TY - JOUR
T1 - Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases
AU - Cottin, Vincent
AU - Hirani, Nikhil A.
AU - Hotchkin, David L.
AU - Nambiar, Anoop M.
AU - Ogura, Takashi
AU - Otaola, María
AU - Skowasch, Dirk
AU - Park, Jong Sun
AU - Poonyagariyagorn, Hataya K.
AU - Wuyts, Wim
AU - Wells, Athol U.
N1 - Funding Information:
Provenance: Publication of this peer-reviewed article was sponsored by Boehringer Ingelheim Pharmaceuticals Inc., Ridgefield, CT, USA (principal sponsor, European Respiratory Review issue 150).
Funding Information:
Acknowledgements: The authors meet criteria for authorship as recommended by the International Committee of Medical Journal Editors (ICMJE). Writing assistance was provided by Ken Sutor of GeoMed, an Ashfield company, part of UDG Healthcare plc, which was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc. (BIPI). BIPI was given the opportunity to review the manuscript for medical and scientific accuracy as well as intellectual property considerations.
Funding Information:
Conflict of interest: V. Cottin reports receiving the following, outside the submitted work: personal fees from Actelion for consultancy, lectures and travel to medical meetings; personal fees from Boehringer Ingelheim for the development of educational presentations, consultancy, lectures and travel to medical meetings; personal fees from Bayer for consultancy; personal fees from Gilead for acting as a member of an adjudication committee; personal fees from GSK for consultancy; personal fees from MSD for consultancy and travel to medical meetings; personal fees from Novartis for consultancy and lectures; personal fees from Roche for consultancy, lecture fees and travel to medical meetings; personal fees from Sanofi for consultancy and lectures; a grant to his institution from Boehringer Ingelheim; a grant to his institution from Roche; personal fees from Promedior for acting as Chair of the DSMB; personal fees from Celgene for the DSMB; and personal fees from Galapagos for consultancy and for acting as Chair of the DSMB. N.A. Hirani has nothing to disclose. D.L. Hotchkin has nothing to disclose. A.M. Nambiar reports receiving the following, outside the submitted work: grants, personal fees, non-financial support and other support from Boehringer Ingelheim; and grants from Genentech-Roche. T. Ogura reports receiving the following, outside the submitted work: grants and personal fees from Boehringer Ingelheim, Japan; grants from the Ministry of Health, Labour and Welfare, Japan; personal fees from Astellas Pharma Inc., Shionogi & Co. Ltd, Toray Industries Inc., AstraZeneca K.K. and Kyorin Inc. M. Otaola has nothing to disclose. D. Skowasch reports receiving the following, outside the submitted work: personal fees/honoraria for consulting and speaking from Boehringer Ingelheim and Roche. J.S. Park has nothing to disclose. H.K. Poonyagariyagorn has nothing to disclose. W. Wuyts reports receiving the following, outside the submitted work: grants paid to his university from Boehringer Ingelheim and Hoffmann La Roche; and travel fees from Galapagos. A.U. Wells reports receiving the following, outside the submitted work: personal fees for speaking and for acting on advisory boards from Boehringer Ingelheim, Roche and Bayer.
Funding Information:
Support statement: The authors received no direct compensation related to the development of the manuscript. Writing assistance was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc. (BIPI).
PY - 2018
Y1 - 2018
N2 - Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased quality of life and, potentially, early death. Idiopathic pulmonary fibrosis may be regarded as a model for other progressive-fibrosing ILDs. Here we focus on other ILDs that may present a progressive-fibrosing phenotype, namely idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, connective tissue disease-associated ILDs (e.g. rheumatoid arthritis-related ILD), fibrotic chronic hypersensitivity pneumonitis, fibrotic chronic sarcoidosis and ILDs related to other occupational exposures. Differential diagnosis of these ILDs can be challenging, and requires detailed consideration of clinical, radiological and histopathological features. Accurate and early diagnosis is crucial to ensure that patients are treated optimally.
AB - Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased quality of life and, potentially, early death. Idiopathic pulmonary fibrosis may be regarded as a model for other progressive-fibrosing ILDs. Here we focus on other ILDs that may present a progressive-fibrosing phenotype, namely idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, connective tissue disease-associated ILDs (e.g. rheumatoid arthritis-related ILD), fibrotic chronic hypersensitivity pneumonitis, fibrotic chronic sarcoidosis and ILDs related to other occupational exposures. Differential diagnosis of these ILDs can be challenging, and requires detailed consideration of clinical, radiological and histopathological features. Accurate and early diagnosis is crucial to ensure that patients are treated optimally.
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U2 - 10.1183/16000617.0076-2018
DO - 10.1183/16000617.0076-2018
M3 - Review article
C2 - 30578335
AN - SCOPUS:85058921596
VL - 27
JO - European Respiratory Review
JF - European Respiratory Review
SN - 0905-9180
IS - 150
M1 - 180076
ER -