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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BMC
2019-11-01
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Series: | BMC Pulmonary Medicine |
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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. |
first_indexed | 2024-12-16T07:25:38Z |
format | Article |
id | doaj.art-286eca04ece24d95aba91dfa51271f9a |
institution | Directory Open Access Journal |
issn | 1471-2466 |
language | English |
last_indexed | 2024-12-16T07:25:38Z |
publishDate | 2019-11-01 |
publisher | BMC |
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series | BMC Pulmonary Medicine |
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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