Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis
Jolene H. Fisher,Jolene H. Fisher,Martin Kolb,Mohmmed Algamdi,Julie Morisset,Kerri A. Johannson,Shane Shapera,Pearce G. Wilcox,T.M. To,Mohsen Sadatsafavi,Hélène Manganas,Nasreen Khalil,Nathan Hambly,Andrew J. Halayko,Andrea S. Gershon,Charlene D. Fell,Gerard Cox,Christopher J. Ryerson +17 more
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TLDR
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.Abstract:
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. 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. 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. 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.read more
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Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline.
Ganesh Raghu,Martine Remy-Jardin,Christopher J. Ryerson,Jeffrey L. Myers,Michael Kreuter,Martina Vasakova,Elena Bargagli,Jonathan H. Chung,Bridget F. Collins,Elisabeth Bendstrup,Hassan Chami,Abigail Chua,Tamera J. Corte,Jean-Charles Dalphin,Sonye K. Danoff,Javier Diaz-Mendoza,Abhijit Duggal,Ryoko Egashira,Thomas Ewing,Mridu Gulati,Yoshikazu Inoue,Alex R Jenkins,Kerri A. Johannson,Takeshi Johkoh,Maximiliano Tamae-Kakazu,Masanori Kitaichi,Shandra L Knight,Dirk Koschel,David J. Lederer,Yolanda N. Mageto,Lisa A. Maier,Carlos Matiz,Ferran Morell,Andrew G. Nicholson,Setu Patolia,Carlos Alberto de Castro Pereira,Elisabetta A. Renzoni,Margaret L. Salisbury,Moisés Selman,Simon L.F. Walsh,Wim Wuyts,Kevin C. Wilson +41 more
TL;DR: A systematic approach was developed to the diagnosis of hypersensitivity pneumonitis and the need for a thorough history and validated questionnaire to identify potential exposures was agreed on.
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Marlies S. Wijsenbeek,Michael Kreuter,Amy L. Olson,Aryeh Fischer,Elisabeth Bendstrup,Christopher D. Wells,Christopher P. Denton,Baher Mounir,Leila Zouad-Lejour,Manuel Quaresma,Vincent Cottin +10 more
TL;DR: It is estimated that 18–32% of patients diagnosed with non-IPF ILDs develop a progressive fibrosing phenotype and that these patients experience significant delays in the diagnosis of ILD and the detection of progressive fibrosis.
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COVID-19 and pulmonary fibrosis: A potential role for lung epithelial cells and fibroblasts.
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Patient Registries in Idiopathic Pulmonary Fibrosis
Daniel A. Culver,Jürgen Behr,John A. Belperio,Tamera J. Corte,Tamera J. Corte,Joao A. de Andrade,Kevin R. Flaherty,Mridu Gulati,Tristan J. Huie,Lisa Lancaster,Jesse Roman,Christopher J. Ryerson,Hyun J Kim +12 more
TL;DR: In the future, analyses of biospecimens linked to detailed patient profiles will provide the opportunity to identify biomarkers linked to disease progression, facilitating the development of precision medicine approaches for prognosis and therapy in patients with IPF.
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Ganesh Raghu,Harold R. Collard,Jim J. Egan,Fernando J. Martinez,Juergen Behr,Kevin K. Brown,Thomas V. Colby,Jean-François Cordier,Kevin R. Flaherty,Joseph A. Lasky,David A. Lynch,Jay H. Ryu,Jeffrey J. Swigris,Athol U. Wells,Julio Ancochea,Demosthenes Bouros,Carlos Roberto Ribeiro de Carvalho,Ulrich Costabel,Masahito Ebina,David M. Hansell,Takeshi Johkoh,Dong Soon Kim,Talmadge E. King,Yasuhiro Kondoh,Jeffrey L. Myers,Nestor L. Müller,Andrew G. Nicholson,Luca Richeldi,Moisés Selman,Rosalind F. Dudden,Barbara S. Griss,Shandra Protzko,Holger J. Schünemann +32 more
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TL;DR: This update is a supplement to the previous 2002 IIP classification document and outlines advances in the past decade and potential areas for future investigation.
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