04ece24d95aba91dfa51271f9a2022-12-21T22:39:31ZengBMCBMC Pulmonary Medicine1471-24662019-11-011911910.1186/s12890-019-0986-4Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary FibrosisJ. H. Fisher0M. Kolb1M. Algamdi2J. Morisset3K. A. Johannson4S. Shapera5P. Wilcox6T. To7M. Sadatsafavi8H. Manganas9N. Khalil10N. Hambly11A. J. Halayko12A. S. Gershon13C. D. Fell14G. Cox15C. J. Ryerson16Department of Medicine, University of TorontoFirestone Institute for Respiratory Health, Department of Medicine, McMaster UniversityDepartment of Pulmonary and Critical Care Medicine, King Fahad Medical CityDépartement de Médecine, Centre Hospitalier de l’Université de MontréalDepartment of Medicine, University of CalgaryDepartment of Medicine, University of TorontoDepartment of Medicine, University of British ColumbiaInstitute for Clinical Evaluative SciencesInstitute for Heart and Lung Health, Department of Medicine, University of British ColumbiaDépartement de Médecine, Centre Hospitalier de l’Université de MontréalDepartment of Medicine, University of British ColumbiaFirestone Institute for Respiratory Health, Department of Medicine, McMaster UniversityDepartment of Internal Medicine, University of ManitobaDepartment of Medicine, University of TorontoDepartment of Medicine, University of CalgaryFirestone Institute for Respiratory Health, Department of Medicine, McMaster UniversityDepartment of Medicine, University of British ColumbiaAbstract Background The CAnadian REgistry for Pulmonary Fibrosis (CARE-PF) is a multi-center, prospective registry designed to study the natural history of fibrotic interstitial lung disease (ILD) in adults. The aim of this cross-sectional sub-study was to describe the baseline characteristics, risk factors, and comorbidities of patients enrolled in CARE-PF to date. Methods Patients completed study questionnaires and clinical measurements at enrollment and each follow-up visit. Environmental exposures were assessed by patient self-report and comorbidities by the Charlson Comorbidity Index (CCI). Baseline characteristics, exposures, and comorbidities were described for the overall study population and for incident cases, and were compared across ILD subtypes. Results The full cohort included 1285 patients with ILD (961 incident cases (74.8%)). Diagnoses included connective tissue disease-associated ILD (33.3%), idiopathic pulmonary fibrosis (IPF) (24.7%), unclassifiable ILD (22.3%), chronic hypersensitivity pneumonitis (HP) (7.5%), sarcoidosis (3.2%), non-IPF idiopathic interstitial pneumonias (3.0%, including idiopathic nonspecific interstitial pneumonia (NSIP) in 0.9%), and other ILDs (6.0%). Patient-reported exposures were most frequent amongst chronic HP, but common across all ILD subtypes. The CCI was ≤2 in 81% of patients, with a narrow distribution and range of values. Conclusions CTD-ILD, IPF, and unclassifiable ILD made up 80% of ILD diagnoses at ILD referral centers in Canada, while idiopathic NSIP was rare when adhering to recommended diagnostic criteria. CCI had a very narrow distribution across our cohort suggesting it may be a poor discriminator in assessing the impact of comorbidities on patients with ILD.http://link.springer.com/article/10.1186/s12890-019-0986-4RegistriesLung diseasesInterstitialIdiopathic pulmonary fibrosisComorbidityEnvironmental exposure
spellingShingle J. H. Fisher
M. Kolb
M. Algamdi
J. Morisset
K. A. Johannson
S. Shapera
P. Wilcox
T. To
M. Sadatsafavi
H. Manganas
N. Khalil
N. Hambly
A. J. Halayko
A. S. Gershon
C. D. Fell
G. Cox
C. J. Ryerson
Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis
BMC Pulmonary Medicine
Registries
Lung diseases
Interstitial
Idiopathic pulmonary fibrosis
Comorbidity
Environmental exposure
title Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis
title_full Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis
title_fullStr Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis
title_full_unstemmed Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis
title_short Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis
title_sort baseline characteristics and comorbidities in the canadian registry for pulmonary fibrosis
topic Registries
Lung diseases
Interstitial
Idiopathic pulmonary fibrosis
Comorbidity
Environmental exposure
url http://link.springer.com/article/10.1186/s12890-019-0986-4
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